Erythroblastopenia

  • 文章类型: English Abstract
    在这篇文章中,我们再次讨论定义,移植失败的危险因素和治疗指南,移植物功能差和红细胞减少症。移植失败是造血细胞移植(HCT)后严重但罕见的并发症。尽管文献存在差异,我们定义了这种并发症,并根据新的研究讨论了因素风险和治疗建议.移植物功能差也是HCT后更常见的并发症。不久将有新的研究来证明本文中基于治疗医学或细胞疗法提出的当前建议。红细胞减少症,是HCT后罕见的并发症。尽管期望有更好的兼容性捐赠者/接受者选择,一些患者仍然患有这种并发症。
    In this article, we discuss again the definition, the risk factor and guideline to treat the graft failure, the poor graft function and erythrobalstopenia. Graft failure is a severe but rare complication after hematopoietic cell transplantation (HCT). Despite disparity in the literature, we defined this complication and discussed the factor risks and recommendation for treatment based on new studies. Poor graft function is also a more frequent complication after HCT. New studies will soon be available to prove or not the current recommendation suggested in this article based on therapeutics medicine or cellular therapy. Erythroblastopenia, is a rarer complication post HCT. Despite anticipation for a better choice of compatibility donor/recipient, some patients still suffer from this complication.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

  • 文章类型: Case Reports
    红细胞减少症和原发性骨髓纤维化的组合非常罕见。我们在这里报告了一名76岁的摩洛哥患者自2018年以来因特发性红细胞减少症进行随访的不寻常病例。最初用皮质治疗,然后用环孢素治疗。两年后,患者报告骨痛伴脾肿大。评估包括脊髓造影,骨髓活检和分子生物学显示骨髓纤维化。骨髓纤维化的病因评估为阴性,证实了其原始性质。患者接受ruxolitinib和输血支持。患者的预后良好,并以一般状况的改善为标志,脾肿大和输血率。红细胞减少症与骨髓增殖性疾病之间的关联是特殊的,文献中仅报道了少数病例。
    The combination of erythroblastopenia and primary myelofibrosis is very rare. We here report the unusual case of a 76-year-old Moroccan patient followed up since 2018 for idiopathic erythroblastopenia, initially treated with corticotherapy and then with ciclosporin. Two years later, the patient reported bone pain with splenomegaly. Assessment including myelogram, bone marrow biopsy and molecular biology showed myelofibrosis. Etiological assessment of myelofibrosis was negative confirming its primitive nature. The patient received ruxolitinib with transfusion support. Patient´s outcome was favorable and marked by improvement of general condition, splenomegaly and transfusion rate. The association between erythroblastopenia and myeloproliferative disorder is exceptional and only a few cases have been reported in the literature.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Case Reports
    纯红细胞发育不全(PRCA)是一种罕见的副肿瘤综合征,在2-5%的胸腺瘤中观察到。文献报道其管理存在很大差异。根据一个说明性的临床案例,我们进行了系统的文献综述,其主要目的是评估PRCA的治疗管理.文献检索基于Medline和Scopus数据库中的PICO方法进行。参考临床病例涉及一名患有IVa胸腺瘤的51岁女性。在对化疗有初始反应后,PRCA发生局部进展,在二线化疗下反应良好.患者最终死于伴有发热性中性粒细胞减少的双红细胞减少症。该系统综述涵盖了1950年至2019年之间发表的135篇文章。单独或与其他疗法联合使用的胸腺切除术显示PRCA的完全缓解(CR)率为31%,而没有报道没有胸腺切除术的抗肿瘤治疗。在免疫调节疗法中,环孢菌素的CR百分比最高(74%)。最后,胸腺切除术和免疫调节治疗的组合显示CR率为45%。胸腺切除术似乎是PRCA最有效的抗肿瘤治疗方法。免疫调节疗法,特别是环孢菌素,被证明是有效的,但必须考虑感染并发症的风险。针对PRCA的抗肿瘤和免疫调节疗法的最佳位置尚未确定。
    Pure red cell aplasia (PRCA) is a rare paraneoplastic syndrome observed in 2-5 % of thymomas. Literature reports great variability in its management. Based on an illustrative clinical case, we present a systematic literature review whose main objective is to evaluate the therapeutic management of PRCA. The literature search was performed based on the PICO method in the Medline and Scopus databases. The reference clinical case concerns a 51-year-old woman with stage IVa thymoma. After initial response to chemotherapy, a locoregional progression occurred with PRCA development that responded favorably under second line chemotherapy. The patient finally died in a context of bicytopenia with febrile neutropenia. The systematic review covers 135 articles published between 1950 and 2019. Thymectomy alone or in combination with other therapies showed a 31 % complete remission (CR) rate for PRCA of, whereas none was reported with anti-tumor treatments without thymectomy. Among immunomodulatory therapies, cyclosporin gave the highest percentage of CR (74 %). Finally, the combination of thymectomy and immunomodulatory treatments showed a CR rate of 45 %. Thymectomy appeared to be the most effective anti-tumor treatment for PRCA. Immunomodulatory therapies, particularly cyclosporine, are shown effective, but the risk of infectious complications must be considered. The optimal place of anti-tumor and immunomodulatory therapies against PRCA has yet to be determined.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Sci-hub)

  • 文章类型: Case Reports
    Acute parvovirus B19 infection may lead to erythroblastopenia crisis in patients with underlying red blood cells disorders. We report herein an uncommon concomitant transient aplastic crisis in a mother and her daughter, both affected by hereditary spherocytosis. The diagnosis was confirmed by the detection of a very high parvovirus B19 DNA load in both the mother\'s and daughter\'s sera, associated with the presence of parvovirus B19 specific immunoglobulin-M antibodies. This rapid etiologic diagnosis allowed to save bone marrow sampling, although blood transfusion was required regarding the severe anemia associated with pancytopenia. Our observation illustrates first line parvovirus B19 hypothesis in the context of transient aplastic crisis and that contagiousness in household contacts should be considered in family with a medical history of red blood cell pathology.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Sci-hub)

       PDF(Pubmed)

  • 文章类型: Case Reports
    暂无摘要。
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Sci-hub)

  • 文章类型: Consensus Development Conference
    Success of allogeneic hematopoietic stem cells transplantation requires both the underlying disease eradication and satisfying reconstitution of hematopoiesis from donor cells. However, reconstitution delays, secondary development or persistence of cytopenia are regularly observed and are potential causes of failure after allogeneic transplantation. These graft dysfunctions should be distinguished from non-engraftment/engraftment failure. Although these situations are relatively common, there is no consensus in the literature for their management. During the workshop of the SFGM-TC, the working group proposed recommendations from an analysis of the literature.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Sci-hub)

  • 文章类型: Case Reports
    Pregnancy induced erythroblastopenia is an exceptional pathologic entity. Only isolated cases have been described in English literature. The aim of this study is to analyze the characteristics of this extremely rare disease through the description of a new case of pregnancy induced erythroblastopenia and through the study of the 17 cases reported before.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Sci-hub)

  • 文章类型: Case Reports
    We report the case of a 2 year-old boy hospitalized into the emergency room for influenza pneumonia infection. The evolution was marked by a respiratory distress syndrome, a severe hemolytic anemia, associated with thrombocytopenia and kidney failure. First, a diagnosis of hemolytic uremic syndrome (HUS) has been judiciously suggested due to the classical triad: kidney failure, hemolytic anemia and thrombocytopenia. But, strikingly, blood smears do not exhibit schizocytes, but instead ghosts and hemighosts, some characteristic features of a glucose-6-phosphate dehydrogenase deficiency. Our hypothesis has been confirmed by enzymatic dosage and molecular biology. The unusual initial aplastic feature of this anemia could be the result of a transient erythroblastopenia due to the viral agent, at the origin of the G6PD crisis on a background of a major erythrocyte anti-oxydant enzyme defect. This case of G6PD defect points out the continuously importance of the cytology, which was able to redirect the diagnosis by the hemighost and ghost detection.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Sci-hub)

  • DOI:
    文章类型: Journal Article
    BACKGROUND: Acquired erythroblastopenia (AE) is a rare clinical situation. It is characterized by the reduction of erythroid precursors in the bone marrow together with the low reticulocyte counts in the peripheral blood.
    BACKGROUND: Main secondary causes of AE are drugs, Parvovirus B19 and other infectious reasons, lymphoid and myeloid neoplasia, autoimmune diseases, thymoma and pregnancy. The aim of this study is to assess the frequencies and clinical associations of AE via analyzing 12340 bone marrow samples in a retrospective manner.
    METHODS: Bone marrow aspirations which were obtained from patients who applied to Hacettepe University Hematology Clinic between 2002 and 2013, were analyzed retrospectively.
    RESULTS: Thirty four erythroblastopenia cases were found. Patients ranged in age from 16 to 80 years with a median of 38 years. Fifteen patients were men (44%) and nineteen were women (56%). In these patients, detected causes of erythroblastopenia were MDS, idiopathic pure red cell aplasia (PRCA), parvovirus infection, post chemotherapy aplasia, plasma proliferative diseases, copper deficiency due to secondary amyloidosis, fever of unknown origin, hemophagocytic syndrome, enteric fever and legionella pneumonia. We found that between those reasons the most common causes of erythroblastopenia are MDS (17.7%) and idiopathic PRCA (17.7%).
    CONCLUSIONS: As a result, erythroblastopenia in the bone marrow may be an early sign of MDS. In those AE cases possibility of being MDS must be kept in mind as it can be mistaken for PRCA.
    CONCLUSIONS: To conclude, in adults MDS without excess blast is one of the most common causes of erythroblastopenia in clinical practice and in case of erythroblastopenia the presence of MDS should be investigated.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

  • 文章类型: Case Reports
    Bartonella quintana is a facultative intracellular bacteria responsible of negative blood culture endocarditis whose diagnosis is often delayed. The occurrence of renal involvement has been exceptionally described in this context. We report the case of a 54-year-old man presenting with Bartonella quintana endocarditis complicated by proliferative glomerulonephritis with acute kidney injury and erythroblastopenia.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

公众号