Wiskott-Aldrich Syndrome (WAS) is an inherited disorder characterized by the classical triad of eczema, micro-thrombocytopenia, and immune deficiency. This disease affects the hematopoietic cells to a variable extent. The spectrum of clinical and laboratory data for WAS has been well described in the literature though there is a paucity of its histopathologic and immunohistochemical correlates. The current case describes the autopsy findings of this rare entity in an 8-year old male child with specific recognition of altered histology noticed in the lymphoreticular tissues. The predominant morphological finding in lymphoid tissue was atretic hyalinized germinal centers labeled as “the follicular dendritic cell (FDC)-only lymphoid follicles.” Immunohistochemistry revealed a reduction in germinal-center B-cells, T-follicular helper cells, attenuated mantle zone, FDC proliferation, and paracortical plasmacytosis. This case highlights the crippled immune cell population in WAS, ultimately leading to the morphology of atretic follicles rich in FDCs.

Wiskott-Aldrich 综合征 (WAS) 是一种遗传性疾病，以湿疹、微血小板减少症和免疫缺陷的经典三联征为特征。这种疾病在不同程度上影响造血细胞。尽管缺乏其组织病理学和免疫组织化学相关性，但 WAS 的临床和实验室数据范围已在文献中得到很好的描述。本病例描述了对一名 8 岁男童的尸检结果，该男童特异性识别淋巴网状组织中发现的组织学改变。淋巴组织中的主要形态学发现是闭锁的透明生发中心，标记为“仅滤泡树突细胞 (FDC) 的淋巴滤泡”。免疫组织化学显示生发中心 B 细胞、T 滤泡辅助细胞、地幔区减弱、FDC 增殖和皮质旁浆细胞增多症。这个案例突出了 WAS 中受损的免疫细胞群，最终导致闭锁滤泡富含 FDCs 的形态。