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首页> 美国卫生研究院文献>The Scientific World Journal >Pure Red Cell Aplasia and Lymphoproliferative Disorders: An Infrequent Association
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Pure Red Cell Aplasia and Lymphoproliferative Disorders: An Infrequent Association

机译：纯红细胞发育不全和淋巴细胞增生性疾病：罕见协会。

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摘要

Pure red cell aplasia (PRCA) is a rare bone marrow failure syndrome defined by a progressive normocytic anaemia and reticulocytopenia without leukocytopenia and thrombocytopenia. Secondary PRCA can be associated with various haematological disorders, such as chronic lymphocytic leukaemia (CLL) or non-Hodgkin lymphoma (NHL). The aim of the present review is to investigate the infrequent association between PRCA and lymphoproliferative disorders. PRCA might precede the appearance of lymphoma, may present simultaneously with the lymphoid neoplastic disease, or might appear following the lymphomatic disorder. Possible pathophysiological molecular mechanisms to explain the rare association between PRCA and lymphoproliferative disorders are reported. Most cases of PRCA are presumed to be autoimmune mediated by antibodies against either erythroblasts or erythropoietin, by T-cells secreting factors selectively inhibiting erythroid colonies in the bone marrow or by NK cells directly lysing erythroblasts. Finally, focus is given to the therapeutical approach, as several treatment regimens have failed for PRCA. Immunosuppressive therapy and/or chemotherapy are effective for improving anaemia in the majority of patients with lymphoma-associated PRCA. Further investigation is required to define the pathophysiology of PRCA at a molecular level and to provide convincing evidence why it might appear as a rare complication of lymphoproliferative disorders.
机译：纯红细胞发育不全（PRCA）是一种罕见的骨髓衰竭综合征，定义为进行性正常红细胞性贫血和网状细胞减少，无白细胞减少和血小板减少。继发性PRCA可能与各种血液疾病有关，例如慢性淋巴细胞性白血病（CLL）或非霍奇金淋巴瘤（NHL）。本文的目的是研究PRCA与淋巴增生性疾病之间的罕见关联。 PRCA可能在淋巴瘤出现之前出现，可能与淋巴肿瘤性疾病同时出现，或者可能在淋巴系统疾病之后出现。据报道，可能的病理生理分子机制解释了PRCA和淋巴增生性疾病之间的罕见关联。推测大多数PRCA病例是由针对成红细胞或促红细胞生成素的抗体，选择性抑制骨髓中红系集落的T细胞分泌因子或直接裂解成红细胞的NK细胞自身免疫介导的。最后，由于几种治疗方案对PRCA均无效，因此将重点放在治疗方法上。免疫抑制疗法和/或化学疗法可有效改善大多数淋巴瘤相关PRCA患者的贫血。需要进一步的研究以在分子水平上定义PRCA的病理生理学，并提供令人信服的证据，为什么它可能会成为淋巴增生性疾病的罕见并发症。

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