Kawano Noriaki, Ono Nobuyuki, Kawano Sayaka, Kuriyama Takuro, Yoshida Shuro, Inoue Sanshiro, Yamashita Kiyoshi, Himeji Daisuke, Shimao Yoshiya, Marutsuka Kousuke, Oshima Koichi, Ueda Akira, Kawano Fumiko
Department of Internal Medicine, Miyazaki Prefectural Miyazaki Hospital.
J Clin Exp Hematop. 2014;54(3):187-96. doi: 10.3960/jslrt.54.187.
Immunodeficiency-associated lymphoproliferative disorders (LPD) represent a rare life-threatening clinical entity characterized by heterogeneous histological findings that range from polymorphic to monomorphic proliferated abnormal lymphocytes. Currently, there is no standard treatment for LPD. To elucidate the clinical features and treatment outcomes of immunodeficiency-associated LPD patients with rheumatoid arthritis (RA), we retrospectively evaluated 9 cases observed over a 5-year period. The diagnoses of these patients included 5 diffuse large B-cell lymphomas, 3 LPD, and 1 mucosa-associated lymphoid tissue lymphoma. At initial diagnosis, 6 patients had advanced-stage RA, and half of these underwent total knee arthroplasty. All patients with RA received methotrexate (MTX) and low-dose prednisolone. Biologics were administered to 4 of 9 patients. After the development of immunodeficiency-associated LPD, MTX discontinuation resulted in 5 complete remissions (CR), 1 partial remission, and 3 cases of stable disease. Relapse was observed in 3 of 5 CR patients in the MTX-withdrawal remission group. Subsequently, conventional chemotherapy, rituximab, and radiation were administered to 4, 3, and 1 patient, respectively. These treatments induced a second CR. In the chemotherapy group, 1 patient developed acute myocardial infarction and another experienced ileus and pulmonary abscess. In the rituximab group, no severe complications were observed. Consequently, all patients remained disease-free during the median 23-month follow-up period. Our results indicate that, depending on the RA disease stage, performance status, and extent of treatment response, less intensive treatments than those commonly indicated for non-Hodgkin lymphoma, involving MTX discontinuation and subsequent therapy containing rituximab, might be an efficient therapeutic strategy for immunodeficiency-associated LPD.
免疫缺陷相关淋巴增殖性疾病（LPD）是一种罕见的危及生命的临床实体，其特征为组织学表现多样，从多形性到单形性增殖的异常淋巴细胞。目前，LPD尚无标准治疗方法。为阐明类风湿关节炎（RA）合并免疫缺陷相关LPD患者的临床特征和治疗结果，我们回顾性评估了5年内观察到的9例患者。这些患者的诊断包括5例弥漫性大B细胞淋巴瘤、3例LPD和1例黏膜相关淋巴组织淋巴瘤。初诊时，6例患者处于RA晚期，其中半数接受了全膝关节置换术。所有RA患者均接受了甲氨蝶呤（MTX）和低剂量泼尼松龙治疗。9例患者中有4例使用了生物制剂。免疫缺陷相关LPD发生后，停用MTX导致5例完全缓解（CR）、1例部分缓解和3例病情稳定。MTX停药缓解组的5例CR患者中有3例复发。随后，分别对4例、3例和1例患者进行了传统化疗、利妥昔单抗治疗和放疗。这些治疗诱导了第二次CR。化疗组中，1例患者发生急性心肌梗死，另1例出现肠梗阻和肺脓肿。利妥昔单抗组未观察到严重并发症。因此，在中位23个月的随访期内，所有患者均无疾病复发。我们的结果表明，根据RA疾病分期、身体状况和治疗反应程度，采用比非霍奇金淋巴瘤常用治疗强度更低的治疗方法，包括停用MTX及随后使用含利妥昔单抗的治疗，可能是免疫缺陷相关LPD的有效治疗策略。
