Department of Adult Hematology, Hôpital Necker, Université Paris Descartes, Faculté de Médecine, Assistance Publique-Hôpitaux de Paris, Paris, France.
Pediatr Blood Cancer. 2010 Jul 1;54(7):1020-2. doi: 10.1002/pbc.22445.
Mutations of Fas or, less frequently, Fas ligand genes result in a rare inherited lymphoid disorder called autoimmune lymphoproliferative syndrome (ALPS) in which lymphoma frequency is increased. We report on a patient with ALPS who had been splenectomized for giant splenomegaly and progressively developed a voluminous abdominal tumor. The histology of the removed tumor revealed that it was an accessory spleen exhibiting typical features of ALPS involvement, as shown by the presence of a large excess of CD3+CD4-CD8- T cells and plasma cells without a detectable monoclonal population. This observation highlights the lymphoma's differential diagnosis in this context.
Fas 或 Fas 配体基因的突变导致一种罕见的遗传性淋巴组织疾病，称为自身免疫性淋巴增生综合征（ALPS），其中淋巴瘤的频率增加。我们报告了一名患有 ALPS 的患者，因巨大脾肿大而行脾切除术，随后逐渐出现巨大的腹部肿瘤。切除的肿瘤组织学显示它是一个副脾，表现出典型的 ALPS 受累特征，表现为大量 CD3+CD4-CD8-T 细胞和浆细胞，而无可检测到的单克隆群体。这一观察结果强调了在这种情况下淋巴瘤的鉴别诊断。
