In:
Pediatric and Developmental Pathology, SAGE Publications, Vol. 25, No. 3 ( 2022-05), p. 345-350
Kurzfassung:
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.
Materialart:
Online-Ressource
ISSN:
1093-5266
,
1615-5742
DOI:
10.1177/10935266211058345
Sprache:
Englisch
Verlag:
SAGE Publications
Publikationsdatum:
2022
ZDB Id:
1480654-X