The Editor welcomes submissions for possible publication in the Letters to the Editor section. Letters commenting on an article published in the Journal or other interesting pieces will be considered if they are recei...The Editor welcomes submissions for possible publication in the Letters to the Editor section. Letters commenting on an article published in the Journal or other interesting pieces will be considered if they are received within 6 weeks of the time the article was published. Authors of the article being commented on will be given an opportunity to offer a timely response to the letter. Authors of letters will be notified that the letter has been received. Unpublished letters cannot be returned.展开更多
MANY cases of pure red cell aplasia （PRCA） were mediated by over-function of immune cells, and responded well to immunosuppressive therapy. Sometimes refractory cases also arose. Fludarabine is an analogue of adenos...MANY cases of pure red cell aplasia （PRCA） were mediated by over-function of immune cells, and responded well to immunosuppressive therapy. Sometimes refractory cases also arose. Fludarabine is an analogue of adenosine resistant to deamination which is widely used for B-chronic lymphocytic leukemia （CLL） and other hematological malignancies.^2 As a strong immunosuppressive agent, fludarabine has generally been used in nonmyeloblative conditioning regimens for hematopoietic stem cells transplantation for hematological malignancies and severe aplastic anemia recently.^3 In this study,展开更多
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 Angioimmunoblastic T-cell lymphoma(AITL)is a peripheral T-cell lymphoma,which is a rare subtype of lymphoma.Patients with AITL often have skin lesions,which are observed in 50%of all cases;the chief complai...BACKGROUND Angioimmunoblastic T-cell lymphoma(AITL)is a peripheral T-cell lymphoma,which is a rare subtype of lymphoma.Patients with AITL often have skin lesions,which are observed in 50%of all cases;the chief complaint of this patient was palpable purpura.AITL often complicates autoimmune or hematological disorders;however,among these,pure red cell aplasia(PRCA)is a very rare complication of AITL.We herein report a case of AITL with PRCA.CASE SUMMARY A 77-year-old Japanese man presented to our hospital with complaints of loss of appetite for 2 mo and a 10-d history of palpable purpura.On physical examination,the patient was afebrile but had bilateral multiple palpable purpuric lesions over the lower extremities,lower abdomen,and part of the upper extremities.Moreover,lymphadenopathy of the bilateral inguinal,cervical,and supraclavicular nodes was noted.Laboratory and imaging studies and skin biopsy were conducted but were inconclusive.Based on inguinal lymph node excisional biopsy,we diagnosed the patient with AITL.Subsequently,the patient developed progressive normocytic normochromic anemia that necessitated almost daily blood transfusion.The clinical presentations and results of bone marrow assessment were consistent with those of PRCA,which is associated with AITL.Chemotherapy was initiated but was not effective.The patient refused further chemotherapy and opted to continue receiving best supportive care.CONCLUSION PRCA is an extremely rare complication of AITL.As the pathophysiology remains unclear,further research is warranted.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
Pure red cell aplasia （PRCA） due to parvovirus B19 （PVB19） infectiori after solid organ transplantation has been rarely reported and most of the cases were renal transplant recipients, Few have been described afte...Pure red cell aplasia （PRCA） due to parvovirus B19 （PVB19） infectiori after solid organ transplantation has been rarely reported and most of the cases were renal transplant recipients, Few have been described after liver transplantation. Moreover, little information on the management of this easily recurring disease is available at present. We describe the first case of a Chinese liver transplant recipient with PVB19-induced PRCA during immunosuppressive therapy. The patient suffered from progressive anemia with the lowest hemoglobin level of 21 g/L. Bone marrow biopsy showed selectively inhibited erythropoiesis with giant pronormoblasts. Detection of PVB19-DNA in serum with quantitative polymerase chain reaction （PCR） revealed a high level of viral load. After 2 courses of intravenous immunoglobulin （IVIG） therapy, bone marrow erythropoiesis recovered with his hemoglobin level increased to 123 g/L. He had a lowlevel PVB19 load for a 5-too follow-up period without recurrence of PRCA, and finally the virus was cleared. Our case indicates that clearance of PVB19 by IVIG in transplant recipients might be delayed after recovery of anemia.展开更多
Pure red cell aplasia (PRCA) is a rare hematological disorder which is characterized by severe anemia,reticulocytopenia and almost complete absence of erythroid precursors in bone marrow.The pathophysiology of PRCA ma...Pure red cell aplasia (PRCA) is a rare hematological disorder which is characterized by severe anemia,reticulocytopenia and almost complete absence of erythroid precursors in bone marrow.The pathophysiology of PRCA may be congenital or acquired.To our knowledge,there is only one case report in the English literature of PRCA after pegylated interferon combination therapy for chronic hepatitis C.We report a second case of PRCA after pegylated interferon combination treatment for chronic hepatitis C.The diagnosis of PRCA was confirmed by the typical findings of bone marrow biopsy.The possible etiologies of our case are also discussed in this paper.展开更多
Variants in the solute carrier family 40 member 1(SLC40A1)gene are the molecular basis of ferroportin disease,which is an autosomal dominant hereditary hemochromatosis.Here,we present a patient with pure red cell apla...Variants in the solute carrier family 40 member 1(SLC40A1)gene are the molecular basis of ferroportin disease,which is an autosomal dominant hereditary hemochromatosis.Here,we present a patient with pure red cell aplasia(PRCA)and large granular lymphocytic leukemia(LGLL)associated with an extremely high levels of serum ferritin and iron overload syndrome.Whole exon sequencing revealed a novel heterozygous variant in SLC40A1(p.T419I),which was found in his daughter as well.A series of functional studies in vitro of the T419I variant in ferroportin were conducted and the results revealed a reduced capacity of iron export from cells without changes in protein localization and its sensitivity to hepcidin.Intracellular iron storage in mutated cells was significantly higher than that of wild-type.These findings suggest that the novel variant p.T419I can cause the classical form of ferroportin disease and an elevated intracellular iron level indicates a potential novel pathogenic mechanism underlying PRCA and LGLL.展开更多
Acquired pure red cell aplasia(aPRCA)is a rare hematological disorder characterized by normochromic,normocytic anemia,reticulocytopenia,and the absence of erythroblasts.The pathogenesis of aPRCA has remained elusive.T...Acquired pure red cell aplasia(aPRCA)is a rare hematological disorder characterized by normochromic,normocytic anemia,reticulocytopenia,and the absence of erythroblasts.The pathogenesis of aPRCA has remained elusive.This review delves into the intricate web of immune mechanisms underlying the development of this enigmatic condition.By exploring immune responses,cytotoxic effects,and antibody-mediated processes,we dissect the immune-driven assault on erythroid progenitors.The classification of aPRCA,including its primary and secondary forms,is elucidated,with a particular emphasis on etiological factors such as viruses,drugs,thymoma,and large granular lymphocytic leukemia.Furthermore,we discuss the implications of cytogenetic changes in erythroid progenitors and immune cells in the pathophysiology of aPRCA.This comprehensive overview aims to shed light on the complex interplay between immune dysregulation and erythroid failure in aPRCA,offering insights that will be crucial for better understanding and treating this disease.展开更多
为了研究ABO血型不合异基因造血干细胞移植(allo-HSCT)后并发纯红细胞再生障碍(pure red cell aplasia,PRCA)的发病情况及危险因素,对本医院以往血型不合异基因造血干细胞移植进行回顾性分析,探讨移植后患者PRCA的发病危险因素。研究结...为了研究ABO血型不合异基因造血干细胞移植(allo-HSCT)后并发纯红细胞再生障碍(pure red cell aplasia,PRCA)的发病情况及危险因素,对本医院以往血型不合异基因造血干细胞移植进行回顾性分析,探讨移植后患者PRCA的发病危险因素。研究结果表明,72例ABO血型不合allo-HSCT患者中,4例发生PRCA,其中A供O3例,A供B1例。PRCA的发生不影响急性移植物抗宿主病(GVHD)或巨细胞病毒(CMV)感染的发生。PRCA患者红系恢复的时间显著长于未PRCA发生患者。结论:PRCA是ABO血型不合移植的主要并发症。A供O可能是ABO血型不合allo-HSCT后并发PRCA的危险因素。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The Editor welcomes submissions for possible publication in the Letters to the Editor section. Letters commenting on an article published in the Journal or other interesting pieces will be considered if they are received within 6 weeks of the time the article was published. Authors of the article being commented on will be given an opportunity to offer a timely response to the letter. Authors of letters will be notified that the letter has been received. Unpublished letters cannot be returned.
基金Supported by the Natural Science Foundation of Jiangsu Province(2004BK424)the 135 Key Department Fund of Jiangsu Province(135XY0416)the Outstanding Person Fund of the First Affiliated Hospital of Soochow University(2004YQG05)
文摘MANY cases of pure red cell aplasia （PRCA） were mediated by over-function of immune cells, and responded well to immunosuppressive therapy. Sometimes refractory cases also arose. Fludarabine is an analogue of adenosine resistant to deamination which is widely used for B-chronic lymphocytic leukemia （CLL） and other hematological malignancies.^2 As a strong immunosuppressive agent, fludarabine has generally been used in nonmyeloblative conditioning regimens for hematopoietic stem cells transplantation for hematological malignancies and severe aplastic anemia recently.^3 In this study,
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.
文摘BACKGROUND Angioimmunoblastic T-cell lymphoma(AITL)is a peripheral T-cell lymphoma,which is a rare subtype of lymphoma.Patients with AITL often have skin lesions,which are observed in 50%of all cases;the chief complaint of this patient was palpable purpura.AITL often complicates autoimmune or hematological disorders;however,among these,pure red cell aplasia(PRCA)is a very rare complication of AITL.We herein report a case of AITL with PRCA.CASE SUMMARY A 77-year-old Japanese man presented to our hospital with complaints of loss of appetite for 2 mo and a 10-d history of palpable purpura.On physical examination,the patient was afebrile but had bilateral multiple palpable purpuric lesions over the lower extremities,lower abdomen,and part of the upper extremities.Moreover,lymphadenopathy of the bilateral inguinal,cervical,and supraclavicular nodes was noted.Laboratory and imaging studies and skin biopsy were conducted but were inconclusive.Based on inguinal lymph node excisional biopsy,we diagnosed the patient with AITL.Subsequently,the patient developed progressive normocytic normochromic anemia that necessitated almost daily blood transfusion.The clinical presentations and results of bone marrow assessment were consistent with those of PRCA,which is associated with AITL.Chemotherapy was initiated but was not effective.The patient refused further chemotherapy and opted to continue receiving best supportive care.CONCLUSION PRCA is an extremely rare complication of AITL.As the pathophysiology remains unclear,further research is warranted.
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Pure red cell aplasia （PRCA） due to parvovirus B19 （PVB19） infectiori after solid organ transplantation has been rarely reported and most of the cases were renal transplant recipients, Few have been described after liver transplantation. Moreover, little information on the management of this easily recurring disease is available at present. We describe the first case of a Chinese liver transplant recipient with PVB19-induced PRCA during immunosuppressive therapy. The patient suffered from progressive anemia with the lowest hemoglobin level of 21 g/L. Bone marrow biopsy showed selectively inhibited erythropoiesis with giant pronormoblasts. Detection of PVB19-DNA in serum with quantitative polymerase chain reaction （PCR） revealed a high level of viral load. After 2 courses of intravenous immunoglobulin （IVIG） therapy, bone marrow erythropoiesis recovered with his hemoglobin level increased to 123 g/L. He had a lowlevel PVB19 load for a 5-too follow-up period without recurrence of PRCA, and finally the virus was cleared. Our case indicates that clearance of PVB19 by IVIG in transplant recipients might be delayed after recovery of anemia.
文摘Pure red cell aplasia (PRCA) is a rare hematological disorder which is characterized by severe anemia,reticulocytopenia and almost complete absence of erythroid precursors in bone marrow.The pathophysiology of PRCA may be congenital or acquired.To our knowledge,there is only one case report in the English literature of PRCA after pegylated interferon combination therapy for chronic hepatitis C.We report a second case of PRCA after pegylated interferon combination treatment for chronic hepatitis C.The diagnosis of PRCA was confirmed by the typical findings of bone marrow biopsy.The possible etiologies of our case are also discussed in this paper.
基金was supported by grants from the National Natural Science Foundation of China under grant numbers 81770119&81700120。
文摘Variants in the solute carrier family 40 member 1(SLC40A1)gene are the molecular basis of ferroportin disease,which is an autosomal dominant hereditary hemochromatosis.Here,we present a patient with pure red cell aplasia(PRCA)and large granular lymphocytic leukemia(LGLL)associated with an extremely high levels of serum ferritin and iron overload syndrome.Whole exon sequencing revealed a novel heterozygous variant in SLC40A1(p.T419I),which was found in his daughter as well.A series of functional studies in vitro of the T419I variant in ferroportin were conducted and the results revealed a reduced capacity of iron export from cells without changes in protein localization and its sensitivity to hepcidin.Intracellular iron storage in mutated cells was significantly higher than that of wild-type.These findings suggest that the novel variant p.T419I can cause the classical form of ferroportin disease and an elevated intracellular iron level indicates a potential novel pathogenic mechanism underlying PRCA and LGLL.
基金supported by Key Technology Research and Development Program of Tianjin China(18ZXDBSY00140 to HW).
文摘Acquired pure red cell aplasia(aPRCA)is a rare hematological disorder characterized by normochromic,normocytic anemia,reticulocytopenia,and the absence of erythroblasts.The pathogenesis of aPRCA has remained elusive.This review delves into the intricate web of immune mechanisms underlying the development of this enigmatic condition.By exploring immune responses,cytotoxic effects,and antibody-mediated processes,we dissect the immune-driven assault on erythroid progenitors.The classification of aPRCA,including its primary and secondary forms,is elucidated,with a particular emphasis on etiological factors such as viruses,drugs,thymoma,and large granular lymphocytic leukemia.Furthermore,we discuss the implications of cytogenetic changes in erythroid progenitors and immune cells in the pathophysiology of aPRCA.This comprehensive overview aims to shed light on the complex interplay between immune dysregulation and erythroid failure in aPRCA,offering insights that will be crucial for better understanding and treating this disease.
文摘为了研究ABO血型不合异基因造血干细胞移植(allo-HSCT)后并发纯红细胞再生障碍(pure red cell aplasia,PRCA)的发病情况及危险因素,对本医院以往血型不合异基因造血干细胞移植进行回顾性分析,探讨移植后患者PRCA的发病危险因素。研究结果表明,72例ABO血型不合allo-HSCT患者中,4例发生PRCA,其中A供O3例,A供B1例。PRCA的发生不影响急性移植物抗宿主病(GVHD)或巨细胞病毒(CMV)感染的发生。PRCA患者红系恢复的时间显著长于未PRCA发生患者。结论:PRCA是ABO血型不合移植的主要并发症。A供O可能是ABO血型不合allo-HSCT后并发PRCA的危险因素。
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。
