一例原发性皮肤γ-δ T细胞淋巴瘤合并噬血细胞性淋巴组织细胞增生症和心脏淀粉样变性的致死病例。

Gibson Juliet Fraser, Kapur Lucy, Sokhn Joseph, Xu Mina, Foss Francine M

Department of Medicine, Yale University School of Medicine New Haven, Connecticut.

Hematopathology, Department of Pathology, Yale University School of Medicine New Haven, Connecticut.

Clin Case Rep. 2015 Jan;3(1):34-8. doi: 10.1002/ccr3.142. Epub 2014 Nov 17.

Gamma-delta T-cell lymphomas (GD-TCL) are rare and rapidly fatal neoplasms that are often associated with Hemophagocytic Lymphohistiocytosis (HLH), a syndrome of fevers, cytopenias, and multiorgan failure that often leads to a rapid death. We report the first case demonstrating an association between GD-TCL, HLH, and cardiac amyloidosis, presenting a novel mechanism for rapid deterioration in these patients.

γδ T细胞淋巴瘤（GD-TCL）是一种罕见且致死率高的肿瘤，常与噬血细胞性淋巴组织细胞增生症（HLH）相关，HLH是一种以发热、血细胞减少和多器官功能衰竭为特征的综合征，常导致迅速死亡。我们报告了首例显示GD-TCL、HLH与心脏淀粉样变性之间存在关联的病例，为这些患者的快速病情恶化提出了一种新机制。