Pedrosa Cláudia L, Rosinha Patrícia, Seabra Patrícia, Ferreira Gisela, Rosado Cláudia, Regadas Luísa, Casais Cláudia, Coutinho Jorge
Department of Clinical Hematology, Centro Hospitalar Universitário Do Porto, Porto, Portugal.
Department of Internal Medicine, Centro Hospitalar Do Baixo Vouga, Aveiro, Portugal.
Case Rep Hematol. 2021 Feb 9;2021:8876249. doi: 10.1155/2021/8876249. eCollection 2021.
Autoimmune cytopenia, a known paraneoplastic complication of lymphoid neoplasms, may occur before, concurrently, at relapse, or even years after completion of lymphoma treatment. In the case of Hodgkin lymphoma (HL), it is thought that immune dysregulation, typical of this neoplasm, may be involved in the genesis of these manifestations. We report a 57-year-old male presenting with stage IIIA, International Prognostic Score (IPS) 4, nodular sclerosis HL, and severe AA (SAA) confirmed on the histologic exam of the bone marrow that showed severe marrow hypoplasia due to a decrease in the elements of the three cell linages with left shift of the myeloid maturation. Immunosuppression with steroids and cyclosporine A was started. Eltrombopag and G-CSF were also added. In spite of prompt initiation of immunosuppressive therapy, the patient presented an unfavorable outcome with progressive pancytopenia and severe acute cerebral hemorrhagic event. The patient died 59 days after admission. Although autoimmune disorders are described in HL, its concomitant diagnosis is extremely rare. Our case shows a rare instance of SAA as the first manifestation of HL.
自身免疫性血细胞减少是一种已知的淋巴系统肿瘤副肿瘤性并发症，可发生在淋巴瘤治疗前、治疗期间、复发时，甚至在淋巴瘤治疗结束数年之后。对于霍奇金淋巴瘤（HL）而言，人们认为这种肿瘤典型的免疫失调可能与这些表现的发生有关。我们报告了一名57岁男性，其表现为ⅢA期、国际预后评分（IPS）为4、结节硬化型HL，骨髓组织学检查确诊为严重再生障碍性贫血（SAA），显示由于三系细胞成分减少且髓系成熟左移导致严重骨髓增生低下。开始使用类固醇和环孢素A进行免疫抑制治疗。还加用了艾曲泊帕和粒细胞集落刺激因子（G-CSF）。尽管迅速启动了免疫抑制治疗，但患者病情预后不佳，出现进行性全血细胞减少和严重的急性脑出血事件。患者入院59天后死亡。虽然HL中描述了自身免疫性疾病，但其同时诊断极为罕见。我们的病例显示了SAA作为HL首发表现的罕见情况。
