背景:自身免疫性血细胞减少症(AC),包括免疫性血小板减少症(ITP),自身免疫性溶血性贫血(AIHA)和自身免疫性粒细胞减少症,是淋巴瘤患者中观察到的罕见并发症。它们可能出现在以前,在淋巴瘤诊断期间或之后,患者是否有疾病进展.
目的:本研究旨在将ACs与淋巴瘤类型相关联,病程和预后。我们对在波兰淋巴瘤研究组(PLRG)的中心诊断和治疗的恶性淋巴瘤和AC共存的成年患者进行了多中心回顾性分析。
方法:分析涵盖2016-2022年,包括51例患者,包括23名女性和28名男性。其中,35例患者被诊断为AIHA,15例ITP患者和1例AIHA和ITP患者。
结果:最常见的淋巴瘤类型是霍奇金淋巴瘤(HL)(12例)和弥漫性大B细胞淋巴瘤(DLBCL)(14例)。在诊断的时候,31(61%)的患者有4期HL或DLBCL,根据安阿伯分类。总的来说,对50名患者的治疗反应进行了评估,其中25人完全缓解,6人部分缓解。我们观察到B细胞症状(p=0.036),骨髓受累(p=0.073),脾肿大(p=0.025),与ITP患者相比,AIHA中超过2行治疗更为常见。相反,在ITP患者中更常被诊断为Eucleopenia(p=0.056)和无淋巴瘤进展的ACs(p=0.002)。
结论:在研究组中,复发和难治性疾病更常见,DLBCL患者的总生存期(OS)较短。我们发现,与普通淋巴瘤患者相比,AC的预后较差。对AC治疗的反应没有差异。为了获得更准确的数据,一个更大的群体,作为多中心研究的一部分,应该进行评估。
BACKGROUND: Autoimmune cytopenias (ACs), including immune thrombocytopenia (ITP), autoimmune hemolytic anemia (AIHA) and autoimmune granulocytopenia, are rare complications observed in lymphoma patients. They may appear before, during or after lymphoma diagnosis, whether the patients had disease progression or not.
OBJECTIVE: This study aims to correlate ACs with lymphoma type, disease course and prognosis. We performed a multicenter retrospective analysis of adult patients with malignant lymphoma and ACs coexistence diagnosed and treated in centers aligned with the Polish Lymphoma Research Group (PLRG).
METHODS: The analysis covers the years 2016-2022 and included 51 patients comprised of 23 women and 28 men. Of these, 35 patients were diagnosed with AIHA, 15 patients with ITP and 1 patient with both AIHA and ITP.
RESULTS: The most common type of lymphoma was Hodgkin lymphoma (HL) (12 patients) and diffuse large B-cell lymphoma (DLBCL) (14 patients). At the time of diagnosis, 31 (61%) of patients had stage 4 of HL or DLBCL, according to Ann Arbor classification. In total, the response to treatment was evaluated in 50 patients, with 25 being in complete remission and 6 in partial remission. We observed that B cell symptoms (p = 0.036), bone marrow involvement (p = 0.073), splenomegaly (p = 0.025), and more than 2 lines of treatment were more common in AIHA compared to ITP patients. Conversely, eucopenia (p = 0.056) and ACs without lymphoma progression (p = 0.002) were more often diagnosed in ITP patients.
CONCLUSIONS: In the study group, relapsed and refractory disease was observed more often, and shorter overall survival (OS) was noted in patients with DLBCL. We found that AC is associated with a worse prognosis in comparison to the general population of lymphoma patients. There were no differences in response to AC therapy. To have more accurate data, a larger group, as part of a multicenter study, should be evaluated.