Huwait Hassan, Wang Beatrice, Shustik Chaim, Michel René P
Department of Pathology, McGill University and McGill University Health Center, Quebec, Canada.
Am J Dermatopathol. 2010 Feb;32(1):65-70. doi: 10.1097/DAD.0b013e3181af7dee.
Patients with rheumatoid arthritis, whether treated or not with immunosuppressive agents including methotrexate, have an increased risk of lymphoproliferative disorders. Termed "iatrogenic immunodeficiency-associated lymphoproliferative disorders" in the 2008 World Health Organization classification of lymphoid neoplasms, they include Hodgkin and non-Hodgkin lymphomas. Composite lymphomas are rare, particularly in skin, with none reported in immunodeficiency states. We report the case of a 67 year-old woman with a long history of rheumatoid arthritis, on methotrexate treatment, who developed multiple skin lesions exhibiting a malignant infiltrate displaying both B- and T-cell phenotypes and dual clonal gene rearrangement by polymerase chain reaction, consistent with a cutaneous composite lymphoma. The patient received chemotherapy including rituximab with partial response, but the T-cell component recurred. To the best of our knowledge, this is the first case report of a cutaneous composite lymphoma in a patient with an iatrogenic immunodeficiency representing a dual challenge, diagnostic for the pathologist and therapeutic for the hematologist.
类风湿关节炎患者，无论是否接受包括甲氨蝶呤在内的免疫抑制剂治疗，发生淋巴增殖性疾病的风险都会增加。在2008年世界卫生组织淋巴肿瘤分类中，这些疾病被称为“医源性免疫缺陷相关淋巴增殖性疾病”，包括霍奇金淋巴瘤和非霍奇金淋巴瘤。复合淋巴瘤很罕见，尤其是在皮肤中，免疫缺陷状态下尚无相关报道。我们报告了一例67岁女性患者，有类风湿关节炎病史且长期接受甲氨蝶呤治疗，该患者出现多个皮肤病变，经聚合酶链反应显示恶性浸润具有B细胞和T细胞表型以及双重克隆基因重排，符合皮肤复合淋巴瘤的表现。患者接受了包括利妥昔单抗在内的化疗，部分缓解，但T细胞成分复发。据我们所知，这是首例医源性免疫缺陷患者发生皮肤复合淋巴瘤的病例报告，这对病理学家来说是诊断挑战，对血液学家来说是治疗挑战。
