Sharma Purva V, Witteman Michael, Sundaravel Swethika, Larocca Tulisa, Zhang Yuanming, Goldsztajn Harry
Department of Internal Medicine, University of Miami Palm Beach Regional Consortium, Atlantis, Fl, USA.
Department of Pathology, JFK Medical Center, Atlantis, Fl, USA.
Intractable Rare Dis Res. 2018 Feb;7(1):61-64. doi: 10.5582/irdr.2017.01077.
Adult T cell lymphoma (ATL), is a peripheral T cell neoplasm associated with infection by human T-lymphotropic virus (HTLV). This is a case of a 28-year-old lady who presented with back pain for the past month and recent onset weakness in her lower extremities bilaterally. She has a history of T-cell lymphoma secondary to HTLV-1 under remission since 2014 and systemic lupus erythematosus complicated by lupus nephritis. On physical examination patient had hyper-reflexia in both knees, ankle clonus bilaterally and spasticity in both her lower extremities. She also had a diffuse, scaly, macular rash in her upper and lower extremities and ulcer-like lesions on the plantar surface of both feet. Her lumbar puncture showed lymphocyte predominance. The Western Blot test was positive for HTLV antibodies in the CSF. The patient was started on IV Methylprednisone which considerably improved her symptoms. The biopsy of her skin lesions showed an immunophenotype of T-cells similar to the cells in the bone marrow at the time of diagnosis of the lymphoma. HTLV infection is an etiologic agent for ATL as well as for tropical spastic paresis. One should have a high degree of suspicion for tropical spastic paresis in patients with HTLV-1 infection as it can easily go undiagnosed. Indolent forms of ATL can also present in the form of skin lesions in later stages. It is also important to distinguish between skin manifestations of ATL and cutaneous T cell lymphomas, and the importance of skin biopsies for the same cannot be undermined.
成人T细胞淋巴瘤(ATL)是一种与人类嗜T淋巴细胞病毒(HTLV)感染相关的外周T细胞肿瘤。这是一例28岁女性患者,她在过去一个月出现背痛,近期双侧下肢出现无力。她有继发于HTLV-1的T细胞淋巴瘤病史,自2014年以来处于缓解期,还有系统性红斑狼疮合并狼疮性肾炎。体格检查时,患者双膝关节反射亢进,双侧踝阵挛,双下肢痉挛。她的上肢和下肢还出现了弥漫性、鳞屑性斑疹,双足底有溃疡样病变。腰椎穿刺显示淋巴细胞占优势。脑脊液的Western Blot检测显示HTLV抗体呈阳性。患者开始接受静脉注射甲基泼尼松治疗,症状有明显改善。她皮肤病变的活检显示T细胞免疫表型与淋巴瘤诊断时骨髓中的细胞相似。HTLV感染是ATL以及热带痉挛性轻瘫的病因。对于HTLV-1感染患者,应高度怀疑热带痉挛性轻瘫,因为它很容易漏诊。惰性形式的ATL在后期也可能以皮肤病变的形式出现。区分ATL的皮肤表现和皮肤T细胞淋巴瘤也很重要,皮肤活检对此的重要性不可忽视。