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