Angioimmunoblastic T-cell lymphoma(AITL)is a unique type of peripheral T-cell lymphoma with a constellation of clinical symptoms and signs,including weight loss,fever,chills,anemia,skin rash,hepatosplenomegaly,lymphad...Angioimmunoblastic T-cell lymphoma(AITL)is a unique type of peripheral T-cell lymphoma with a constellation of clinical symptoms and signs,including weight loss,fever,chills,anemia,skin rash,hepatosplenomegaly,lymphadenopathy,thrombocytopenia and polyclonal hypergammaglobulinemia.The histological features of AITL are also distinctive.Pure red cell aplasia is a bone marrow failure characterized by progressive normocytic anemia and reticulocytopenia without leucopenia or thrombocytopenia.However,AITL with abdominal pain and pure red cell aplasia has rarely been reported.Here,we report a rare case of AITL-associated pure red cell aplasia with abdominal pain.The diagnosis was verified by a biopsy of the enlarged abdominal lymph nodes with immunohistochemical staining.展开更多
BACKGROUND T-cell large granular lymphocytic leukemia(T-LGLL)is a rare type of aplastic anemia with diverse clinical manifestations.Concomitant diseases are often present at the first manifestation.We describe the tre...BACKGROUND T-cell large granular lymphocytic leukemia(T-LGLL)is a rare type of aplastic anemia with diverse clinical manifestations.Concomitant diseases are often present at the first manifestation.We describe the treatment of a patient with CD57-negativeγδT-LGLL with pure red cell aplasia(PRCA).CASE SUMMARY A 34-year-old woman with a 20-year history of anemia visited our hospital owing to severe dizziness and was admitted.Her condition was diagnosed as CD57-negativeγδT-LGLL with PRCA through bone marrow cytology,bone marrow pathology,bone marrow flow cytometry,bone marrow multiplex polymerase chain reaction combined with fluorescent fragment analysis,and other tests.Treatment with prednisone,methotrexate,and subcutaneous erythropoietin did not significantly change her hemoglobin level.After treatment with oral cyclophosphamide for 3 mo,her hemoglobin level increased to approximately 100 g/L.After 5 mo of treatment,the patient could perform activities of daily living independently.CONCLUSION The treatment of CD57-negativeγδT-LGLL with PRCA with cyclophosphamide helps to improve prognosis.展开更多
Dear Editor,Haploidentical allogeneic hematopoietic stem cell transplantation(haplo-HSCT),a curative therapy for severe aplastic anemia(SAA)patients,has been used clinically for decades.Two models,not involving ex vit...Dear Editor,Haploidentical allogeneic hematopoietic stem cell transplantation(haplo-HSCT),a curative therapy for severe aplastic anemia(SAA)patients,has been used clinically for decades.Two models,not involving ex vitro T-cell depletion,have been adopted for haplo-HSCT in patients with SAA.The first is referred to as the"Beijing protocol"(Xu et al.,2017),and comprises a conditioning regimen using busulfex(BU),cyclophosphamide(CY).展开更多
Objective To evaluate the efficacy of unrelated do-nor allogeneic hematopoietic stem cell transplantation(URD allo-HSCT).for children and adolescents with severe aplastic anemia(SAA).Methods Clinical data of34 SAA chi...Objective To evaluate the efficacy of unrelated do-nor allogeneic hematopoietic stem cell transplantation(URD allo-HSCT).for children and adolescents with severe aplastic anemia(SAA).Methods Clinical data of34 SAA children and adolescents undergoing allo-HSCT were retrospectively analyzed from October 2001 to October 2015.According to the source of donor,the展开更多
Parvovirus B19(B19V)infection can cause pure red cell aplasia(PRCA)in patients with human immunodeficiency virus(HIV)infection.Intravenous immunoglobulin(IVIG)is a preferred treatment option.From July 2019 to March 20...Parvovirus B19(B19V)infection can cause pure red cell aplasia(PRCA)in patients with human immunodeficiency virus(HIV)infection.Intravenous immunoglobulin(IVIG)is a preferred treatment option.From July 2019 to March 2022,four patients with HIV infection were admitted to Guangzhou Eighth People’s Hospital with dizziness and fatigue and were diagnosed with PRCA.Blood investigations revealed severe anemia and the B19V genome.Therefore,the four patients were diagnosed with B19V-induced PRCA.All four patients received red blood cell transfusion in the setting of antiretroviral therapy,and two of the four patients received intravenous immunoglobulin(IVIG).After 3-7 months of treatment,all four patients recovered,although two did not receive IVIG.This suggests that IVIG is not always necessary for the treatment of PRCA in patients with HIV infection and that effective antiretroviral therapy and immunological reconstitution play an important role in the eradication of parvovirus.展开更多
Objective: To explore the mechanism ofintegrated traditional Chinese and Westernmedicine (TCM--WM ) therapy on chronicaplastic anemia (CAA). Methods: The RBClife span of 30 normal human subjects and 30patients with CA...Objective: To explore the mechanism ofintegrated traditional Chinese and Westernmedicine (TCM--WM ) therapy on chronicaplastic anemia (CAA). Methods: The RBClife span of 30 normal human subjects and 30patients with CAA were measured by sir labelled technique before and after TCM--WMtherapy. The morphology and distribution ofRBC membrane protein granules were observed by freeze fracture etching and transmission electron microscope. Results: The halflife of erythrocytes (RBC TI/2)was shortenedin CAA cases and there was a significant difference compared to healthy control (P <0. 01). After therapy, the RBC life span prolonged and approached the normal level. Before treatment, there existed abnormal in morphology, decrease in amount and uneven indistribution of protein granules in protoplasmicface (PF) and extracellular face (EF) of RBCmembrane. After treatment, the protein granules of RBC membrane was improved and approached to control. Conclusions: The morphology, amount, quality and distribution ofRBC membrane protein granule were closelyrelated to its life span. The therapeutic effectof TCM--WM was better than that of WMalone and it had a function both in stabilizingmembrane protein and extending the RBC lifespan.展开更多
本文回顾性分析2021年3月山东大学附属威海市立医院收治的1例CD4^(-)CD8^(-)TCRγδ^(+)T细胞大颗粒淋巴细胞白血病(T-cell large granular lymphocyte leukemia,T-LGLL)合并纯红细胞再生障碍性贫血(Pure red cell aplasia,PRCA)患者的...本文回顾性分析2021年3月山东大学附属威海市立医院收治的1例CD4^(-)CD8^(-)TCRγδ^(+)T细胞大颗粒淋巴细胞白血病(T-cell large granular lymphocyte leukemia,T-LGLL)合并纯红细胞再生障碍性贫血(Pure red cell aplasia,PRCA)患者的临床资料,并复习相关文献,以提高对CD4^(-)CD8^(-)TCRγδ^(+)T-LGLL合并PRCA的认知,从而减少临床中的误诊及漏诊。展开更多
本研究检测获得性纯红细胞再生障碍性贫血(acquired pure red cell aplastic anemia,A-PRCA)患者γδT细胞亚群数量和功能变化,探讨γδΤ细胞在A-PRCA发病机制中的作用。对11例经骨髓涂片和活检确诊的A-PRCA患者,给予环胞菌素A和雷公...本研究检测获得性纯红细胞再生障碍性贫血(acquired pure red cell aplastic anemia,A-PRCA)患者γδT细胞亚群数量和功能变化,探讨γδΤ细胞在A-PRCA发病机制中的作用。对11例经骨髓涂片和活检确诊的A-PRCA患者,给予环胞菌素A和雷公藤多甙治疗;采用流式细胞术检测免疫抑制剂治疗前后患者T细胞亚群和γδT细胞水平;分离患者外周血中单个核细胞并置于含有10%FCS,PHA10μg/ml,rIL-250U/ml的RPMI1640培养液培养2周,然后用TCRγδ磁珠分选出γδT细胞,在体外与正常人骨髓共同培养,观察对红系、粒系集落生长的影响。结果显示,治疗前组外周血CD3+/CD8+细胞数较正常对照组升高,CD4+/CD8+比例倒置(P<0.05);治疗前组患者γδT细胞水平有明显升高(P<0.05)。治疗后,有效组CD3+/CD8+细胞数下降(P<0.05),CD4+/CD8+比值上升(P<0.01);γδT细胞水平较治疗前有明显降低(P<0.05)。从患者外周血分离的γδT细胞体外能抑制正常骨髓红系CFU-E和BFU-E生长,并随浓度的增长抑制作用越明显,而在不同的浓度梯度下对粒系生长无明显影响。结论:A-PRCA患者γδT细胞亚群增高,其在PRCA的发病机制中起了一定作用;体外分离培养的患者γδT细胞可抑制正常红系集落生长,对粒系集落生长影响不明显;环胞素A治疗A-PRCA有较好疗效。展开更多
文摘Angioimmunoblastic T-cell lymphoma(AITL)is a unique type of peripheral T-cell lymphoma with a constellation of clinical symptoms and signs,including weight loss,fever,chills,anemia,skin rash,hepatosplenomegaly,lymphadenopathy,thrombocytopenia and polyclonal hypergammaglobulinemia.The histological features of AITL are also distinctive.Pure red cell aplasia is a bone marrow failure characterized by progressive normocytic anemia and reticulocytopenia without leucopenia or thrombocytopenia.However,AITL with abdominal pain and pure red cell aplasia has rarely been reported.Here,we report a rare case of AITL-associated pure red cell aplasia with abdominal pain.The diagnosis was verified by a biopsy of the enlarged abdominal lymph nodes with immunohistochemical staining.
基金Supported by Xiamen Medical and Health Guidance Project,No.3502Z20199137Fujian Medical and Health Training Project for Young and Middle-aged Backbone Talents,No.2020GGB068Educational and Scientific Research Program for Young and Middle-Aged Teachers of Fujian Province,No.JAT190838.
文摘BACKGROUND T-cell large granular lymphocytic leukemia(T-LGLL)is a rare type of aplastic anemia with diverse clinical manifestations.Concomitant diseases are often present at the first manifestation.We describe the treatment of a patient with CD57-negativeγδT-LGLL with pure red cell aplasia(PRCA).CASE SUMMARY A 34-year-old woman with a 20-year history of anemia visited our hospital owing to severe dizziness and was admitted.Her condition was diagnosed as CD57-negativeγδT-LGLL with PRCA through bone marrow cytology,bone marrow pathology,bone marrow flow cytometry,bone marrow multiplex polymerase chain reaction combined with fluorescent fragment analysis,and other tests.Treatment with prednisone,methotrexate,and subcutaneous erythropoietin did not significantly change her hemoglobin level.After treatment with oral cyclophosphamide for 3 mo,her hemoglobin level increased to approximately 100 g/L.After 5 mo of treatment,the patient could perform activities of daily living independently.CONCLUSION The treatment of CD57-negativeγδT-LGLL with PRCA with cyclophosphamide helps to improve prognosis.
文摘Dear Editor,Haploidentical allogeneic hematopoietic stem cell transplantation(haplo-HSCT),a curative therapy for severe aplastic anemia(SAA)patients,has been used clinically for decades.Two models,not involving ex vitro T-cell depletion,have been adopted for haplo-HSCT in patients with SAA.The first is referred to as the"Beijing protocol"(Xu et al.,2017),and comprises a conditioning regimen using busulfex(BU),cyclophosphamide(CY).
文摘Objective To evaluate the efficacy of unrelated do-nor allogeneic hematopoietic stem cell transplantation(URD allo-HSCT).for children and adolescents with severe aplastic anemia(SAA).Methods Clinical data of34 SAA children and adolescents undergoing allo-HSCT were retrospectively analyzed from October 2001 to October 2015.According to the source of donor,the
基金Guangzhou Basic Research Program on People’s Livelihood Science and Technology(202002020005)National Natural Science Foundation of China(82072265)+1 种基金Technology Planning Project of Guangdong Province(2021B1212040017)Sun Yat-sen University Founded Program(2022_76220_B21127)。
文摘Parvovirus B19(B19V)infection can cause pure red cell aplasia(PRCA)in patients with human immunodeficiency virus(HIV)infection.Intravenous immunoglobulin(IVIG)is a preferred treatment option.From July 2019 to March 2022,four patients with HIV infection were admitted to Guangzhou Eighth People’s Hospital with dizziness and fatigue and were diagnosed with PRCA.Blood investigations revealed severe anemia and the B19V genome.Therefore,the four patients were diagnosed with B19V-induced PRCA.All four patients received red blood cell transfusion in the setting of antiretroviral therapy,and two of the four patients received intravenous immunoglobulin(IVIG).After 3-7 months of treatment,all four patients recovered,although two did not receive IVIG.This suggests that IVIG is not always necessary for the treatment of PRCA in patients with HIV infection and that effective antiretroviral therapy and immunological reconstitution play an important role in the eradication of parvovirus.
文摘Objective: To explore the mechanism ofintegrated traditional Chinese and Westernmedicine (TCM--WM ) therapy on chronicaplastic anemia (CAA). Methods: The RBClife span of 30 normal human subjects and 30patients with CAA were measured by sir labelled technique before and after TCM--WMtherapy. The morphology and distribution ofRBC membrane protein granules were observed by freeze fracture etching and transmission electron microscope. Results: The halflife of erythrocytes (RBC TI/2)was shortenedin CAA cases and there was a significant difference compared to healthy control (P <0. 01). After therapy, the RBC life span prolonged and approached the normal level. Before treatment, there existed abnormal in morphology, decrease in amount and uneven indistribution of protein granules in protoplasmicface (PF) and extracellular face (EF) of RBCmembrane. After treatment, the protein granules of RBC membrane was improved and approached to control. Conclusions: The morphology, amount, quality and distribution ofRBC membrane protein granule were closelyrelated to its life span. The therapeutic effectof TCM--WM was better than that of WMalone and it had a function both in stabilizingmembrane protein and extending the RBC lifespan.
文摘本文回顾性分析2021年3月山东大学附属威海市立医院收治的1例CD4^(-)CD8^(-)TCRγδ^(+)T细胞大颗粒淋巴细胞白血病(T-cell large granular lymphocyte leukemia,T-LGLL)合并纯红细胞再生障碍性贫血(Pure red cell aplasia,PRCA)患者的临床资料,并复习相关文献,以提高对CD4^(-)CD8^(-)TCRγδ^(+)T-LGLL合并PRCA的认知,从而减少临床中的误诊及漏诊。
文摘本研究检测获得性纯红细胞再生障碍性贫血(acquired pure red cell aplastic anemia,A-PRCA)患者γδT细胞亚群数量和功能变化,探讨γδΤ细胞在A-PRCA发病机制中的作用。对11例经骨髓涂片和活检确诊的A-PRCA患者,给予环胞菌素A和雷公藤多甙治疗;采用流式细胞术检测免疫抑制剂治疗前后患者T细胞亚群和γδT细胞水平;分离患者外周血中单个核细胞并置于含有10%FCS,PHA10μg/ml,rIL-250U/ml的RPMI1640培养液培养2周,然后用TCRγδ磁珠分选出γδT细胞,在体外与正常人骨髓共同培养,观察对红系、粒系集落生长的影响。结果显示,治疗前组外周血CD3+/CD8+细胞数较正常对照组升高,CD4+/CD8+比例倒置(P<0.05);治疗前组患者γδT细胞水平有明显升高(P<0.05)。治疗后,有效组CD3+/CD8+细胞数下降(P<0.05),CD4+/CD8+比值上升(P<0.01);γδT细胞水平较治疗前有明显降低(P<0.05)。从患者外周血分离的γδT细胞体外能抑制正常骨髓红系CFU-E和BFU-E生长,并随浓度的增长抑制作用越明显,而在不同的浓度梯度下对粒系生长无明显影响。结论:A-PRCA患者γδT细胞亚群增高,其在PRCA的发病机制中起了一定作用;体外分离培养的患者γδT细胞可抑制正常红系集落生长,对粒系集落生长影响不明显;环胞素A治疗A-PRCA有较好疗效。
