Camagna A, Cedrone L, Caré A, Samoggia P, De Marco M C, Del Duca P, De Martinis C, Testa U
Department of Clinical Sciences, University La Sapienza, Rome, Italy.
Br J Haematol. 2001 Jan;112(1):204-7. doi: 10.1046/j.1365-2141.2001.02483.x.
Evidence is accumulating regarding CD95/CD95 ligand (Fas/FasL) pathway dysregulation in clonal diseases of the lymphohaemopoietic lineages. According to these observations, it has been proposed that this defect may represent one of the mechanisms of tumour progression. In large granular lymphocyte (LGL) leukaemia, dysregulated apoptosis may represent a key event in the development of malignancy and autoimmunity. This case report describes dysregulation of the Fas/FasL pathway in a chronic polyclonal expansion of CD3(+) LGLs associated with numerous serological immune abnormalities.
关于淋巴造血谱系克隆性疾病中CD95/CD95配体（Fas/FasL）途径失调的证据正在不断积累。根据这些观察结果，有人提出这种缺陷可能是肿瘤进展的机制之一。在大颗粒淋巴细胞（LGL）白血病中，凋亡失调可能是恶性肿瘤和自身免疫发展中的关键事件。本病例报告描述了与多种血清学免疫异常相关的CD3(+) LGL慢性多克隆扩增中Fas/FasL途径的失调。
