Devitt Katherine, Cerny Jan, Switzer Bradley, Ramanathan Muthalagu, Nath Rajneesh, Yu Hongbo, Woda Bruce A, Chen Benjamin J
Department of Pathology UMass Memorial Medical Center and University of Massachusetts Medical School, 1 Innovation Drive, Biotech 3, Worcester, MA 01605, USA.
Department of Hematology/Oncology, UMass Memorial Medical Center and University of Massachusetts Medical School, Worcester, MA 01605, USA.
Leuk Res Rep. 2014 Jun 2;3(2):42-5. doi: 10.1016/j.lrr.2014.05.004. eCollection 2014.
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening clinical syndrome characterized by dysregulation of the immune system. Impaired function of cytotoxic T cells and natural killer cells is often seen, and T-cell malignancies represent most cases of lymphoma-associated HLH. HLH associated with B-cell lymphoma is rare. We describe a case of a 30-year-old man who presented with fever, splenomegaly, and hyperferritinemia. Bone marrow biopsy revealed T-cell/histiocyte-rich large B-cell lymphoma, a rare, aggressive B-cell malignancy. This case highlights the interplay between a pro-inflammatory cytokine microenvironment and tumor-mediated immune suppression, and addresses the importance of accurately diagnosing these entities for appropriate clinical management.
噬血细胞性淋巴组织细胞增生症（HLH）是一种危及生命的临床综合征，其特征为免疫系统失调。常可见细胞毒性T细胞和自然杀伤细胞功能受损，且T细胞恶性肿瘤占大多数淋巴瘤相关HLH病例。与B细胞淋巴瘤相关的HLH较为罕见。我们报告一例30岁男性患者，其表现为发热、脾肿大和高铁蛋白血症。骨髓活检显示富含T细胞/组织细胞的大B细胞淋巴瘤，这是一种罕见的侵袭性B细胞恶性肿瘤。该病例突出了促炎细胞因子微环境与肿瘤介导的免疫抑制之间的相互作用，并强调了准确诊断这些疾病以进行适当临床管理的重要性。
