Objective Acquired aplastic anemia(aAA)is characterized by an autologous immunological attack against hematopoietic stem and progenitor cells,and immunotolerance disruption is frequent,with reduced T regulatory cells(...Objective Acquired aplastic anemia(aAA)is characterized by an autologous immunological attack against hematopoietic stem and progenitor cells,and immunotolerance disruption is frequent,with reduced T regulatory cells(Tregs)frequencies and increased effector cytotoxic cells.Tregs are reduced in aAA and increase in number after successful immunosuppressive therapies.Methods In this retrospective study,we investigated the frequency of circulating Tregs by multiparametric flow cytometry immunophenotyping in non-severe aAA patients before and after immunosuppressive therapy.The samples were stained with the following antibodies:ECD anti-CD3,PE or PC5 anti-CD4,FITC anti-CD8,and PE anti-CD25,and Tregs were identified by first gating on linear parameters for lymphocyte identification and then for CD3 expression.In CD3+CD4+cells,Tregs were further identified on the basis of CD25 and FOXP3 expression.Results Although the number of Tregs tended to increase after immunosuppressive treatments,their circulating frequency remained lower than that of healthy subjects,regardless of their responsiveness to therapies.Moreover,the relative frequency combined with absolute Treg counts might be more informative in the differential diagnosis of bone marrow failure syndromes.Conclusions The persistent decrease in circulating Tregs could be the result of immunosuppressive agents that could preferentially expand other T-cell subsets.At the same time,an imbalance in immunotolerance might persist,which is also favored by chronic antigen stimulation.展开更多
Objective:To observe the effects of moxibustion on the hematopoietic function in mice with aplastic anemia(AA)induced by bone marrow(BM)suppression,and to investigate the intervention effects of moxibustion on AA from...Objective:To observe the effects of moxibustion on the hematopoietic function in mice with aplastic anemia(AA)induced by bone marrow(BM)suppression,and to investigate the intervention effects of moxibustion on AA from the perspective of intestinal bacteria.Methods:A total of 24 C57BL/6 J male mice were randomly and evenly divided into control,model and moxibustion groups.The myelosuppression-induced AA model was established by cyclophosphamide(CTX)and cyclosporine(Cs)intraperitoneal injection.Mice in the moxibustion group were intervened in mild moxibustion at unilateral“Zusanli(ST36)”acupoint for 15 min per day,and the sides were switched the next day.The intervention of mild moxibustion lasted 60 days consecutively.The red blood cell(RBC),white blood cell(WBC),platelet(PLT)counts and haemoglobin(Hb)concentration levels of mice in each group were detected by peripheral blood cells count staining,and the BM hematopoietic cells and hematopoietic structures were observed by BM smear Wright-Giemsa staining and HE staining.16SrDNA sequencing was used to analyze the gut bacterial species abundance and diversity in mice from each group.Results:After all the intervention,compared to the control group,the model group had lower levels of RBC,WBC,PLT counts and Hb concentration in peripheral blood(P<0.05)and fewer hematopoietic cells and hematopoietic structures;compared to the model group,moxibustion group had higher levels of RBC,WBC,PLT counts and Hb concentration in peripheral blood(P<0.05),and more BM hematopoietic cells and hematopoietic structures.Gut flora showed that moxibustion increased the species richness and diversity of intestinal bacteria in mice;compared with the control group,the relative abundance of Faecalibaculum and Anaeroplasmataceae in the model group was higher(P<0.05);whereas,the relative abundance of Faecalibaculum and Anaeroplasmataceae in the moxibustion group was lower(P<0.05)when compared with the model group.In addition,Faecalibaculum was significantly correlated with RBC,WBC,PLT count and Hb concentration(P<0.05).Conclusion:Moxibustion can improved BM histology,restored hematopoietic cells function,and increased peripheral blood cells count and Hb concentration in AA mice.The mechanism may be related to the fact that moxibustion regulates the abundance of specific intestinal bacteria to maintain the stability of the flora structure.展开更多
OBJECTIVE:To investigate the effect of Shisiwei Jianzhong decoction(十四味建中汤,SJD)on non-severe aplastic anemia(NSAA).METHODS:Bone marrow mesenchymal stem cells(BMSCs)were isolated from bone marrow samples of 15 NS...OBJECTIVE:To investigate the effect of Shisiwei Jianzhong decoction(十四味建中汤,SJD)on non-severe aplastic anemia(NSAA).METHODS:Bone marrow mesenchymal stem cells(BMSCs)were isolated from bone marrow samples of 15 NSAA patients and 3 healthy controls.Cells were treated with gradient concentrations of SJD,and a portion was transfected with a vector overexpressing the nuclear factor of activated T cells,cytoplasmic 4(NFATC4).Cell viability and apoptosis were detected by cell counting kit-8 and flow cytometry,respectively.After adipogenic differentiation induction,lipid droplet formation in BMSCs was examined by Oil Red O staining.The expression of NFATC4,peroxisome proliferator-activated receptor gamma(PPARG),fatty acid-binding protein 4(FABP4),peroxisome proliferator-activated receptor-gamma coactivator(PGC-1α),and acetylated PGC-1αwas measured by quantitative real-time polymerase chain reaction or Western blot.RESULTS:SJD significantly increased the viability and decreased the apoptosis of NSAA-derived BMSCs.It also dose-dependently inhibited lipid droplet formation and decreased the expression of PPARG and FABP4 in NSAA-derived BMSCs.NFATC4 expression was higher in patients with NSAA than in healthy controls,and SJD downregulated its expression.NFATC4 overexpression reversed the inhibitory effect of SJD on adipogenic differentiation.Additionally,SJD promoted the deacetylation of PGC-1αin NSAA-derived BMSCs,which was also partially eliminated by NFATC4 overexpression.CONCLUSIONS:SJD inhibits adipogenic differentiation of BMSCs through downregulating NFATC4,thereby contributing to the remission of NSAA.展开更多
To investigate the efficacy of a structured continuous nursing intervention program on clinical outcomes,self-efficacy,and sleep quality in patients diagnosed with aplastic anemia(AA).Methods:This study was a single-c...To investigate the efficacy of a structured continuous nursing intervention program on clinical outcomes,self-efficacy,and sleep quality in patients diagnosed with aplastic anemia(AA).Methods:This study was a single-center,parallel-group,randomized controlled trial.A total of 64 patients with AA,treated at our hospital’s hematology department from May 2022 to May 2025,were enrolled.Participants were randomly allocated in a 1:1 ratio to either the control group(n=32),receiving routine nursing care,or the intervention group(n=32),receiving a six-month continuous nursing intervention program in addition to routine care.The intervention program consisted of structured health education,individualized psychological support,regular telephone follow-ups,and a 24/7 online communication platform.The primary outcome was the change in the Pittsburgh Sleep Quality Index(PSQI)score from baseline to six months.Secondary outcomes included hematological parameters(hemoglobin[Hb],absolute neutrophil count[ANC],platelet count[PLT]),incidence of adverse events(infections,bleeding episodes),quality of life(assessed by the Functional Assessment of Cancer Therapy–Anemia[FACT-An]),and self-efficacy(assessed by the General Self-Efficacy Scale[GSE]).Statistical analyses were performed using independent t-tests,and chi-square tests or Fisher’s exact tests as appropriate.Results:A total of 55 patients(28 in the intervention group and 27 in the control group)completed the study.At baseline,there were no statistically significant differences in demographic data,clinical characteristics,or outcome measures between the two groups(p>0.05).After six months,the mean PSQI score in the intervention group was significantly lower than that in the control group(7.12±2.05 vs.13.49±2.88;t=−13.450;p<0.001).The intervention group also demonstrated a significantly lower incidence of infections(4/28,14.29%vs.10/27,37.04%;p=0.048)and bleeding episodes requiring intervention(3/28,10.71%vs.9/27,33.33%;p=0.042).Furthermore,patients in the intervention group reported significantly higher scores on the FACT-An(125.70±10.31 vs.109.44±12.10;t=7.934;p<0.001)and the GSE scale(29.82±4.11 vs.23.51±4.80;t=7.311;p<0.001).While hematological parameters showed a trend toward improvement in the intervention group,the differences were not statistically significant compared to the control group(p>0.05).Conclusion:The implementation of a structured continuous nursing intervention program can significantly improve sleep quality,reduce the incidence of complications,and enhance quality of life and self-efficacy in patients with aplastic anemia.This evidence-based model of care should be considered for integration into standard clinical practice for the long-term management of this patient population.展开更多
OBJECTIVE： To explore the effect of kidney-rein- forcing, blood-activating and stasis-removing recipes on adhesion molecule expression of bone mar- row mesenchymal stem cells （MSCs） from patients with chronic aplas...OBJECTIVE： To explore the effect of kidney-rein- forcing, blood-activating and stasis-removing recipes on adhesion molecule expression of bone mar- row mesenchymal stem cells （MSCs） from patients with chronic aplastic anemia （CAA）. METHODS： We used three Traditional Chinese Medicine recipes, namely a kidney-reinforcing recipe （KRR）, blood-activating and stasis-removing recipe （BASRR）, and kidney-reinforcing, blood-activating and stasis-removing recipe （KRBASFIR）, and a nor- mal saline control to prepare herbal medicine se- rum in Sprague Dawley rats. Thirty CAA patients were enrolled in the experimental group, including 17 kidney-Yang deficient patients and 13 kidney-Yin deficient patients. Ten healthy individuals were included in the control group. MSCs were isolated from bone marrow samples, and the cell density was observed to measure their proliferation ability by microscopy on days 2, 7, and 14 after isolation. In addition, the expression of adhesion molecules of bone marrow MSCs （CD106, CD49d, CD31 and CD44） were detected by flow cytometry after 48 h of treatment with the four different herbal medi- cine serums. RESULTS： The proliferation of MSCs from kid- ney-Yang deficient and kidney-Yin deficient pa- tients was weaker than that of MSCs from the con- trol group. The expression of all adhesion mole- cules of bone marrow MSCs from CAA patients was obviously lower than that in the control group （P〈 0.01）. The expression of CD49d and CD31 in MSCs from patients with a kidney-Yin deficiency was low- er than in those with a kidney-yang deficiency （P〈 0.05 and P〈O.01, respectively）. For kidney-Yang defi- cient patients, CD31 expression in the KRBASRR group was significantly higher than that in the BASRR group （P〈O.01）, while CD44 in the KRBASRR group was significantly higher than that in both KRR and BASRR groups （P〈O.01）. For kidney-Yin defi- cient patients, CD106 and CD49d expression in the KRBASRR group was obviously higher than that in the KRR group （P〈0.05）, while CD31 and CD44 ex- pression in the KRBASRR group was significantly higher than that in both KRR and BASRR groups （P〈 0.05 and P〈0.01, respectively）. CONCLUSION： The bone marrow microenviron- ment in CAA patients is abnormal. The effect of KRBASRR may be better than that of KRR and BASRR for kidney-Yang deficient and kidney-Yin de- ficient patients by improving the expression levels of MSC adhesion molecules.展开更多
OBJECTIVE:To compare the efficacy of modified treatments based on "kidney reinforcing" in the management of chronic aplastic anemia(CAA),and explore their advantages and specialties.METHODS:One hundred and e...OBJECTIVE:To compare the efficacy of modified treatments based on "kidney reinforcing" in the management of chronic aplastic anemia(CAA),and explore their advantages and specialties.METHODS:One hundred and eleven patients with CAA were randomly divided into three groups:kidney reinforcing alone(KA),"kidney reinforcing and Qi tonifying"(KQ),and "kidney reinforcing and blood circulation invigorating"(KC).Normal and positive control groups were also formed.All patients were treated for 6 months(two courses).Hemograms,Traditional Chinese Medicine(TCM) syndrome scores,and therapeutic effects were assessed,and changes in T-lymphocyte subsets,regulatory T cells and cytokines were detected.RESULTS:The KQ and KC groups had lower TCM syndrome scores than the positive control group after 6 months(P < 0.05).The KQ group had a higher overall efficacy than the positive control group after 3 months(P < 0.05),while platelet counts increased in the KC group after 6 months(P < 0.05).CD3+ T-lymphocyte ratios decreased only in the KQ group,while CD3 + CD4 + CD8-Tlymphocytes increased only in the KC group after 6 months(P <0.05).Levels of interferon-γ,tumor necrosis fac-tor-α,interleukin(IL)-2 and IL-6 decreased and levels of IL-4 and IL-10 increased in all treated groups after 6 months.Levels of IL-6 in the KQ and KC groups were lower than those in the positive control group(P < 0.05).CONCLUSION:Treatments based on kidney reinforcing have a rebalancing effect on cytotoxic and T helper cells,and regulate expression of interferon-γ,IL-2,IL-6 and IL-4.KQ may be more effective in treating CAA,and KC may have an advantage in platelet recovery.展开更多
Objective:To explore the difference of lymphocyte subsets in peripheral blood(PB)between aplastic anemia(AA)and hypoplastic myelodysplastic syndrome(hypo-MDS)patients,meanwhile to compare the clinical parameters obtai...Objective:To explore the difference of lymphocyte subsets in peripheral blood(PB)between aplastic anemia(AA)and hypoplastic myelodysplastic syndrome(hypo-MDS)patients,meanwhile to compare the clinical parameters obtained from PB and bone marrow(BM).Methods:The lymphocyte subsets in hypo-MDS(n=25)and.AA(n=33)patients were investigated by flow cytometry.Meanwhile,the differences in PB cell counts,biochemical indicators,BM cell counts and abnormal chromosomes between the two groups were analyzed.Results:The percentage of CD8^(+)T cells in AA group was significantly higher than that in hypo-MDS group(P=0.001),while the percentage of CD4^(+)T cells and the CD4^(+)/CD8^(+)ratio in AA group were obviously lower than those in hypo-MDS group(P=0.015 and 0.001,respectively).Furthermore,the proportion of CD4^(+)and CD8^(+)activated T(TA)cells,and memory Tregs in AA group was distinctly lower than those in hypo-MDS group(P=0.043,0.015 and 0.024,respectively).Nevertheless,the percentage of CD8^(+)naive T(TN)cells in AA patients was remarkably higher(P=0.044).And hypo-MDS patients had declined lymphocyte counts(P=0.025),increased levels of total bilirubin(TBil),lactate dehydrogenase(LDH),vitamin B12 and proportion of BM blasts than AA patients(P=0.019,0.023,0.027 and.0.045,respectively).Conclusion:In this study it was confirmed that the percentages of CD4^(+)and CD8^(+)TA cells,memory Tregs and CD8^(+)TN cells were significantly different between AA and hypo-MDS patients,which provide an essential basis for the identification of these two diseases.展开更多
Telaprevir and Boceprevir are the first direct acting antivirals approved for chronic hepatitis C in combination with peg-interferon alfa and ribavirin.Pancytopenia due to myelotoxicity caused by these drugs may occur...Telaprevir and Boceprevir are the first direct acting antivirals approved for chronic hepatitis C in combination with peg-interferon alfa and ribavirin.Pancytopenia due to myelotoxicity caused by these drugs may occur,but severe hematological abnormalities or aplastic anemia(AA) have not been described.We collected all cases of severe pancytopenia observed during triple therapy with telaprevir in four Spanish centers since approval of the drug in 2011.Among 142 cirrhotic patients receiving treatment,7 cases of severe pancytopenia(5%) were identified and three were consistent with the diagnosis of AA.Mean age was 59 years,five patients had compensated cirrhosis and two patients had severe hepatitis C recurrence after liver transplantation.Severe pancytopenia was diagnosed a median of 10 wk after the initiation of therapy.Three patients had pre-treatment hematological abnormalities related to splenomegaly.In six patients,antiviral treatment was interrupted at the onset of hematological abnormalities.Two patients died due to septic complications and one patient due to acute alveolar hemorrhage.The remaining patients recovered.Severe pancytopenia and especially AA,are not rare during triple therapy with telaprevir in patients with advanced liver disease.Close monitoring is imperative in this setting to promptly detect serious hematological disorders and to prevent further complications.展开更多
OBJECTIVE:To observe the clinical efficacy of Busuishengxue granules on non-severe aplastic anemia(NSAA)and investigate its effect on the mitogen-activated protein kinase/extracellular signal-regulated kinase(MAPK/ERK...OBJECTIVE:To observe the clinical efficacy of Busuishengxue granules on non-severe aplastic anemia(NSAA)and investigate its effect on the mitogen-activated protein kinase/extracellular signal-regulated kinase(MAPK/ERK)pathway.METHODS:Sixty NSAA patients were divided equally into two groups.Subjects in the experimental group were treated with Busuishengxue granules,and the control group with Zaizaoshengxue tablets.The treatment course was 6 months and cu-rative efficacy was compared between the two groups as well as with 10 healthy individuals.Flow cytometry(FCM)was used to detect the intracellular concentration of Ca2+([Ca2+]i).Western blotting was employed to detect the expression of enzymes in the MAPK/ERK pathway.RESULTS:The efficacy of Busuishengxue granules was significantly better than that of Zaizaoshengxue tablets(P<0.05).Before treatment,expression of JNK,phospho-ERK 1/2 and p-JNK was higher,and[Ca2+]i higher,than that of the control group(P<0.05).After treatment with Busuishengxue granules,expression of all enzymes related to signal transduction pathways in the blood cells of NSSA patients were altered to different degrees.CONCLUSION:Busuishengxue granules had a better effect with regard to improving symptom scores,increasing the number of blood leukocytes,and increasing hemoglobin levels than Zaizaoshengxue tablets,and they differed slightly in terms of increasing the number of platelets.展开更多
OBJECTIVE： To explore the effect of antithymocyte globulin （ATG）/antilymphocyte globulin （ALG） plus kidney-nourishing Chinese medicinal （KNCM） on se- vere aplastic anemia （SAA）. METHODS： Twenty-five subjects...OBJECTIVE： To explore the effect of antithymocyte globulin （ATG）/antilymphocyte globulin （ALG） plus kidney-nourishing Chinese medicinal （KNCM） on se- vere aplastic anemia （SAA）. METHODS： Twenty-five subjects of severe aplastic anemia were treated with ATG/ALD plus KNCM be- tween 1992 and 2009, and the clinical data before and after treatment were collected and analyzed. RESULTS： Of the 25 patients, 9 were nearly cured, 6 were improved, 5 were in remission, and 5 failed. The overall effective rate was 80.0%. The 3-year, 5-year, 10-year, 15-year survival rate were respec- tively 98.6%, 97.3%, 97.3%, 67.5%, and median sur- vival time was 180 months. Compared to the condi- tions before administering the medication of ATG/ ALG plus KNCM, after 2 weeks, reticulocyte was first improved （P=0.001）, one month later, followed by palette （P=0.037）, two months later, by neutrophil cell in peripheral blood （P=0.001）; three months lat- er, then by the hemoglobin （P=0.012）. By conduct- ing 1-year follow-up, 1 case of complication--parox-ysmal nocturnal hemoglobinuria （PNH） was identi- fied and the patient still alive today. CONCLUSION： ATG/ALG fect on SAA and could rate. plus KNCM had better ef- improve patients＇ survival展开更多
Acquired aplastic anemia(AA) is a bone marrow failure syndrome characterized by peripheral cytopenias and bone marrow hypoplasia. It is ultimately fatal without treatment, most commonly from infection or hemorrhage. C...Acquired aplastic anemia(AA) is a bone marrow failure syndrome characterized by peripheral cytopenias and bone marrow hypoplasia. It is ultimately fatal without treatment, most commonly from infection or hemorrhage. Current treatments focus on suppressing immune-mediated destruction of bone marrow stem cells or replacing hematopoietic stem cells(HSCs) by transplantation. Our incomplete understanding of the pathogenesis of AA has limited development of targeted treatment options. Mesenchymal stem cells(MSCs) play a vital role in HSC proliferation; they also modulate immune responses and maintain an environment supportive of hematopoiesis. Some of the observed clinical manifestations of AA can be explained by mesenchymal dysfunction. MSC infusions have been shown to be safe and may offer new approaches for the treatment of this disorder. Indeed, infusions of MSCs may help suppress auto-reactive, T-cell mediated HSC destruction and help restore an environment that supports hematopoiesis. Small pilot studies using MSCs as monotherapy or as adjuncts to HSC transplantation have been attempted as treatments for AA. Here we review the current understanding of the pathogenesis of AA and the function of MSCs, and suggest that MSCs should be a target for further research and clinical trials in this disorder.展开更多
Recent studies indicate that immune-associated aplastic anemia(AA)resembles such autoimmune diseases as insulin-dependent diabetes and chronic autoimmune thyroiditis that belong to organ-specific autoimmune diseases.M...Recent studies indicate that immune-associated aplastic anemia(AA)resembles such autoimmune diseases as insulin-dependent diabetes and chronic autoimmune thyroiditis that belong to organ-specific autoimmune diseases.Many independent investigation groups have successfully isolated the pathopoiesis-associated T cell clone causing hematopoiesis failure with a CD4 phenotype from peripheral blood and bone marrow(BM)in AA patients.In the current study,BM CD4+ T cells were isolated from AA patients and healthy con...展开更多
Objective： To explore the therapy to further elevate the efficacy of the treatment of chronic aplastic anemia （CAA）. Methods： Forty-five patients with CCA were assigned into two groups, the 26 patients in the trea...Objective： To explore the therapy to further elevate the efficacy of the treatment of chronic aplastic anemia （CAA）. Methods： Forty-five patients with CCA were assigned into two groups, the 26 patients in the treated group were treated by Shengxuening （ 生血宁, a Chinese herbal preparation） and cyclosporin A （CsA）, and the 19 patients in the control group were treated with androgen alone, with the therapeutic course lasting for over 3 months. Changes of peripheral blood picture, and the colony productivity of burst forming unit-erythroid （BFU-E）, colony forming unit-erythroid （CFU-E） and colony forming unit-granulocyte macrophage （CFU-GM） in bone marrow were observed before and after 3 months treatment. The amount of erythrocyte and platelet infusion, frequency of infection, condition of hemorrhage and relevant death were also observed. The follow-up study was conducted for over half a year. Results： The total effective rate in the treated group was 84.6 %, which was significantly higher than that in the control group （52.6 %, P〈0.05）. Levels of hemoglobin, reticulocyte, neutrophil and platelet increased after treatment in the treated group, as compared with those before treatment, with significant difference （ P〈0.05）, and the colony productivity of BFU-E, CFU-E and CFU-GM in bone marrow also got significantly increased （ P〈0.01 ）, and showed significant difference from those in the control group （P〈0.05）. Conclusion： Shengxuening-assisting CsA therapy is an effective measure for treatment of CAA.展开更多
Objective: To observe the effect of Busui Shengxue Granule (补髓生血颗粒Herbal granule for replenishing marrow to produce blood) on chronic aplastic anemia (CAA) patients’ integrin α 6 (VLA-6/CD49f) and laminin (Ln)...Objective: To observe the effect of Busui Shengxue Granule (补髓生血颗粒Herbal granule for replenishing marrow to produce blood) on chronic aplastic anemia (CAA) patients’ integrin α 6 (VLA-6/CD49f) and laminin (Ln). Methods: Sixty-five patients were divided into experimental group and control group through random number table. There were 34 patients, 17 were male and 17 female, aged 2–67, with a medianage of 30.2 ± 8.6, in the experimental group, including 17 patients of kidney-yin deficiency and 17 of kidney-yang deficiency, treated by Busui Shengxue Granule. There were 31 patients in the control group,16 were male and 15 female, aged 4–65, with a medianage of 31.2 ± 8.0; administered Zaizhang Shengxue Tablet (再障生血片Herbal tablet for chronic aplastic anemia). Both groups were treated for six months and compared with 10 normal persons after the treatment. Flow cytometry was adopted to detect the change in the expression of VLA-6/CD49f, receptor in mononuclear cells of CAA patients and normal persons. Enzyme-linked immunosorbent assay was applied to detect the expression of peripheral serum Ln. Results: CAA patients’ VLA-6/CD49f was in the state of low expression and Ln in the state of high expression. After the treatment, both VLA-6/CD49f and Ln were regulated to some extent and the change in the experimental group was better than that of the control group. Compared with the kidney-yin deficiency patients, those indices of kidney-yang deficiency patients were easier to correct. Conclusion: The VLA-6/CD49f and Ln expressions of CAA patients are abnormal. The treatment with Busui Shengxue Granule makes both of them improved.展开更多
In order to investigate the levels of stem cell factor (SCF) and its receptor c-kit protein and mRNA in pediatric aplastic anemia (AA) and their relevance to the pathogenesis, immunocytochemical and in situ hybridizat...In order to investigate the levels of stem cell factor (SCF) and its receptor c-kit protein and mRNA in pediatric aplastic anemia (AA) and their relevance to the pathogenesis, immunocytochemical and in situ hybridization were utilized to detect the expression of SCF and its receptor c-kit gene protein and mRNA, respectively in 59 children with AA and 51 normal controls. The relationship between SCF and c-kit and the pathogenesis of AA was analyzed subsequently. The results showed that the positive rate of SCF protein and mRNA expression in children with AA was significantly lower than that in healthy controls (P<0.05). However, there was no significant difference in the positive rate of c-kit protein and mRNA expression between children with AA and control group (P>0.05). It was concluded that the expression of SCF is significantly decreased in children with AA, which may be closely associated with the pathogenesis of the AA. c-kit may be unrelated to the development of pediatric AA. Therefore, AA in children may have abnormalities at SCF/c-kit signal transduction levels.展开更多
Rationale:Trichosporon,an anamorphic fungus,proliferates under high humidity,causing serious opportunistic infections collectively called trichosporonosis.Among the Trichosporon species causing trichosporonosis are Tr...Rationale:Trichosporon,an anamorphic fungus,proliferates under high humidity,causing serious opportunistic infections collectively called trichosporonosis.Among the Trichosporon species causing trichosporonosis are Trichosporon(T.)asahii,T.asteroides,T.cutaneum etc.Patient concerns:A 38-year-old Chinese male with severe aplastic anemia was admitted due to multiple joints pain,poor appetite,and right ankle swelling.One year earlier he had undergone allogeneic hematopoietic stem cell transplantation.Diagnosis:T.asahii infection and severe aplastic anemia.Interventions:Combined treatment of amphotericin B liposomes(55 mg/24 h)and voriconazole(200 mg/12 h)for 8 days.Outcomes:The symptoms of the patient’s ankle were relieved and effusion cultures showed no T.asahii.Lessons:To the best of our knowledge,T.asahii ankle cavity effusion infections are rare.Trichosporon infections may be attributed to risk factors such as improper long-term use of antimicrobials for an underlying disease(e.g.,anemia,hypoalbuminemia).Attention should be paid to prevent and control Trichosporon infections in order to avoid comorbidities.展开更多
BACKGROUND Aplastic anemia(AA)presents a significant clinical challenge as a life-threatening condition due to failure to produce essential blood cells,with the current the-rapeutic options being notably limited.AIM T...BACKGROUND Aplastic anemia(AA)presents a significant clinical challenge as a life-threatening condition due to failure to produce essential blood cells,with the current the-rapeutic options being notably limited.AIM To assess the therapeutic potential of ginsenoside Rg1 on AA,specifically its protective effects,while elucidating the mechanism at play.METHODS We employed a model of myelosuppression induced by cyclophosphamide(CTX)in C57 mice,followed by administration of ginsenoside Rg1 over 13 d.The invest-igation included examining the bone marrow,thymus and spleen for pathological changes via hematoxylin-eosin staining.Moreover,orbital blood of mice was collected for blood routine examinations.Flow cytometry was employed to identify the impact of ginsenoside Rg1 on cell apoptosis and cycle in the bone marrow of AA mice.Additionally,the study further evaluated cytokine levels with enzyme-linked immunosorbent assay and analyzed the expression of key proteins in the MAPK signaling pathway via western blot.RESULTS Administration of CTX led to significant damage to the bone marrow’s structural integrity and a reduction in hematopoietic cells,establishing a model of AA.Ginsenoside Rg1 successfully reversed hematopoietic dysfunction in AA mice.In comparison to the AA group,ginsenoside Rg1 provided relief by reducing the induction of cell apoptosis and inflammation factors caused by CTX.Furthermore,it helped alleviate the blockade in the cell cycle.Treatment with ginsenoside Rg1 significantly alleviated myelosuppression in mice by inhibiting the MAPK signaling pathway.CONCLUSION This study suggested that ginsenoside Rg1 addresses AA by alleviating myelosuppression,primarily through modulating the MAPK signaling pathway,which paves the way for a novel therapeutic strategy in treating AA,highlighting the potential of ginsenoside Rg1 as a beneficial intervention.展开更多
BACKGROUND Cumulative evidence suggests that the aberrant immune responses in acquired aplastic anemia(AA) are sustained by active chronic infections in genetically susceptible individuals. Recently, the constant sour...BACKGROUND Cumulative evidence suggests that the aberrant immune responses in acquired aplastic anemia(AA) are sustained by active chronic infections in genetically susceptible individuals. Recently, the constant source to trigger and sustain the pathophysiology has been proposed to come from the altered gut microbiota and chronic intestinal inflammation. In this case, our serendipitous finding provides convincing evidence that the persistently dysregulated autoimmunity may be generated, at least in a significant proposition of AA patients, by the altered gut microbiota and compromised intestinal epithelium.CASE SUMMARY A 30-year-old Chinese male patient with refractory severe AA experienced a 3-month-long febrile episode, and his fever was refractory to many kinds of injected broad-spectrum antibiotics. When presenting with abdominal cramps, he was prescribed oral mannitol and gentamycin to get rid of the gut infection. This treatment resulted in a quick resolution of the fever. Unanticipatedly, it also produced an excellent hematological response. He had undergone three episodes of recurrence within the one-year treatment, with each recurrence occurring 7-8 wk from the gastrointestinal inflammation eliminating preparations. However,subsequent treatments were able to produce subsequent remissions and consecutive treatments were successful in achieving durative hematological improvements, strongly indicating an etiological association between chronic gut inflammation and the development of AA. Interestingly, comorbid diseases superimposed on this patient(namely, psychiatric disorders, hypertension,insulin resistance, and renal dysfunction) were ameliorated together with the hematological improvements.CONCLUSION Chronic gut inflammation may be responsible for AA pathogenesis. The comorbidities and AA may share a common etiological association.展开更多
Distinguishing between aplastic anemia(AA)and hypoblastic myelodysplastic syndrome(hMDS)with a low percentage of bone marrow(BM)blasts(<5%)can be difficult due to the overlap in clonality and a spectrum of genetic ...Distinguishing between aplastic anemia(AA)and hypoblastic myelodysplastic syndrome(hMDS)with a low percentage of bone marrow(BM)blasts(<5%)can be difficult due to the overlap in clonality and a spectrum of genetic alternations between the two subtypes of diseases.However,due to recent advances in DNA sequencing technology,both spectnim and frequency of mutations can be accurately determined and monitored by next-generation sequencing(NGS)at initial diagnosis and during immunosuppressive therapy(1ST)in patients with AA or hMDS.This improvement in acquiring a patient's genetic status and clonal evolution can provide more proper,precise,and on-time information to guide disease management,which is especially helpful in the absence of traditional morphologic/cytogenetic evidence.展开更多
The content of 12 trace elements in the hair of 20 aplastic anemia patients was determined and compared with that of normal subjects as control. The results showed that patients with deficiency of yin had a significan...The content of 12 trace elements in the hair of 20 aplastic anemia patients was determined and compared with that of normal subjects as control. The results showed that patients with deficiency of yin had a significant decrease in lithium, calcium, strontium, and chromium, those with deficiency of yang had a distinct decrease in zinc magnesium barium, strontium, calcium, and lithium, and those with deficiency of both yin and yang had a general decrease in all the 12 trace elements. Changes in trace element content in hair may serve as a guide to opening up new vistas in the treatment of aplastic anemia on the basis of an overall analysis of symptoms and signs.展开更多
文摘Objective Acquired aplastic anemia(aAA)is characterized by an autologous immunological attack against hematopoietic stem and progenitor cells,and immunotolerance disruption is frequent,with reduced T regulatory cells(Tregs)frequencies and increased effector cytotoxic cells.Tregs are reduced in aAA and increase in number after successful immunosuppressive therapies.Methods In this retrospective study,we investigated the frequency of circulating Tregs by multiparametric flow cytometry immunophenotyping in non-severe aAA patients before and after immunosuppressive therapy.The samples were stained with the following antibodies:ECD anti-CD3,PE or PC5 anti-CD4,FITC anti-CD8,and PE anti-CD25,and Tregs were identified by first gating on linear parameters for lymphocyte identification and then for CD3 expression.In CD3+CD4+cells,Tregs were further identified on the basis of CD25 and FOXP3 expression.Results Although the number of Tregs tended to increase after immunosuppressive treatments,their circulating frequency remained lower than that of healthy subjects,regardless of their responsiveness to therapies.Moreover,the relative frequency combined with absolute Treg counts might be more informative in the differential diagnosis of bone marrow failure syndromes.Conclusions The persistent decrease in circulating Tregs could be the result of immunosuppressive agents that could preferentially expand other T-cell subsets.At the same time,an imbalance in immunotolerance might persist,which is also favored by chronic antigen stimulation.
基金Supported by the State Administration of Traditional Chinese Medicine high-level key disciplines construction project:zyyzdxk-2023068Three-year Action Plan for Shanghai TCM Development and Inheritance Program:ZY(2021-2023)-0302。
文摘Objective:To observe the effects of moxibustion on the hematopoietic function in mice with aplastic anemia(AA)induced by bone marrow(BM)suppression,and to investigate the intervention effects of moxibustion on AA from the perspective of intestinal bacteria.Methods:A total of 24 C57BL/6 J male mice were randomly and evenly divided into control,model and moxibustion groups.The myelosuppression-induced AA model was established by cyclophosphamide(CTX)and cyclosporine(Cs)intraperitoneal injection.Mice in the moxibustion group were intervened in mild moxibustion at unilateral“Zusanli(ST36)”acupoint for 15 min per day,and the sides were switched the next day.The intervention of mild moxibustion lasted 60 days consecutively.The red blood cell(RBC),white blood cell(WBC),platelet(PLT)counts and haemoglobin(Hb)concentration levels of mice in each group were detected by peripheral blood cells count staining,and the BM hematopoietic cells and hematopoietic structures were observed by BM smear Wright-Giemsa staining and HE staining.16SrDNA sequencing was used to analyze the gut bacterial species abundance and diversity in mice from each group.Results:After all the intervention,compared to the control group,the model group had lower levels of RBC,WBC,PLT counts and Hb concentration in peripheral blood(P<0.05)and fewer hematopoietic cells and hematopoietic structures;compared to the model group,moxibustion group had higher levels of RBC,WBC,PLT counts and Hb concentration in peripheral blood(P<0.05),and more BM hematopoietic cells and hematopoietic structures.Gut flora showed that moxibustion increased the species richness and diversity of intestinal bacteria in mice;compared with the control group,the relative abundance of Faecalibaculum and Anaeroplasmataceae in the model group was higher(P<0.05);whereas,the relative abundance of Faecalibaculum and Anaeroplasmataceae in the moxibustion group was lower(P<0.05)when compared with the model group.In addition,Faecalibaculum was significantly correlated with RBC,WBC,PLT count and Hb concentration(P<0.05).Conclusion:Moxibustion can improved BM histology,restored hematopoietic cells function,and increased peripheral blood cells count and Hb concentration in AA mice.The mechanism may be related to the fact that moxibustion regulates the abundance of specific intestinal bacteria to maintain the stability of the flora structure.
基金Supported by Zhejiang Province Traditional Chinese Medicine Science and Technology Plan Project:Research on the Mechanism of Shisiwei Jianzhong Decoction in Treating Non-severe Aplastic Anemia with Kidney Yang Deficiency Based on the C Vactivated T Nuclear Factor(NFATc4)(2020ZB086)Zhejiang Province Traditional Chinese Medicine Science and Technology Youth Talent Plan Project:Study on the Academic thought and Clinical Application of Professor Zhou Yuhong in the Treatment of Chronic Aplastic Anemia(2021ZQ029)。
文摘OBJECTIVE:To investigate the effect of Shisiwei Jianzhong decoction(十四味建中汤,SJD)on non-severe aplastic anemia(NSAA).METHODS:Bone marrow mesenchymal stem cells(BMSCs)were isolated from bone marrow samples of 15 NSAA patients and 3 healthy controls.Cells were treated with gradient concentrations of SJD,and a portion was transfected with a vector overexpressing the nuclear factor of activated T cells,cytoplasmic 4(NFATC4).Cell viability and apoptosis were detected by cell counting kit-8 and flow cytometry,respectively.After adipogenic differentiation induction,lipid droplet formation in BMSCs was examined by Oil Red O staining.The expression of NFATC4,peroxisome proliferator-activated receptor gamma(PPARG),fatty acid-binding protein 4(FABP4),peroxisome proliferator-activated receptor-gamma coactivator(PGC-1α),and acetylated PGC-1αwas measured by quantitative real-time polymerase chain reaction or Western blot.RESULTS:SJD significantly increased the viability and decreased the apoptosis of NSAA-derived BMSCs.It also dose-dependently inhibited lipid droplet formation and decreased the expression of PPARG and FABP4 in NSAA-derived BMSCs.NFATC4 expression was higher in patients with NSAA than in healthy controls,and SJD downregulated its expression.NFATC4 overexpression reversed the inhibitory effect of SJD on adipogenic differentiation.Additionally,SJD promoted the deacetylation of PGC-1αin NSAA-derived BMSCs,which was also partially eliminated by NFATC4 overexpression.CONCLUSIONS:SJD inhibits adipogenic differentiation of BMSCs through downregulating NFATC4,thereby contributing to the remission of NSAA.
文摘To investigate the efficacy of a structured continuous nursing intervention program on clinical outcomes,self-efficacy,and sleep quality in patients diagnosed with aplastic anemia(AA).Methods:This study was a single-center,parallel-group,randomized controlled trial.A total of 64 patients with AA,treated at our hospital’s hematology department from May 2022 to May 2025,were enrolled.Participants were randomly allocated in a 1:1 ratio to either the control group(n=32),receiving routine nursing care,or the intervention group(n=32),receiving a six-month continuous nursing intervention program in addition to routine care.The intervention program consisted of structured health education,individualized psychological support,regular telephone follow-ups,and a 24/7 online communication platform.The primary outcome was the change in the Pittsburgh Sleep Quality Index(PSQI)score from baseline to six months.Secondary outcomes included hematological parameters(hemoglobin[Hb],absolute neutrophil count[ANC],platelet count[PLT]),incidence of adverse events(infections,bleeding episodes),quality of life(assessed by the Functional Assessment of Cancer Therapy–Anemia[FACT-An]),and self-efficacy(assessed by the General Self-Efficacy Scale[GSE]).Statistical analyses were performed using independent t-tests,and chi-square tests or Fisher’s exact tests as appropriate.Results:A total of 55 patients(28 in the intervention group and 27 in the control group)completed the study.At baseline,there were no statistically significant differences in demographic data,clinical characteristics,or outcome measures between the two groups(p>0.05).After six months,the mean PSQI score in the intervention group was significantly lower than that in the control group(7.12±2.05 vs.13.49±2.88;t=−13.450;p<0.001).The intervention group also demonstrated a significantly lower incidence of infections(4/28,14.29%vs.10/27,37.04%;p=0.048)and bleeding episodes requiring intervention(3/28,10.71%vs.9/27,33.33%;p=0.042).Furthermore,patients in the intervention group reported significantly higher scores on the FACT-An(125.70±10.31 vs.109.44±12.10;t=7.934;p<0.001)and the GSE scale(29.82±4.11 vs.23.51±4.80;t=7.311;p<0.001).While hematological parameters showed a trend toward improvement in the intervention group,the differences were not statistically significant compared to the control group(p>0.05).Conclusion:The implementation of a structured continuous nursing intervention program can significantly improve sleep quality,reduce the incidence of complications,and enhance quality of life and self-efficacy in patients with aplastic anemia.This evidence-based model of care should be considered for integration into standard clinical practice for the long-term management of this patient population.
基金Supported by 2011 Zhejiang province key science and technology innovation team(No.2011R09042-02)Special Item of Important Disease of Zhejiang Province TCM Sci-Tech Innovation Platform(No.2009ZDJB01,2009ZDJB01-08)
文摘OBJECTIVE： To explore the effect of kidney-rein- forcing, blood-activating and stasis-removing recipes on adhesion molecule expression of bone mar- row mesenchymal stem cells （MSCs） from patients with chronic aplastic anemia （CAA）. METHODS： We used three Traditional Chinese Medicine recipes, namely a kidney-reinforcing recipe （KRR）, blood-activating and stasis-removing recipe （BASRR）, and kidney-reinforcing, blood-activating and stasis-removing recipe （KRBASFIR）, and a nor- mal saline control to prepare herbal medicine se- rum in Sprague Dawley rats. Thirty CAA patients were enrolled in the experimental group, including 17 kidney-Yang deficient patients and 13 kidney-Yin deficient patients. Ten healthy individuals were included in the control group. MSCs were isolated from bone marrow samples, and the cell density was observed to measure their proliferation ability by microscopy on days 2, 7, and 14 after isolation. In addition, the expression of adhesion molecules of bone marrow MSCs （CD106, CD49d, CD31 and CD44） were detected by flow cytometry after 48 h of treatment with the four different herbal medi- cine serums. RESULTS： The proliferation of MSCs from kid- ney-Yang deficient and kidney-Yin deficient pa- tients was weaker than that of MSCs from the con- trol group. The expression of all adhesion mole- cules of bone marrow MSCs from CAA patients was obviously lower than that in the control group （P〈 0.01）. The expression of CD49d and CD31 in MSCs from patients with a kidney-Yin deficiency was low- er than in those with a kidney-yang deficiency （P〈 0.05 and P〈O.01, respectively）. For kidney-Yang defi- cient patients, CD31 expression in the KRBASRR group was significantly higher than that in the BASRR group （P〈O.01）, while CD44 in the KRBASRR group was significantly higher than that in both KRR and BASRR groups （P〈O.01）. For kidney-Yin defi- cient patients, CD106 and CD49d expression in the KRBASRR group was obviously higher than that in the KRR group （P〈0.05）, while CD31 and CD44 ex- pression in the KRBASRR group was significantly higher than that in both KRR and BASRR groups （P〈 0.05 and P〈0.01, respectively）. CONCLUSION： The bone marrow microenviron- ment in CAA patients is abnormal. The effect of KRBASRR may be better than that of KRR and BASRR for kidney-Yang deficient and kidney-Yin de- ficient patients by improving the expression levels of MSC adhesion molecules.
基金Supported by Special Item of Important Disease of Zhejiang Province Traditional Chinese Medicine Sic-Tech Innovation Platform(Effect and Mechanism of Traditional Chinese Medicine on the Treatment of Aplastic Anemia and the Funding of Traditional Chinese Medicine Assessment Criterion,No.2009ZDJB01)Subject of Key Sic-Tech Innovation Team of Zhejiang Province(Clinical Study on Treatment of Chronic Aplastic Anemia by Tonifying Kidney and Promoting Blood Circulation,No.2011R09042-02)+2 种基金Special Research Funds for Traditional Chinese Medicine Industry(Effect of Traditional Chinese Medicine on the Treatment of Risk Factors of Chronic Aplastic Anemia,No.201107001)Special Research Funds for Traditional Chinese Medicine Industry(Clinical Study on the Diagnosis and Treatment of Aplastic Anemia Based on the Syndrome And Stage Differentiation,No.201407001)Zhejiang Provincial Traditional Chinese Medicine Administration Bureau Program(Establishment of Traditional Chinese Medicine Clinical Pathway on Aplastic Anemia,No.2010ZA039)
文摘OBJECTIVE:To compare the efficacy of modified treatments based on "kidney reinforcing" in the management of chronic aplastic anemia(CAA),and explore their advantages and specialties.METHODS:One hundred and eleven patients with CAA were randomly divided into three groups:kidney reinforcing alone(KA),"kidney reinforcing and Qi tonifying"(KQ),and "kidney reinforcing and blood circulation invigorating"(KC).Normal and positive control groups were also formed.All patients were treated for 6 months(two courses).Hemograms,Traditional Chinese Medicine(TCM) syndrome scores,and therapeutic effects were assessed,and changes in T-lymphocyte subsets,regulatory T cells and cytokines were detected.RESULTS:The KQ and KC groups had lower TCM syndrome scores than the positive control group after 6 months(P < 0.05).The KQ group had a higher overall efficacy than the positive control group after 3 months(P < 0.05),while platelet counts increased in the KC group after 6 months(P < 0.05).CD3+ T-lymphocyte ratios decreased only in the KQ group,while CD3 + CD4 + CD8-Tlymphocytes increased only in the KC group after 6 months(P <0.05).Levels of interferon-γ,tumor necrosis fac-tor-α,interleukin(IL)-2 and IL-6 decreased and levels of IL-4 and IL-10 increased in all treated groups after 6 months.Levels of IL-6 in the KQ and KC groups were lower than those in the positive control group(P < 0.05).CONCLUSION:Treatments based on kidney reinforcing have a rebalancing effect on cytotoxic and T helper cells,and regulate expression of interferon-γ,IL-2,IL-6 and IL-4.KQ may be more effective in treating CAA,and KC may have an advantage in platelet recovery.
文摘Objective:To explore the difference of lymphocyte subsets in peripheral blood(PB)between aplastic anemia(AA)and hypoplastic myelodysplastic syndrome(hypo-MDS)patients,meanwhile to compare the clinical parameters obtained from PB and bone marrow(BM).Methods:The lymphocyte subsets in hypo-MDS(n=25)and.AA(n=33)patients were investigated by flow cytometry.Meanwhile,the differences in PB cell counts,biochemical indicators,BM cell counts and abnormal chromosomes between the two groups were analyzed.Results:The percentage of CD8^(+)T cells in AA group was significantly higher than that in hypo-MDS group(P=0.001),while the percentage of CD4^(+)T cells and the CD4^(+)/CD8^(+)ratio in AA group were obviously lower than those in hypo-MDS group(P=0.015 and 0.001,respectively).Furthermore,the proportion of CD4^(+)and CD8^(+)activated T(TA)cells,and memory Tregs in AA group was distinctly lower than those in hypo-MDS group(P=0.043,0.015 and 0.024,respectively).Nevertheless,the percentage of CD8^(+)naive T(TN)cells in AA patients was remarkably higher(P=0.044).And hypo-MDS patients had declined lymphocyte counts(P=0.025),increased levels of total bilirubin(TBil),lactate dehydrogenase(LDH),vitamin B12 and proportion of BM blasts than AA patients(P=0.019,0.023,0.027 and.0.045,respectively).Conclusion:In this study it was confirmed that the percentages of CD4^(+)and CD8^(+)TA cells,memory Tregs and CD8^(+)TN cells were significantly different between AA and hypo-MDS patients,which provide an essential basis for the identification of these two diseases.
基金(in part)Instituto de Salud Carlos III(PI11/01907),Ministerio de Economia y Competitividad,co-funded by Fondo Europeo de Desarrollo Regional,Union Europea,Una manera de hacer EuropaRoche Organ Transplantation Research Foundation(ROTRF,CI:442035057)(all to Forns X)
文摘Telaprevir and Boceprevir are the first direct acting antivirals approved for chronic hepatitis C in combination with peg-interferon alfa and ribavirin.Pancytopenia due to myelotoxicity caused by these drugs may occur,but severe hematological abnormalities or aplastic anemia(AA) have not been described.We collected all cases of severe pancytopenia observed during triple therapy with telaprevir in four Spanish centers since approval of the drug in 2011.Among 142 cirrhotic patients receiving treatment,7 cases of severe pancytopenia(5%) were identified and three were consistent with the diagnosis of AA.Mean age was 59 years,five patients had compensated cirrhosis and two patients had severe hepatitis C recurrence after liver transplantation.Severe pancytopenia was diagnosed a median of 10 wk after the initiation of therapy.Three patients had pre-treatment hematological abnormalities related to splenomegaly.In six patients,antiviral treatment was interrupted at the onset of hematological abnormalities.Two patients died due to septic complications and one patient due to acute alveolar hemorrhage.The remaining patients recovered.Severe pancytopenia and especially AA,are not rare during triple therapy with telaprevir in patients with advanced liver disease.Close monitoring is imperative in this setting to promptly detect serious hematological disorders and to prevent further complications.
基金Supported by the National Natural Science Foundation of China(No.81202680)Specialized Research Fund for the Doctoral Program of Higher Education(No.200802280003,20092327120001)+2 种基金China Postdoctoral Science Foundation(20100481034)Heilongjiang administration of Traditional Chinese Medicine Foundation(ZHYO-W42)Heilongjiang University of Chinese Medicine Foundation(No.200901)
文摘OBJECTIVE:To observe the clinical efficacy of Busuishengxue granules on non-severe aplastic anemia(NSAA)and investigate its effect on the mitogen-activated protein kinase/extracellular signal-regulated kinase(MAPK/ERK)pathway.METHODS:Sixty NSAA patients were divided equally into two groups.Subjects in the experimental group were treated with Busuishengxue granules,and the control group with Zaizaoshengxue tablets.The treatment course was 6 months and cu-rative efficacy was compared between the two groups as well as with 10 healthy individuals.Flow cytometry(FCM)was used to detect the intracellular concentration of Ca2+([Ca2+]i).Western blotting was employed to detect the expression of enzymes in the MAPK/ERK pathway.RESULTS:The efficacy of Busuishengxue granules was significantly better than that of Zaizaoshengxue tablets(P<0.05).Before treatment,expression of JNK,phospho-ERK 1/2 and p-JNK was higher,and[Ca2+]i higher,than that of the control group(P<0.05).After treatment with Busuishengxue granules,expression of all enzymes related to signal transduction pathways in the blood cells of NSSA patients were altered to different degrees.CONCLUSION:Busuishengxue granules had a better effect with regard to improving symptom scores,increasing the number of blood leukocytes,and increasing hemoglobin levels than Zaizaoshengxue tablets,and they differed slightly in terms of increasing the number of platelets.
文摘OBJECTIVE： To explore the effect of antithymocyte globulin （ATG）/antilymphocyte globulin （ALG） plus kidney-nourishing Chinese medicinal （KNCM） on se- vere aplastic anemia （SAA）. METHODS： Twenty-five subjects of severe aplastic anemia were treated with ATG/ALD plus KNCM be- tween 1992 and 2009, and the clinical data before and after treatment were collected and analyzed. RESULTS： Of the 25 patients, 9 were nearly cured, 6 were improved, 5 were in remission, and 5 failed. The overall effective rate was 80.0%. The 3-year, 5-year, 10-year, 15-year survival rate were respec- tively 98.6%, 97.3%, 97.3%, 67.5%, and median sur- vival time was 180 months. Compared to the condi- tions before administering the medication of ATG/ ALG plus KNCM, after 2 weeks, reticulocyte was first improved （P=0.001）, one month later, followed by palette （P=0.037）, two months later, by neutrophil cell in peripheral blood （P=0.001）; three months lat- er, then by the hemoglobin （P=0.012）. By conduct- ing 1-year follow-up, 1 case of complication--parox-ysmal nocturnal hemoglobinuria （PNH） was identi- fied and the patient still alive today. CONCLUSION： ATG/ALG fect on SAA and could rate. plus KNCM had better ef- improve patients＇ survival
基金Supported by National Center for Advancing Translational Sciences,National Institutes of Health,through Grant Nos.UL1TR001436 and 1TL1TR001437(to Broglie L)MACC Fund(to Margolis D and Medin JA)
文摘Acquired aplastic anemia(AA) is a bone marrow failure syndrome characterized by peripheral cytopenias and bone marrow hypoplasia. It is ultimately fatal without treatment, most commonly from infection or hemorrhage. Current treatments focus on suppressing immune-mediated destruction of bone marrow stem cells or replacing hematopoietic stem cells(HSCs) by transplantation. Our incomplete understanding of the pathogenesis of AA has limited development of targeted treatment options. Mesenchymal stem cells(MSCs) play a vital role in HSC proliferation; they also modulate immune responses and maintain an environment supportive of hematopoiesis. Some of the observed clinical manifestations of AA can be explained by mesenchymal dysfunction. MSC infusions have been shown to be safe and may offer new approaches for the treatment of this disorder. Indeed, infusions of MSCs may help suppress auto-reactive, T-cell mediated HSC destruction and help restore an environment that supports hematopoiesis. Small pilot studies using MSCs as monotherapy or as adjuncts to HSC transplantation have been attempted as treatments for AA. Here we review the current understanding of the pathogenesis of AA and the function of MSCs, and suggest that MSCs should be a target for further research and clinical trials in this disorder.
基金supported by the Specialized Research Fund for the Doctoral Program of Higher Education of China(No.200804871044)
文摘Recent studies indicate that immune-associated aplastic anemia(AA)resembles such autoimmune diseases as insulin-dependent diabetes and chronic autoimmune thyroiditis that belong to organ-specific autoimmune diseases.Many independent investigation groups have successfully isolated the pathopoiesis-associated T cell clone causing hematopoiesis failure with a CD4 phenotype from peripheral blood and bone marrow(BM)in AA patients.In the current study,BM CD4+ T cells were isolated from AA patients and healthy con...
文摘Objective： To explore the therapy to further elevate the efficacy of the treatment of chronic aplastic anemia （CAA）. Methods： Forty-five patients with CCA were assigned into two groups, the 26 patients in the treated group were treated by Shengxuening （ 生血宁, a Chinese herbal preparation） and cyclosporin A （CsA）, and the 19 patients in the control group were treated with androgen alone, with the therapeutic course lasting for over 3 months. Changes of peripheral blood picture, and the colony productivity of burst forming unit-erythroid （BFU-E）, colony forming unit-erythroid （CFU-E） and colony forming unit-granulocyte macrophage （CFU-GM） in bone marrow were observed before and after 3 months treatment. The amount of erythrocyte and platelet infusion, frequency of infection, condition of hemorrhage and relevant death were also observed. The follow-up study was conducted for over half a year. Results： The total effective rate in the treated group was 84.6 %, which was significantly higher than that in the control group （52.6 %, P〈0.05）. Levels of hemoglobin, reticulocyte, neutrophil and platelet increased after treatment in the treated group, as compared with those before treatment, with significant difference （ P〈0.05）, and the colony productivity of BFU-E, CFU-E and CFU-GM in bone marrow also got significantly increased （ P〈0.01 ）, and showed significant difference from those in the control group （P〈0.05）. Conclusion： Shengxuening-assisting CsA therapy is an effective measure for treatment of CAA.
基金supported by the the National Natural Science Foundation of China (No.90709039)Specialized Research Fund for the Doctoral Program of Higher Education (No.200802280003, No.20092327120001)Heilongjiang Provincial Natural Science Foundation of China (No.D2005-62)
文摘Objective: To observe the effect of Busui Shengxue Granule (补髓生血颗粒Herbal granule for replenishing marrow to produce blood) on chronic aplastic anemia (CAA) patients’ integrin α 6 (VLA-6/CD49f) and laminin (Ln). Methods: Sixty-five patients were divided into experimental group and control group through random number table. There were 34 patients, 17 were male and 17 female, aged 2–67, with a medianage of 30.2 ± 8.6, in the experimental group, including 17 patients of kidney-yin deficiency and 17 of kidney-yang deficiency, treated by Busui Shengxue Granule. There were 31 patients in the control group,16 were male and 15 female, aged 4–65, with a medianage of 31.2 ± 8.0; administered Zaizhang Shengxue Tablet (再障生血片Herbal tablet for chronic aplastic anemia). Both groups were treated for six months and compared with 10 normal persons after the treatment. Flow cytometry was adopted to detect the change in the expression of VLA-6/CD49f, receptor in mononuclear cells of CAA patients and normal persons. Enzyme-linked immunosorbent assay was applied to detect the expression of peripheral serum Ln. Results: CAA patients’ VLA-6/CD49f was in the state of low expression and Ln in the state of high expression. After the treatment, both VLA-6/CD49f and Ln were regulated to some extent and the change in the experimental group was better than that of the control group. Compared with the kidney-yin deficiency patients, those indices of kidney-yang deficiency patients were easier to correct. Conclusion: The VLA-6/CD49f and Ln expressions of CAA patients are abnormal. The treatment with Busui Shengxue Granule makes both of them improved.
文摘In order to investigate the levels of stem cell factor (SCF) and its receptor c-kit protein and mRNA in pediatric aplastic anemia (AA) and their relevance to the pathogenesis, immunocytochemical and in situ hybridization were utilized to detect the expression of SCF and its receptor c-kit gene protein and mRNA, respectively in 59 children with AA and 51 normal controls. The relationship between SCF and c-kit and the pathogenesis of AA was analyzed subsequently. The results showed that the positive rate of SCF protein and mRNA expression in children with AA was significantly lower than that in healthy controls (P<0.05). However, there was no significant difference in the positive rate of c-kit protein and mRNA expression between children with AA and control group (P>0.05). It was concluded that the expression of SCF is significantly decreased in children with AA, which may be closely associated with the pathogenesis of the AA. c-kit may be unrelated to the development of pediatric AA. Therefore, AA in children may have abnormalities at SCF/c-kit signal transduction levels.
文摘Rationale:Trichosporon,an anamorphic fungus,proliferates under high humidity,causing serious opportunistic infections collectively called trichosporonosis.Among the Trichosporon species causing trichosporonosis are Trichosporon(T.)asahii,T.asteroides,T.cutaneum etc.Patient concerns:A 38-year-old Chinese male with severe aplastic anemia was admitted due to multiple joints pain,poor appetite,and right ankle swelling.One year earlier he had undergone allogeneic hematopoietic stem cell transplantation.Diagnosis:T.asahii infection and severe aplastic anemia.Interventions:Combined treatment of amphotericin B liposomes(55 mg/24 h)and voriconazole(200 mg/12 h)for 8 days.Outcomes:The symptoms of the patient’s ankle were relieved and effusion cultures showed no T.asahii.Lessons:To the best of our knowledge,T.asahii ankle cavity effusion infections are rare.Trichosporon infections may be attributed to risk factors such as improper long-term use of antimicrobials for an underlying disease(e.g.,anemia,hypoalbuminemia).Attention should be paid to prevent and control Trichosporon infections in order to avoid comorbidities.
基金Supported by Hangzhou Municipal Bureau of Science and Technology,No.2021WJCY366.
文摘BACKGROUND Aplastic anemia(AA)presents a significant clinical challenge as a life-threatening condition due to failure to produce essential blood cells,with the current the-rapeutic options being notably limited.AIM To assess the therapeutic potential of ginsenoside Rg1 on AA,specifically its protective effects,while elucidating the mechanism at play.METHODS We employed a model of myelosuppression induced by cyclophosphamide(CTX)in C57 mice,followed by administration of ginsenoside Rg1 over 13 d.The invest-igation included examining the bone marrow,thymus and spleen for pathological changes via hematoxylin-eosin staining.Moreover,orbital blood of mice was collected for blood routine examinations.Flow cytometry was employed to identify the impact of ginsenoside Rg1 on cell apoptosis and cycle in the bone marrow of AA mice.Additionally,the study further evaluated cytokine levels with enzyme-linked immunosorbent assay and analyzed the expression of key proteins in the MAPK signaling pathway via western blot.RESULTS Administration of CTX led to significant damage to the bone marrow’s structural integrity and a reduction in hematopoietic cells,establishing a model of AA.Ginsenoside Rg1 successfully reversed hematopoietic dysfunction in AA mice.In comparison to the AA group,ginsenoside Rg1 provided relief by reducing the induction of cell apoptosis and inflammation factors caused by CTX.Furthermore,it helped alleviate the blockade in the cell cycle.Treatment with ginsenoside Rg1 significantly alleviated myelosuppression in mice by inhibiting the MAPK signaling pathway.CONCLUSION This study suggested that ginsenoside Rg1 addresses AA by alleviating myelosuppression,primarily through modulating the MAPK signaling pathway,which paves the way for a novel therapeutic strategy in treating AA,highlighting the potential of ginsenoside Rg1 as a beneficial intervention.
文摘BACKGROUND Cumulative evidence suggests that the aberrant immune responses in acquired aplastic anemia(AA) are sustained by active chronic infections in genetically susceptible individuals. Recently, the constant source to trigger and sustain the pathophysiology has been proposed to come from the altered gut microbiota and chronic intestinal inflammation. In this case, our serendipitous finding provides convincing evidence that the persistently dysregulated autoimmunity may be generated, at least in a significant proposition of AA patients, by the altered gut microbiota and compromised intestinal epithelium.CASE SUMMARY A 30-year-old Chinese male patient with refractory severe AA experienced a 3-month-long febrile episode, and his fever was refractory to many kinds of injected broad-spectrum antibiotics. When presenting with abdominal cramps, he was prescribed oral mannitol and gentamycin to get rid of the gut infection. This treatment resulted in a quick resolution of the fever. Unanticipatedly, it also produced an excellent hematological response. He had undergone three episodes of recurrence within the one-year treatment, with each recurrence occurring 7-8 wk from the gastrointestinal inflammation eliminating preparations. However,subsequent treatments were able to produce subsequent remissions and consecutive treatments were successful in achieving durative hematological improvements, strongly indicating an etiological association between chronic gut inflammation and the development of AA. Interestingly, comorbid diseases superimposed on this patient(namely, psychiatric disorders, hypertension,insulin resistance, and renal dysfunction) were ameliorated together with the hematological improvements.CONCLUSION Chronic gut inflammation may be responsible for AA pathogenesis. The comorbidities and AA may share a common etiological association.
基金the National Natural Science Foundation of China(No.81470009).
文摘Distinguishing between aplastic anemia(AA)and hypoblastic myelodysplastic syndrome(hMDS)with a low percentage of bone marrow(BM)blasts(<5%)can be difficult due to the overlap in clonality and a spectrum of genetic alternations between the two subtypes of diseases.However,due to recent advances in DNA sequencing technology,both spectnim and frequency of mutations can be accurately determined and monitored by next-generation sequencing(NGS)at initial diagnosis and during immunosuppressive therapy(1ST)in patients with AA or hMDS.This improvement in acquiring a patient's genetic status and clonal evolution can provide more proper,precise,and on-time information to guide disease management,which is especially helpful in the absence of traditional morphologic/cytogenetic evidence.
文摘The content of 12 trace elements in the hair of 20 aplastic anemia patients was determined and compared with that of normal subjects as control. The results showed that patients with deficiency of yin had a significant decrease in lithium, calcium, strontium, and chromium, those with deficiency of yang had a distinct decrease in zinc magnesium barium, strontium, calcium, and lithium, and those with deficiency of both yin and yang had a general decrease in all the 12 trace elements. Changes in trace element content in hair may serve as a guide to opening up new vistas in the treatment of aplastic anemia on the basis of an overall analysis of symptoms and signs.
