Barcellini Wilma, Fattizzo Bruno
Hematology, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan, Italy.
Department of Oncology and Hemato-Oncology, University of Milan, Milan, Italy.
Hematol Oncol. 2025 Jun;43 Suppl 2(Suppl 2):e70063. doi: 10.1002/hon.70063.
Peripheral autoimmune cytopenias may complicate a fraction of lymphoproliferative disorders (LPD), particularly chronic lymphocytic leukemia, non-Hodgkin B-cell lymphomas, angioimmunoblastic T-cell lymphoma and large granular lymphocytic leukemia. The most frequent complications are autoimmune hemolytic anemia and immune thrombocytopenia, followed by pure red cell aplasia, autoimmune neutropenia and other systemic/organ specific autoimmune diseases. The latter are less frequently reported and probably underdiagnosed, and include systemic lupus erythematosus, rheumatoid arthritis, Sjögren's syndrome, antiphospholipid syndrome and disorders of the hemostatic system. Therapy is mainly directed at the specific autoimmune complication when it arises in the context of a non-active LPD. However, autoimmune complications that are refractory to first line therapy usually require an LPD-directed treatment. Of note, several B-cell and T cell directed therapies that are used in LPD are also indicated or in trials for primary autoimmune cytopenias, underlying the overlapping pathogenic mechanisms between LPD and autoimmunity.
外周自身免疫性血细胞减少症可能使一部分淋巴增殖性疾病（LPD）复杂化，尤其是慢性淋巴细胞白血病、非霍奇金B细胞淋巴瘤、血管免疫母细胞性T细胞淋巴瘤和大颗粒淋巴细胞白血病。最常见的并发症是自身免疫性溶血性贫血和免疫性血小板减少症，其次是纯红细胞再生障碍、自身免疫性中性粒细胞减少症和其他全身性/器官特异性自身免疫性疾病。后者的报道较少，可能诊断不足，包括系统性红斑狼疮、类风湿关节炎、干燥综合征、抗磷脂综合征和止血系统疾病。当在非活动性LPD的背景下出现特定的自身免疫性并发症时，治疗主要针对该并发症。然而，一线治疗难治的自身免疫性并发症通常需要针对LPD的治疗。值得注意的是，几种用于LPD的B细胞和T细胞定向疗法也适用于原发性自身免疫性血细胞减少症或正在进行相关试验，这表明LPD和自身免疫之间存在重叠的致病机制。
