“FDC-only lymphoid follicles” in lymphoid tissue in a young boy with Wiskott-Aldrich Syndrome: a morphologic correlate of defective immune synapse

Wiskott-Aldrich Syndrome (WAS) is an inherited disorder characterized by classical triad of eczema, micro-thrombocytopenia, and immune-deficiency. This disease affects all the three hematopoietic lineages to a variable extent. The spectrum of clinical and laboratory data for WAS has been well described in the literature though there is paucity of its histopathologic correlates. The current case describes the autopsy findings of a case of WAS with specific recognition of altered histology noticed in the lympho-reticular tissues- ‘the follicular dendritic cell (FDC)-only lymphoid follicles.’ The possible mechanisms of this altered histology has been discussed.