Kakiuchi Seiji, Yakushijin Kimikazu, Takagi Ikumi, Rikitake Junpei, Akiyama Hiroaki, Matsuba Hiroyuki, Hayashi Yoshitake, Kajimoto Kazuyoshi, Iwata Nobuko
Department of Hematology, Yodogawa Christian Hospital, Osaka, Japan.
Division of Medical Oncology and Hematology, Kobe University Graduate School of Medicine, Kobe, Japan.
Front Med (Lausanne). 2020 Dec 23;7:625442. doi: 10.3389/fmed.2020.625442. eCollection 2020.
Immunosuppressants are widely used to treat patients with rheumatoid arthritis (RA), and their adverse effects have been known to cause other iatrogenic immunodeficiency-associated lymphoproliferative disorders (OIIA-LPDs). We report a patient with RA who had been treated with methotrexate (MTX) and tacrolimus (TAC) and who developed whole body lymphadenopathy. We simultaneously confirmed angioimmunoblastic T-cell lymphoma (AITL) through a right cervical lymph node biopsy and Epstein-Barr virus-positive B-cell lymphoproliferative disorder (EBV-positive B-LPD) through a bone marrow examination. After cessation of immunosuppressant therapy, both LPDs completely disappeared. Patients with AITL are occasionally reported to develop B-cell lymphoma through reactivation of the EBV, which leads to clonal expansion in the microenvironment. Immunohistochemistry results revealed that both LPD components were positive for EBV-encoded RNA. Moreover, in this patient, the plasma EBV DNA level was found to be high; therefore, EBV infection was a probable etiology. Synchronous coexistence of AITL and B-LPD as an OIIA-LPD has rarely been reported. This case report is the first to discuss the disappearance of both LPDs on withdrawal of immunosuppressants only. AITL occasionally accompany B-LPD; however, this composite lymphoma comprised AITL and B-LPD, and OIIA-LPDs should not be overlooked.
免疫抑制剂被广泛用于治疗类风湿关节炎(RA)患者,其不良反应已知会导致其他医源性免疫缺陷相关的淋巴增殖性疾病(OIIA-LPDs)。我们报告了一名接受甲氨蝶呤(MTX)和他克莫司(TAC)治疗的RA患者,该患者出现了全身淋巴结肿大。我们通过右颈淋巴结活检同时确诊了血管免疫母细胞性T细胞淋巴瘤(AITL),并通过骨髓检查确诊了EB病毒阳性B细胞淋巴增殖性疾病(EBV阳性B-LPD)。停用免疫抑制治疗后,两种淋巴增殖性疾病均完全消失。偶尔有报道称AITL患者会通过EBV重新激活而发生B细胞淋巴瘤,这会导致微环境中的克隆性扩增。免疫组化结果显示,两种淋巴增殖性疾病成分的EBV编码RNA均为阳性。此外,在该患者中,发现血浆EBV DNA水平较高;因此,EBV感染可能是病因。AITL和B-LPD作为OIIA-LPD的同步共存很少被报道。本病例报告首次讨论了仅停用免疫抑制剂后两种淋巴增殖性疾病均消失的情况。AITL偶尔会伴有B-LPD;然而,这种复合淋巴瘤由AITL和B-LPD组成,OIIA-LPDs不应被忽视。