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诊断时和霍奇金淋巴瘤随访期间的自身免疫性溶血性贫血和自身免疫性血小板减少症。

Autoimmune hemolytic anemia and autoimmune thrombocytopenia at diagnosis and during follow-up of Hodgkin lymphoma.

机构信息

Department of Hematology, Athens Medical Center, Athens, Greece.

出版信息

Leuk Lymphoma. 2012 Aug;53(8):1481-7. doi: 10.3109/10428194.2012.660628. Epub 2012 Apr 2.

Abstract

Autoimmune hemolytic anemia and thrombocytopenia (AIHA/AITP) frequently complicate the course of non-Hodgkin lymphomas, especially low-grade, but they are very rarely observed in Hodgkin lymphoma (HL). Consequently the frequency and the profile of patients with HL-associated AIHA/AITP have not been well defined. Among 1029 patients with HL diagnosed between 1990 and 2010, two cases of AIHA (0.19%) and three of AITP (0.29%) were identified at the presentation of disease. These patients were significantly older, and more frequently had features of advanced disease and non-nodular sclerosing histology, compared to the majority of patients, who did not have autoimmune cytopenias at diagnosis. ABVD combination chemotherapy (doxorubicin, bleomycin, vinblastine, dacarbazine) provided effective control of HL and the autoimmune condition as well. During approximately 6600 person-years of follow-up for the remaining 1024 patients, seven (0.7%) patients developed autoimmune cytopenias (three AITP, three AIHA, one autoimmune pancytopenia) for a 10- and 15-year actuarial incidence of 0.95% and 1.40%, respectively. Their features did not differ compared to the general population of adult HL. In this large series of consecutive, unselected patients, those who presented with autoimmune cytopenias had a particular demographic and disease-related profile. In contrast, patients developing autoimmune cytopenias during follow-up did not appear to differ significantly from those who did not.

摘要

自身免疫性溶血性贫血和血小板减少症(AIHA/AITP)常使非霍奇金淋巴瘤(NHL)的病程复杂化,尤其是低度恶性 NHL,但在霍奇金淋巴瘤(HL)中则非常罕见。因此,HL 相关 AIHA/AITP 的患者频率和特征尚未明确。在 1990 年至 2010 年间诊断的 1029 例 HL 患者中,有 2 例 AIHA(0.19%)和 3 例 AITP(0.29%)在疾病初诊时出现。与大多数未诊断出自身免疫性血细胞减少症的患者相比,这些患者年龄明显更大,且更常具有晚期疾病特征和非结节性硬化组织学特征。ABVD 联合化疗(阿霉素、博来霉素、长春碱、达卡巴嗪)可有效控制 HL 和自身免疫状态。在其余 1024 例患者约 6600 人年的随访期间,有 7 例(0.7%)患者发生自身免疫性血细胞减少症(3 例 AITP、3 例 AIHA、1 例自身免疫性全血细胞减少症),10 年和 15 年的累积发生率分别为 0.95%和 1.40%。其特征与成人 HL 总体人群相比无差异。在这项连续、未经选择的患者大系列研究中,那些初诊时存在自身免疫性血细胞减少症的患者具有特定的人口统计学和疾病相关特征。相比之下,在随访期间发生自身免疫性血细胞减少症的患者似乎与未发生的患者没有显著差异。

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