Durani Urshila, Go Ronald S, Kay Neil E
Mayo Clinic, Rochester, Minnesota.
Clin Adv Hematol Oncol. 2018 Oct;16(10):670-676.
Autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia purpura (ITP) have been associated with B-cell lymphoproliferative disorders. Here, we review the epidemiology, pathogenesis, diagnosis, and treatment of these autoimmune disorders, specifically in the setting of B-cell malignancies. AIHA and ITP are classically associated with chronic lymphocytic leukemia (CLL) but have also been reported in plasmacytic and lymphoproliferative disorders. AIHA includes both warm AIHA and cold agglutinin disease, the latter of which is strongly associated with Waldenström macroglobulinemia. The pathogenesis of these cytopenias varies with the underlying disease, but malignant cells serving as antigen-presenting cells to T lymphocytes, with the generation of autoreactive lymphocytes, may be involved. The diagnosis requires the presence of hemolysis and a positive direct antiglobulin test result. In a minority of cases, the direct antiglobulin test result is negative, and more specialized testing may be required. Data on the prognostic effect of these comorbidities are conflicting, and the prognosis may vary depending on when in the B-cell malignant process the cytopenia(s) develops. The treatment of AIHA and ITP in the setting of B-cell lymphoproliferative disorders often involves treatment of the underlying disorder, although in some cases of CLL, treatment of the underlying disorder is not indicated, and management is similar to that for idiopathic AIHA or ITP.
自身免疫性溶血性贫血（AIHA）和免疫性血小板减少性紫癜（ITP）与B细胞淋巴增殖性疾病相关。在此，我们综述这些自身免疫性疾病的流行病学、发病机制、诊断和治疗，特别是在B细胞恶性肿瘤背景下的情况。AIHA和ITP传统上与慢性淋巴细胞白血病（CLL）相关，但也有报道见于浆细胞性和淋巴增殖性疾病。AIHA包括温抗体型AIHA和冷凝集素病，后者与华氏巨球蛋白血症密切相关。这些血细胞减少症的发病机制因基础疾病而异，但恶性细胞作为抗原呈递细胞作用于T淋巴细胞并产生自身反应性淋巴细胞可能参与其中。诊断需要存在溶血且直接抗球蛋白试验结果为阳性。在少数情况下，直接抗球蛋白试验结果为阴性，可能需要更专门的检测。关于这些合并症的预后影响的数据相互矛盾，预后可能因血细胞减少症在B细胞恶性过程中的发生时间而异。在B细胞淋巴增殖性疾病背景下，AIHA和ITP的治疗通常涉及基础疾病的治疗，尽管在某些CLL病例中，不建议对基础疾病进行治疗，其管理与特发性AIHA或ITP相似。
