Liver cirrhosis portal hypertension patients to reduce the number of blood cells are common in clinical, and often affect the prognosis. This paper discusses cirrhotic portal hypertension patients complicated by the r...Liver cirrhosis portal hypertension patients to reduce the number of blood cells are common in clinical, and often affect the prognosis. This paper discusses cirrhotic portal hypertension patients complicated by the reason of the decrease in the number of peripheral blood cells and what is the clinical significance of these reasons so as to provide theoretical support for the choice of treatment. Splenomegaly and hypersplenism caused should be the main reason for reducing the number of blood cells, but not all, other reasons are alcohol and virus inhibition of bone marrow, liver function impairment, autoimmune damage and loss of blood, etc. If it is a function of the spleen hyperfunction caused by blood cells decreases, blood should rise to normal after splenectomy, or consider other reason or there are other reasons at the same time.展开更多
<span style="font-family:""><span style="font-family:Verdana;">Acute panmyelosis with myelofibrosis (APMF) is a subtype of acute myeloid leukemia (AML) classified among the categor...<span style="font-family:""><span style="font-family:Verdana;">Acute panmyelosis with myelofibrosis (APMF) is a subtype of acute myeloid leukemia (AML) classified among the category of “AML, not otherwise specified” in the WHO 2016 classification of hematopoietic tumors. It is a rare, fatal hematological neoplasm that is characterized by acute onset of cytopenias and bone marrow fibrosis in the absence of splenomegaly or fibrosis related morphological changes in the red blood cells. The difficulty of diagnosis and management explains why APMF is rarely reported in Africa. We report here the case of a 30-year-old man who presented with dizziness, palpitations and dyspnea. Diagnosis of APMF was retained on bone marrow histology and immunohistochemistry which showed bone marrow fibrosis and high cellularity with majority of myeloid blast cells. The patient was treated by low dose cytarabine monotherapy 30 mg/m</span><sup><span style="font-family:Verdana;">2</span></sup><span style="font-family:Verdana;"> per week. At 3 months of treatment, the patient was transfusion-independent, with normalization of hemoglobin and platelets counts. However, the death occurred after 8 months. This case highlights the diagnosis specificity and management of AMPF, knowing the number of potential differential diagnoses and difficulties of its therapeutic management.展开更多
A higher prevalence of immunological processes has recently been reported in patients with hepatitis C virus(HCV)infection,focusing the attention of physicians and researchers on the close association between HCV and ...A higher prevalence of immunological processes has recently been reported in patients with hepatitis C virus(HCV)infection,focusing the attention of physicians and researchers on the close association between HCV and immune disorders.HCV lymphotropism represents the most important step in the pathogenesis of virusrelated immunological diseases and experimental,virologic,and clinical evidence has demonstrated a trigger role for HCV both in systemic autoimmune diseases,such as rheumatoid arthritis,Sj?gren syndrome,hemolytic anemia and severe thrombocytopenia,and in organ-specific autoimmune diseases,such as autoimmune hepatitis,thyroid disorders and diabetes.This review will outline the principal aspects of such HCVinduced immunological alterations,focusing on the prevalence of these less characterized HCV extrahepatic manifestations.展开更多
AIM To study the management, complications and outcomes of adult patients admitted with hemophagocytic lymphohistiocytosis(HLH) in the intensive care unit(ICU).METHODS We performed a retrospective observational study ...AIM To study the management, complications and outcomes of adult patients admitted with hemophagocytic lymphohistiocytosis(HLH) in the intensive care unit(ICU).METHODS We performed a retrospective observational study of adult patients with the diagnosis of "HLH" admitted to the two academic medical ICUs of Baylor College of Medicine between 01/01/2013 to 06/30/2017. HLH was diagnosed using the HLH-2004 criteria proposed by the Histiocyte Society.RESULTS Sixteen adult cases of HLH were admitted to the medical ICUs over 4 years.Median age of presentation was 49 years and 10(63%) were males. Median Sequential Organ Failure Assessment(SOFA) score at the time of ICU admission was 10. Median ICU length of stay(LOS) was 11.5 d and median hospital LOS was 29 d. Septic shock and acute respiratory failure accounted for majority of diagnoses necessitating ICU admission. Septic shock was the most common ICU complication seen in(88%) patients, followed by acute kidney injury(81%) and acute respiratory failure requiring mechanical ventilation(75%). Nine patients(56%) developed disseminated intravascular coagulation and eight(50%) had acute liver failure. 10 episodes of clinically significant bleeding were observed.Multi system organ failure was the most common cause of death seen in 12(75%)patients. The 30 d mortality was 37%(6 cases) and 90 d mortality was 81%(13 cases). There was no difference in mortality based on age(above or less than 50 years), SOFA score on ICU admission(more than or less than 10),immunosuppression, time to diagnose HLH or direct ICU admission versus floor transfer.CONCLUSION HLH is a devastating disease associated with poor outcomes in ICU. Intensivists need to have a high degree of clinical suspicion for HLH in patients with septic shock/multi system organ failure and progressive bi/pancytopenia who are not responding to standard management in ICU.展开更多
BACKGROUND Therapy-related acute promyelocytic leukemia(t-APL)is a rare complication observed in solitary bone plasmacytoma(SBP),and SBP after radiotherapy evolving to APL harboring the FMS-like tyrosine kinase 3-inte...BACKGROUND Therapy-related acute promyelocytic leukemia(t-APL)is a rare complication observed in solitary bone plasmacytoma(SBP),and SBP after radiotherapy evolving to APL harboring the FMS-like tyrosine kinase 3-internal tandem duplication(FLT3-ITD)mutation has never been reported.Here,we present the first case reported until now.CASE SUMMARY We describe a 64-year-old woman who presented with lumbar pain and was initially diagnosed with SBP.However,after one year of radiotherapy treatment,this patient experienced a long-standing bone-marrow-suppressive period and finally developed APL harboring the FLT3-ITD mutation,as confirmed by analyses of clinical features,bone marrow morphology,flow cytometry,cytogenetic examination,and molecular biology.On admission,the patient had disseminated intravascular coagulation and intracranial hemorrhage,and the peripheral blood and bone marrow smear displayed abundant abnormal promyelocytes.Unfortunately,she died when the definite diagnosis was made.CONCLUSION The patient with t-APL harboring FLT3-ITD mutation evolving from SBP after radiotherapy had not been reported and had poor clinical outcomes.FLT3-ITD mutation in t-APL may be a potential pathogenesis of leukemogenesis.We should consider the potential risk of secondary neoplasms in SBP patients after radiotherapy.展开更多
Objective To investigate the clinical features and prognosis of prolonged cytopenia(PC)in patients with large B-cell lymphoma(LBCL)undergoing anti-CD19 chimeric antigen receptor T(CAR-T)cell therapy Methods A retrospe...Objective To investigate the clinical features and prognosis of prolonged cytopenia(PC)in patients with large B-cell lymphoma(LBCL)undergoing anti-CD19 chimeric antigen receptor T(CAR-T)cell therapy Methods A retrospective case series study was conducted on LBCL patients who received CAR-T cell therapy with a survival time of over one month at the Hematology Department of Peking Union Medical College Hospital from March 2019 to December 2023.Statistical analyses were performed on hematologic changes at 1,3,6,and 12 months post-CAR-T infusion,as well as on the progression-freesurvival(PFS)and post-treatment adverse events,including infections.Patientswere categorized into the PC and non-PC groups based on the occurrence of cytopenia at 90 days post-infusion.Differences between1groups were compared,and univariate logistic regression analysis was used to identify risk factors.Results The median age of 27 LBCL patients receiving CAR-T cell therapy was 58 years(range 27-69 years),with 18 males.Among the 27 LBCL patients who received CAR-T cell therapy,PC was observed in 19 patients(70.4%),with instances of neutropenia(48.1%,13 cases),anemia(37.0%,10 cases),and thrombocytopenia(22.2%,6 cases).Univariate logistic regression analysis revealed that prior chemotherapy sensitivity(0R=18.00,95%CI 1.56-207.45,P=0.020)and bone marrow suppression(OR=18.00,95%CI 1.38-235.69,P=0.028)were associated with PC.The median follow-up time was 13.5 months.The PC group exhibited a higher risk of infection within 3 months(9/19 vs.1/8)and a shorter mean PFS(19.3 months vs.24.4 months),although the difference was not statistically significant(both P>0.05).Conclusion PC is common following CAR-T cell therapy and is associated with an increased risk of infection and poorer prognosis.Prior treatment sensitivity and bone marrow suppression may serve as indicators of PC.展开更多
Background Waldenstr（o）m macroglobulinemia （WM） is an uncommon lymphoid malignancy.The characteristics and prognosis of WM have never been systematically studied in the East.Methods We analyzed the clinical charac...Background Waldenstr（o）m macroglobulinemia （WM） is an uncommon lymphoid malignancy.The characteristics and prognosis of WM have never been systematically studied in the East.Methods We analyzed the clinical characteristics and the prognostic factors of 90 Chinese WM patients,and compared them with the Western reports.Results The median age was 62 years old with a male-to-female ratio of 3.74.The most common symptoms at diagnosis were fatigue （77.8％） and bleeding （20％）,while only 6 patients （6.7％） were asymptomatic.In the univariate analysis,age ＞62 years,thrombocytopenia,leucopenia,cytopenias ≥2,and high risk on the international prognostic scoring system for WM were the adverse risk factors,but only age ＞62 years and ≥2 cytopenias were the independent prognostic factors in the multivariate analysis.Using age ＜62 years and ≥2 cytopenias,three significantly different prognostic groups could been distinguished,with 5-year overall survival of 71.6％,48.6％,and 17.0％ （P ＜0.001）.Conclusion Distinct characteristics exist in WM in China compared to the West and we describe a new simple prognostic model for newly diagnosed WM patients.展开更多
Background Langerhans cell histiocytosis(LCH)is a group of diseases characterized by the proliferation and accumulation of Langerhans cells.Clinical presentations of LCH vary widely.Data sources A PubMed search was co...Background Langerhans cell histiocytosis(LCH)is a group of diseases characterized by the proliferation and accumulation of Langerhans cells.Clinical presentations of LCH vary widely.Data sources A PubMed search was conducted using Clinical Queries with the key term "Langerhans cell histiocytosis".The search strategy included meta-analyses,randomized controlled trials,clinical trials,observational studies,and reviews.This paper is based on,but not limited to,the search results.Results Generally,patients with LCH can be divided into two groups based on the extent of involvement at diagnosis,namely,single-system LCH and multisystem LCH.The involvement may be unifocal or multifocal.Patients with isolated bone lesions typically present between 5 and 15 years of age,whereas those with multisystem LCH tend to present before 5 years of age.The clinical spectrum is broad,ranging from an asymptomatic isolated skin or bone lesion to a life-threatening multisystem condition.Clinical manifestations include,among others,"punched out" lytic bone lesion,seborrheic dermatitis-like erup-tion,erythematous/reddish-brown crusted/scaly papules/maculopapules/plaques/patches,and eczematous lesions,diabetes insipidus,hepatosplenomegaly,cytopenias,lymphadenopathy,and an acute fulminant disseminated multisystem condition presenting with fever,skin rash,anemia,thrombocytopenia,lymphadenopathy,and hepatosplenomegaly.The diagnosis is clinicopathologic,based on typical clinical findings and histologic/immunohistochemical examination of a biopsy of lesional tissue.Positive CD1a,S100,and/or CD207(Langerin)immunohistochemical staining of lesional cells is required for a definitive diagnosis.Watchful waiting is recommended for patients with skin-only LCH.Patients with symptomatic or refractory skin-only LCH may be treated with topical tacrolimus/corticosteroids,topical nitrogen mustard,oral methotrexate,or oral hydroxyurea.The current recommended first-line therapy for patients with multisystem LCH is 12 months therapy with prednisone and vinblastine.Mercaptopurine is added for patients with risk organ involvements.Conclusions Because of the broad spectrum of clinical manifestations and the extreme diversity of disease,LCH remains a diagnostic dilemma.Morphological identification of LCH cells and positive immunochemical staining with CD1a,S100,and/or CD207(Langerin)of lesional cells are necessary for a definitive diagnosis.展开更多
Objective To test NK cell quantities and function in patients with positive BMMNC-Coombs test(CBCPC)and cytopenia and to explore how NK cell participate in the progress of this disease.Methods The percentage of CD3-CD...Objective To test NK cell quantities and function in patients with positive BMMNC-Coombs test(CBCPC)and cytopenia and to explore how NK cell participate in the progress of this disease.Methods The percentage of CD3-CD56+NK cell in peripheral blood lymphocytes,the expression of activating receptor(NKG2D,NKp46,NKp44),inhibitory receptor(CD158a,CD158b),per-展开更多
文摘Liver cirrhosis portal hypertension patients to reduce the number of blood cells are common in clinical, and often affect the prognosis. This paper discusses cirrhotic portal hypertension patients complicated by the reason of the decrease in the number of peripheral blood cells and what is the clinical significance of these reasons so as to provide theoretical support for the choice of treatment. Splenomegaly and hypersplenism caused should be the main reason for reducing the number of blood cells, but not all, other reasons are alcohol and virus inhibition of bone marrow, liver function impairment, autoimmune damage and loss of blood, etc. If it is a function of the spleen hyperfunction caused by blood cells decreases, blood should rise to normal after splenectomy, or consider other reason or there are other reasons at the same time.
文摘<span style="font-family:""><span style="font-family:Verdana;">Acute panmyelosis with myelofibrosis (APMF) is a subtype of acute myeloid leukemia (AML) classified among the category of “AML, not otherwise specified” in the WHO 2016 classification of hematopoietic tumors. It is a rare, fatal hematological neoplasm that is characterized by acute onset of cytopenias and bone marrow fibrosis in the absence of splenomegaly or fibrosis related morphological changes in the red blood cells. The difficulty of diagnosis and management explains why APMF is rarely reported in Africa. We report here the case of a 30-year-old man who presented with dizziness, palpitations and dyspnea. Diagnosis of APMF was retained on bone marrow histology and immunohistochemistry which showed bone marrow fibrosis and high cellularity with majority of myeloid blast cells. The patient was treated by low dose cytarabine monotherapy 30 mg/m</span><sup><span style="font-family:Verdana;">2</span></sup><span style="font-family:Verdana;"> per week. At 3 months of treatment, the patient was transfusion-independent, with normalization of hemoglobin and platelets counts. However, the death occurred after 8 months. This case highlights the diagnosis specificity and management of AMPF, knowing the number of potential differential diagnoses and difficulties of its therapeutic management.
文摘A higher prevalence of immunological processes has recently been reported in patients with hepatitis C virus(HCV)infection,focusing the attention of physicians and researchers on the close association between HCV and immune disorders.HCV lymphotropism represents the most important step in the pathogenesis of virusrelated immunological diseases and experimental,virologic,and clinical evidence has demonstrated a trigger role for HCV both in systemic autoimmune diseases,such as rheumatoid arthritis,Sj?gren syndrome,hemolytic anemia and severe thrombocytopenia,and in organ-specific autoimmune diseases,such as autoimmune hepatitis,thyroid disorders and diabetes.This review will outline the principal aspects of such HCVinduced immunological alterations,focusing on the prevalence of these less characterized HCV extrahepatic manifestations.
文摘AIM To study the management, complications and outcomes of adult patients admitted with hemophagocytic lymphohistiocytosis(HLH) in the intensive care unit(ICU).METHODS We performed a retrospective observational study of adult patients with the diagnosis of "HLH" admitted to the two academic medical ICUs of Baylor College of Medicine between 01/01/2013 to 06/30/2017. HLH was diagnosed using the HLH-2004 criteria proposed by the Histiocyte Society.RESULTS Sixteen adult cases of HLH were admitted to the medical ICUs over 4 years.Median age of presentation was 49 years and 10(63%) were males. Median Sequential Organ Failure Assessment(SOFA) score at the time of ICU admission was 10. Median ICU length of stay(LOS) was 11.5 d and median hospital LOS was 29 d. Septic shock and acute respiratory failure accounted for majority of diagnoses necessitating ICU admission. Septic shock was the most common ICU complication seen in(88%) patients, followed by acute kidney injury(81%) and acute respiratory failure requiring mechanical ventilation(75%). Nine patients(56%) developed disseminated intravascular coagulation and eight(50%) had acute liver failure. 10 episodes of clinically significant bleeding were observed.Multi system organ failure was the most common cause of death seen in 12(75%)patients. The 30 d mortality was 37%(6 cases) and 90 d mortality was 81%(13 cases). There was no difference in mortality based on age(above or less than 50 years), SOFA score on ICU admission(more than or less than 10),immunosuppression, time to diagnose HLH or direct ICU admission versus floor transfer.CONCLUSION HLH is a devastating disease associated with poor outcomes in ICU. Intensivists need to have a high degree of clinical suspicion for HLH in patients with septic shock/multi system organ failure and progressive bi/pancytopenia who are not responding to standard management in ICU.
基金Natural Science Foundation of Zhejiang Province,No.LY19H290003Zhejiang Provincial Medical and Health Science and Technology Project,No.2020KY196and Foundation of Zhejiang Province Chinese Medicine Science and Technology Planes,No.2017ZB030.
文摘BACKGROUND Therapy-related acute promyelocytic leukemia(t-APL)is a rare complication observed in solitary bone plasmacytoma(SBP),and SBP after radiotherapy evolving to APL harboring the FMS-like tyrosine kinase 3-internal tandem duplication(FLT3-ITD)mutation has never been reported.Here,we present the first case reported until now.CASE SUMMARY We describe a 64-year-old woman who presented with lumbar pain and was initially diagnosed with SBP.However,after one year of radiotherapy treatment,this patient experienced a long-standing bone-marrow-suppressive period and finally developed APL harboring the FLT3-ITD mutation,as confirmed by analyses of clinical features,bone marrow morphology,flow cytometry,cytogenetic examination,and molecular biology.On admission,the patient had disseminated intravascular coagulation and intracranial hemorrhage,and the peripheral blood and bone marrow smear displayed abundant abnormal promyelocytes.Unfortunately,she died when the definite diagnosis was made.CONCLUSION The patient with t-APL harboring FLT3-ITD mutation evolving from SBP after radiotherapy had not been reported and had poor clinical outcomes.FLT3-ITD mutation in t-APL may be a potential pathogenesis of leukemogenesis.We should consider the potential risk of secondary neoplasms in SBP patients after radiotherapy.
文摘Objective To investigate the clinical features and prognosis of prolonged cytopenia(PC)in patients with large B-cell lymphoma(LBCL)undergoing anti-CD19 chimeric antigen receptor T(CAR-T)cell therapy Methods A retrospective case series study was conducted on LBCL patients who received CAR-T cell therapy with a survival time of over one month at the Hematology Department of Peking Union Medical College Hospital from March 2019 to December 2023.Statistical analyses were performed on hematologic changes at 1,3,6,and 12 months post-CAR-T infusion,as well as on the progression-freesurvival(PFS)and post-treatment adverse events,including infections.Patientswere categorized into the PC and non-PC groups based on the occurrence of cytopenia at 90 days post-infusion.Differences between1groups were compared,and univariate logistic regression analysis was used to identify risk factors.Results The median age of 27 LBCL patients receiving CAR-T cell therapy was 58 years(range 27-69 years),with 18 males.Among the 27 LBCL patients who received CAR-T cell therapy,PC was observed in 19 patients(70.4%),with instances of neutropenia(48.1%,13 cases),anemia(37.0%,10 cases),and thrombocytopenia(22.2%,6 cases).Univariate logistic regression analysis revealed that prior chemotherapy sensitivity(0R=18.00,95%CI 1.56-207.45,P=0.020)and bone marrow suppression(OR=18.00,95%CI 1.38-235.69,P=0.028)were associated with PC.The median follow-up time was 13.5 months.The PC group exhibited a higher risk of infection within 3 months(9/19 vs.1/8)and a shorter mean PFS(19.3 months vs.24.4 months),although the difference was not statistically significant(both P>0.05).Conclusion PC is common following CAR-T cell therapy and is associated with an increased risk of infection and poorer prognosis.Prior treatment sensitivity and bone marrow suppression may serve as indicators of PC.
基金This work was supported by grants from Science and Technology Infrastructure Program of Tianjin （No.12ZCDZSY17600）,National Public Health Grand Research Foundation （No.201202017）,and National Nature Science Foundation of China （No.81370632 and No.81200395）.
文摘Background Waldenstr（o）m macroglobulinemia （WM） is an uncommon lymphoid malignancy.The characteristics and prognosis of WM have never been systematically studied in the East.Methods We analyzed the clinical characteristics and the prognostic factors of 90 Chinese WM patients,and compared them with the Western reports.Results The median age was 62 years old with a male-to-female ratio of 3.74.The most common symptoms at diagnosis were fatigue （77.8％） and bleeding （20％）,while only 6 patients （6.7％） were asymptomatic.In the univariate analysis,age ＞62 years,thrombocytopenia,leucopenia,cytopenias ≥2,and high risk on the international prognostic scoring system for WM were the adverse risk factors,but only age ＞62 years and ≥2 cytopenias were the independent prognostic factors in the multivariate analysis.Using age ＜62 years and ≥2 cytopenias,three significantly different prognostic groups could been distinguished,with 5-year overall survival of 71.6％,48.6％,and 17.0％ （P ＜0.001）.Conclusion Distinct characteristics exist in WM in China compared to the West and we describe a new simple prognostic model for newly diagnosed WM patients.
文摘Background Langerhans cell histiocytosis(LCH)is a group of diseases characterized by the proliferation and accumulation of Langerhans cells.Clinical presentations of LCH vary widely.Data sources A PubMed search was conducted using Clinical Queries with the key term "Langerhans cell histiocytosis".The search strategy included meta-analyses,randomized controlled trials,clinical trials,observational studies,and reviews.This paper is based on,but not limited to,the search results.Results Generally,patients with LCH can be divided into two groups based on the extent of involvement at diagnosis,namely,single-system LCH and multisystem LCH.The involvement may be unifocal or multifocal.Patients with isolated bone lesions typically present between 5 and 15 years of age,whereas those with multisystem LCH tend to present before 5 years of age.The clinical spectrum is broad,ranging from an asymptomatic isolated skin or bone lesion to a life-threatening multisystem condition.Clinical manifestations include,among others,"punched out" lytic bone lesion,seborrheic dermatitis-like erup-tion,erythematous/reddish-brown crusted/scaly papules/maculopapules/plaques/patches,and eczematous lesions,diabetes insipidus,hepatosplenomegaly,cytopenias,lymphadenopathy,and an acute fulminant disseminated multisystem condition presenting with fever,skin rash,anemia,thrombocytopenia,lymphadenopathy,and hepatosplenomegaly.The diagnosis is clinicopathologic,based on typical clinical findings and histologic/immunohistochemical examination of a biopsy of lesional tissue.Positive CD1a,S100,and/or CD207(Langerin)immunohistochemical staining of lesional cells is required for a definitive diagnosis.Watchful waiting is recommended for patients with skin-only LCH.Patients with symptomatic or refractory skin-only LCH may be treated with topical tacrolimus/corticosteroids,topical nitrogen mustard,oral methotrexate,or oral hydroxyurea.The current recommended first-line therapy for patients with multisystem LCH is 12 months therapy with prednisone and vinblastine.Mercaptopurine is added for patients with risk organ involvements.Conclusions Because of the broad spectrum of clinical manifestations and the extreme diversity of disease,LCH remains a diagnostic dilemma.Morphological identification of LCH cells and positive immunochemical staining with CD1a,S100,and/or CD207(Langerin)of lesional cells are necessary for a definitive diagnosis.
文摘Objective To test NK cell quantities and function in patients with positive BMMNC-Coombs test(CBCPC)and cytopenia and to explore how NK cell participate in the progress of this disease.Methods The percentage of CD3-CD56+NK cell in peripheral blood lymphocytes,the expression of activating receptor(NKG2D,NKp46,NKp44),inhibitory receptor(CD158a,CD158b),per-
