间变性淋巴瘤激酶 (ALK) 阴性间变性大细胞淋巴瘤 (ALCL) 是非霍奇金淋巴瘤的一种罕见亚型，胰腺受累极为罕见，仅在少数病例报告中记录。我们介绍了一名 31 岁人类免疫缺陷病毒 (HIV) 阳性男性的独特病例，患有多部位 ALK 阴性 ALCL，他最初表现为臀部溃疡，导致怀疑原发性皮肤 ALCL 或淋巴瘤样丘疹病。然而，多个皮外部位的发现，包括非典型胰头受累，证实了伴有皮肤表现的全身性 ALK 阴性 ALCL 的诊断。该患者接受了六个周期的 brentuximab vedotin 环磷酰胺-阿霉素-泼尼松 (BV CHP) 治疗，实现了胰头肿块大小的大幅减小，并且在正电子发射断层扫描 (PET) 扫描中未检测到氟脱氧葡萄糖 (FDG) 摄取。该病例强调了结外表现的 HIV 阳性患者中 ALK 阴性 ALCL 的诊断挑战，并证明了针对此类病例的靶向治疗策略的潜在有效性。版权所有 2023，Huang 等人。

Anaplastic lymphoma kinase (ALK)-negative anaplastic large cell lymphoma (ALCL) is an uncommon subtype of non-Hodgkin lymphoma, with pancreatic involvement being exceedingly rare and documented in only a handful of case reports. We present a unique case of a 31-year-old human immunodeficiency virus (HIV)-positive male with multisite ALK-negative ALCL, who initially presented with a buttock ulcer, leading to a suspicion of primary cutaneous ALCL or lymphomatoid papulosis. However, the discovery of multiple extracutaneous sites, including an atypical pancreatic head involvement, confirmed the diagnosis of systemic ALK-negative ALCL with cutaneous manifestation. The patient received six cycles of brentuximab vedotin + cyclophosphamide-doxorubicin-prednisone (BV + CHP) treatment, achieving a substantial reduction in the size of the pancreatic head mass and no detectable fluorodeoxyglucose (FDG) uptake on positron emission tomography (PET) scan. This case underscores the diagnostic challenges of ALK-negative ALCL in HIV-positive patients with extranodal presentations and demonstrates the potential effectiveness of targeted therapeutic strategies for such cases.Copyright 2023, Huang et al.