15PPrimary skeletal muscle peripheral T-cell lymphoma associated with HIV presenting with myopathic symptoms

IF 2.8 4区医学 Q2 CLINICAL NEUROLOGY

Neuromuscular Disorders Pub Date : 2025-09-01 DOI:10.1016/j.nmd.2025.105479

G. Ji , F. Sun , X. Song

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Abstract

Primary skeletal muscle lymphoma (PSML) is a rare form of non-Hodgkin lymphoma (NHL), predominantly of B-cell lineage. T-cell variants are exceedingly uncommon, with fewer than ten reported cases of primary skeletal muscle peripheral T-cell lymphoma, not otherwise specified (PSM-PTCL, NOS). We present a case of HIV-associated PSM-PTCL, NOS with clinical features mimicking inflammatory myopathy. A 44-year-old man with a 5-year history of HIV presented with a 2-month history of intermittent fever and progressive limb weakness. Physical examination showed proximal-dominant muscle weakness and generalized edema. Laboratory tests revealed leukocytosis, anemia, elevated creatine kinase, metabolic acidosis, and negative myositis-specific autoantibodies. Electromyography showed a myopathic pattern. Due to rapidly worsening symptoms and lack of autoimmune markers, a muscle biopsy was performed. Histopathology showed diffuse infiltration of atypical lymphoid cells between muscle fibers. Immunohistochemistry revealed strong CD3 positivity and CD20 negativity, with high Ki-67 proliferation index (∼90%). Tumor cells were negative for CD10, CD117, CD34, CD68, CD99, pan-CK, MyoD1, MPO, TDT, and desmin, ruling out myogenic, myeloid, and epithelial malignancies. PET/CT revealed diffuse FDG uptake across multiple skeletal muscles with only minimal lymph node involvement. Bone marrow examination showed no infiltration. These findings confirmed the diagnosis of PSM-PTCL, NOS. Despite initiation of corticosteroids and chemotherapy, the disease progressed rapidly due to heavy tumor burden. The patient developed systemic complications and died shortly after discharge. This case underscores the diagnostic challenge in distinguishing aggressive lymphoma from inflammatory myopathies, particularly in immunocompromised individuals. Although HIV increases the risk of NHL, primary skeletal muscle involvement is rare and often misdiagnosed. Early biopsy is critical in patients with muscle-dominant symptoms and inconclusive autoimmune workup. This report emphasizes the need to consider lymphoma in the differential diagnosis of unexplained myopathic symptoms in HIV-positive patients.

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原发性骨骼肌外周t细胞淋巴瘤与HIV相关，表现为肌病症状

原发性骨骼肌淋巴瘤（PSML）是一种罕见的非霍奇金淋巴瘤（NHL），主要是b细胞谱系。t细胞变异非常罕见，只有不到10例报告的原发性骨骼肌外周t细胞淋巴瘤，没有其他指定（PSM-PTCL， NOS）。我们提出一例hiv相关的PSM-PTCL， NOS的临床特征模拟炎症性肌病。44岁男性，5年HIV感染史，2个月间歇性发热，进行性肢体无力。体格检查显示近端优势肌无力和全身性水肿。实验室检查显示白细胞增多、贫血、肌酸激酶升高、代谢性酸中毒和肌炎特异性自身抗体阴性。肌电图显示肌病型。由于症状迅速恶化和缺乏自身免疫标志物，进行了肌肉活检。组织病理显示肌纤维间弥漫非典型淋巴样细胞浸润。免疫组织化学显示强烈的CD3阳性和CD20阴性，Ki-67增殖指数高（约90%）。肿瘤细胞CD10、CD117、CD34、CD68、CD99、pan-CK、MyoD1、MPO、TDT和desmin均为阴性，排除了肌源性、髓系和上皮性恶性肿瘤。PET/CT显示弥漫性FDG摄取横跨多个骨骼肌，仅少量淋巴结受累。骨髓检查未见浸润。这些结果证实了PSM-PTCL， NOS的诊断。尽管开始使用皮质类固醇和化疗，但由于肿瘤负担过重，疾病进展迅速。患者出现全身并发症，出院后不久死亡。本病例强调了区分侵袭性淋巴瘤和炎性肌病的诊断挑战，特别是在免疫功能低下的个体中。虽然HIV增加了NHL的风险，但原发性骨骼肌受累是罕见的，而且经常被误诊。早期活检对有肌肉显性症状和自身免疫检查不确定的患者至关重要。本报告强调需要考虑淋巴瘤在鉴别诊断不明原因的肌病症状的hiv阳性患者。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Neuromuscular Disorders 医学-临床神经学

CiteScore

4.60

自引率

3.60%

发文量

543

审稿时长

53 days

期刊介绍： This international, multidisciplinary journal covers all aspects of neuromuscular disorders in childhood and adult life (including the muscular dystrophies, spinal muscular atrophies, hereditary neuropathies, congenital myopathies, myasthenias, myotonic syndromes, metabolic myopathies and inflammatory myopathies). The Editors welcome original articles from all areas of the field: • Clinical aspects, such as new clinical entities, case studies of interest, treatment, management and rehabilitation (including biomechanics, orthotic design and surgery). • Basic scientific studies of relevance to the clinical syndromes, including advances in the fields of molecular biology and genetics. • Studies of animal models relevant to the human diseases. The journal is aimed at a wide range of clinicians, pathologists, associated paramedical professionals and clinical and basic scientists with an interest in the study of neuromuscular disorders.