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
Aplastic anemia(AA)is a rare but serious condition in which the bone marrow fails to produce sufficient new blood cells,leading to fatigue,increased susceptibility to infection,and uncontrolled bleeding.In this editor...Aplastic anemia(AA)is a rare but serious condition in which the bone marrow fails to produce sufficient new blood cells,leading to fatigue,increased susceptibility to infection,and uncontrolled bleeding.In this editorial,we review and comment on an article by Wang et al published in 2024.This study aimed to evaluate the potential therapeutic benefits of ginsenoside Rg1 in AA,focusing on its protective effects and uncovering the underlying mechanisms.Cyclophosphamide(CTX)administration caused substantial damage to the structural integrity of the bone marrow and decreased the number of hematopoietic stem cells,thereby establishing an AA model.Compared with the AA group,ginsenoside Rg1 alleviated the effects of CTX by reducing apoptosis and inflammatory factors.Mechanistically,treatment with ginsenoside Rg1 significantly mitigated myelosuppression in mice by inhibiting the mitogen activated protein kinase signaling pathway.Thus,this study indicates that ginsenoside Rg1 could be effective in treating AA by reducing myelosuppression,primarily through its influence on the mitogen activated protein kinase signaling pathway.We expect that our review and comments will provide valuable insights for the scientific community related to this research and enhance the overall clarity of this article.展开更多
Aplastic anemia (AA) is a rare, life-threatening disease characterized by pancytopenia and bone marrow failure. However, since the introduction of immunosuppressive therapy and allogeneic stem cell transplantation, ou...Aplastic anemia (AA) is a rare, life-threatening disease characterized by pancytopenia and bone marrow failure. However, since the introduction of immunosuppressive therapy and allogeneic stem cell transplantation, outcomes have improved considerably, with 5-year survival reported to be 70% - 90%. In Congo, contemporary data on survival are lacking. We performed a retrospective study to describe the epidemiological, diagnostic, and therapeutic characteristics of patients with AA diagnosed in the clinical hematology department of the University Hospital of Brazzaville from 2017 to 2023. Chemically induced aplasia was excluded from the study. The CAMITTA criteria were used to classify the severity of AA. In total, 45 confirmed cases were identified, and 35 files provided sufficient data for the descriptive study. The median age was 26 years (range: 1 - 50). Adults made up 75% of the study population. The sex ratio was 1.05 (0.5 in children and 1.17 in adults). One case of AA was secondary to treatment with imatinib mesylate;the other cases (97.1%) were idiopathic. Pancytopenia was present in all patients. Moderate, severe, and very severe AA represented 11.4%, 74.3%, and 14.3% of cases, respectively. Severe and very severe forms were more frequent in adults (77.4% vs. 22.6%) and in men. Nine patients (26%) received cyclosporine monotherapy. Only one received treatment regularly and obtained the only favorable response. No patient received ATG or eltrombopag. Hemorrhagic syndrome was the most common cause of death (4 out of 6 cases) due to the unavailability of platelet concentrates. Eighteen patients (51.4% of cases) were lost to follow-up. The median follow-up was 28.7 (1 - 96) months. In conclusion, the prognosis of AA remains poor and could be improved with affordable immunosuppressive treatments, availability of platelet concentrates, and implementation of allogeneic bone marrow transplantation.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
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 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
Summary: Mice with immune induced aplastic anemia (AA) were given 5 mg ligustrazine intraperitoneally twice a day. On the 14th day, the expression of CD 49d , CD 49e , cyclinD 2 in bone marrow mononuclear ...Summary: Mice with immune induced aplastic anemia (AA) were given 5 mg ligustrazine intraperitoneally twice a day. On the 14th day, the expression of CD 49d , CD 49e , cyclinD 2 in bone marrow mononuclear cells (MNC) was examined by flow cytometry, and VCAM 1 on stromal cells was immunohistochemically measured by Strept Avidin Biotin Complex (SABC). The expression of CD 49d , CD 49e , VCAM 1 and cyclinD 2 in ligustrazine treated group was significantly higher than that in AA group ( P <0 01), but the ratio of G 0+G 1 phase cells was significantly lower than that in AA group ( P <0.01).The results showed that ligustrazine could improve the expression of adherent molecule and cyclin D 2 in the bone marrow of mice with immune induced aplastic anemia, thereby promoting the growth of hematopoietic cells.展开更多
We previously reported a serendipitous finding from a patient with refractory severe aplastic anemia who had gotten an unexpected hematological response to treatment with gut-cleansing preparations(GCPs).This patient ...We previously reported a serendipitous finding from a patient with refractory severe aplastic anemia who had gotten an unexpected hematological response to treatment with gut-cleansing preparations(GCPs).This patient experienced three recurrences over the ensuing one year of intermittent GCP treatments,with each recurrence occurring 7-8 wk from a GCP.After his third recurrence,he was prescribed successive treatment with rifampicin,berberine,and monthly administered GCP for 4 mo,and he developed an erythroid proliferative neoplasma and an overwhelming enteropathy,and eventually died of septic shock.Laboratory investigations had validated the resolution of myelosuppression and the appearance of malignant clonal hematopoiesis.From the treatment process and laboratory investigations,it is reasonably inferred that the engagement of gut inflammation is critically required in sustaining the overall pathophysiology of acquired aplastic anemia probably by creating a chronic inflammatory state.Incorporation of rifampicin,berberine,and monthly GCP into cyclosporine can enhance the immunosuppressive effect.In a subgroup of acquired aplastic anemia patients whose pathogenesis is associated with genotoxic exposure,the suppressed normal hematopoiesis may result from the bystander insult that is mediated by the soluble inflammatory cytokines generated in response to the immunogenic products of damaged hematopoietic cells in the context of chronic inflammatory state and may offer a protective antineoplastic mechanism against malignant proliferation.展开更多
Objective. To explore the relationship between human parvovirus B19 (HPV B19) infection and aplastic anemia (AA) and to investigate the role of HPV B19 in the occurrence of AA. Methods. The presence of HPV B19 DNA was...Objective. To explore the relationship between human parvovirus B19 (HPV B19) infection and aplastic anemia (AA) and to investigate the role of HPV B19 in the occurrence of AA. Methods. The presence of HPV B19 DNA was detected in the peripheral blood samples of 60 patients with AA (children 38 and adults 22) by nested polymerase chain reaction (PCR) assay, and 30 healthy persons were selected as control. Results. Sixteen (26.7％ ) of 60 AA cases were HPV B19 DNA positive, while all the samples in the control group were negative for HPV B19 (P = 0.000914). Among the case group, the positive rates of HPV B19 DNA were 21.4％ (6 / 28), 30.0％ (3 / 10), 20.0％ (1 / 5) and 35.3％ (6 / 17) in children acute AA (AAA), children chronic AA (CAA), adults AAA and adults CAA patients respectively, which were significantly higher than that in the control group. Furthermore, there was no remarkable difference between children AA and adults AA in the 16 HPV B19 DNA positive patients; neither was there between AAA and CAA. Conclusions. HPV B19 infection is not only correlated with the occurrence of children AAA and CAA, but also with adults AAA and CAA, and might be an important viral cause for AA in humans.展开更多
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Aplastic anemia(AA)is a rare but serious condition in which the bone marrow fails to produce sufficient new blood cells,leading to fatigue,increased susceptibility to infection,and uncontrolled bleeding.In this editorial,we review and comment on an article by Wang et al published in 2024.This study aimed to evaluate the potential therapeutic benefits of ginsenoside Rg1 in AA,focusing on its protective effects and uncovering the underlying mechanisms.Cyclophosphamide(CTX)administration caused substantial damage to the structural integrity of the bone marrow and decreased the number of hematopoietic stem cells,thereby establishing an AA model.Compared with the AA group,ginsenoside Rg1 alleviated the effects of CTX by reducing apoptosis and inflammatory factors.Mechanistically,treatment with ginsenoside Rg1 significantly mitigated myelosuppression in mice by inhibiting the mitogen activated protein kinase signaling pathway.Thus,this study indicates that ginsenoside Rg1 could be effective in treating AA by reducing myelosuppression,primarily through its influence on the mitogen activated protein kinase signaling pathway.We expect that our review and comments will provide valuable insights for the scientific community related to this research and enhance the overall clarity of this article.
文摘Aplastic anemia (AA) is a rare, life-threatening disease characterized by pancytopenia and bone marrow failure. However, since the introduction of immunosuppressive therapy and allogeneic stem cell transplantation, outcomes have improved considerably, with 5-year survival reported to be 70% - 90%. In Congo, contemporary data on survival are lacking. We performed a retrospective study to describe the epidemiological, diagnostic, and therapeutic characteristics of patients with AA diagnosed in the clinical hematology department of the University Hospital of Brazzaville from 2017 to 2023. Chemically induced aplasia was excluded from the study. The CAMITTA criteria were used to classify the severity of AA. In total, 45 confirmed cases were identified, and 35 files provided sufficient data for the descriptive study. The median age was 26 years (range: 1 - 50). Adults made up 75% of the study population. The sex ratio was 1.05 (0.5 in children and 1.17 in adults). One case of AA was secondary to treatment with imatinib mesylate;the other cases (97.1%) were idiopathic. Pancytopenia was present in all patients. Moderate, severe, and very severe AA represented 11.4%, 74.3%, and 14.3% of cases, respectively. Severe and very severe forms were more frequent in adults (77.4% vs. 22.6%) and in men. Nine patients (26%) received cyclosporine monotherapy. Only one received treatment regularly and obtained the only favorable response. No patient received ATG or eltrombopag. Hemorrhagic syndrome was the most common cause of death (4 out of 6 cases) due to the unavailability of platelet concentrates. Eighteen patients (51.4% of cases) were lost to follow-up. The median follow-up was 28.7 (1 - 96) months. In conclusion, the prognosis of AA remains poor and could be improved with affordable immunosuppressive treatments, availability of platelet concentrates, and implementation of allogeneic bone marrow transplantation.
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.
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.
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.
文摘Summary: Mice with immune induced aplastic anemia (AA) were given 5 mg ligustrazine intraperitoneally twice a day. On the 14th day, the expression of CD 49d , CD 49e , cyclinD 2 in bone marrow mononuclear cells (MNC) was examined by flow cytometry, and VCAM 1 on stromal cells was immunohistochemically measured by Strept Avidin Biotin Complex (SABC). The expression of CD 49d , CD 49e , VCAM 1 and cyclinD 2 in ligustrazine treated group was significantly higher than that in AA group ( P <0 01), but the ratio of G 0+G 1 phase cells was significantly lower than that in AA group ( P <0.01).The results showed that ligustrazine could improve the expression of adherent molecule and cyclin D 2 in the bone marrow of mice with immune induced aplastic anemia, thereby promoting the growth of hematopoietic cells.
文摘We previously reported a serendipitous finding from a patient with refractory severe aplastic anemia who had gotten an unexpected hematological response to treatment with gut-cleansing preparations(GCPs).This patient experienced three recurrences over the ensuing one year of intermittent GCP treatments,with each recurrence occurring 7-8 wk from a GCP.After his third recurrence,he was prescribed successive treatment with rifampicin,berberine,and monthly administered GCP for 4 mo,and he developed an erythroid proliferative neoplasma and an overwhelming enteropathy,and eventually died of septic shock.Laboratory investigations had validated the resolution of myelosuppression and the appearance of malignant clonal hematopoiesis.From the treatment process and laboratory investigations,it is reasonably inferred that the engagement of gut inflammation is critically required in sustaining the overall pathophysiology of acquired aplastic anemia probably by creating a chronic inflammatory state.Incorporation of rifampicin,berberine,and monthly GCP into cyclosporine can enhance the immunosuppressive effect.In a subgroup of acquired aplastic anemia patients whose pathogenesis is associated with genotoxic exposure,the suppressed normal hematopoiesis may result from the bystander insult that is mediated by the soluble inflammatory cytokines generated in response to the immunogenic products of damaged hematopoietic cells in the context of chronic inflammatory state and may offer a protective antineoplastic mechanism against malignant proliferation.
基金This study was supported by the Starting Fund for Returned Scholars of PLA (No. 947008).
文摘Objective. To explore the relationship between human parvovirus B19 (HPV B19) infection and aplastic anemia (AA) and to investigate the role of HPV B19 in the occurrence of AA. Methods. The presence of HPV B19 DNA was detected in the peripheral blood samples of 60 patients with AA (children 38 and adults 22) by nested polymerase chain reaction (PCR) assay, and 30 healthy persons were selected as control. Results. Sixteen (26.7％ ) of 60 AA cases were HPV B19 DNA positive, while all the samples in the control group were negative for HPV B19 (P = 0.000914). Among the case group, the positive rates of HPV B19 DNA were 21.4％ (6 / 28), 30.0％ (3 / 10), 20.0％ (1 / 5) and 35.3％ (6 / 17) in children acute AA (AAA), children chronic AA (CAA), adults AAA and adults CAA patients respectively, which were significantly higher than that in the control group. Furthermore, there was no remarkable difference between children AA and adults AA in the 16 HPV B19 DNA positive patients; neither was there between AAA and CAA. Conclusions. HPV B19 infection is not only correlated with the occurrence of children AAA and CAA, but also with adults AAA and CAA, and might be an important viral cause for AA in humans.
