An Autopsy Case of Wiskott-Aldrich Syndrome Revealing "FDC-Only Lymphoid Follicles" in Lymphoid Tissue: A Morphologic Correlate of Defective Immune Synapse. (May 2022)

• Record Type: Journal Article
• Authors: Jindal, Ankur K.; Rastogi, Pulkit; Anjani, Gummadi; Rikhi, Rashmi; Rawat, Amit; Ahluwalia, Jasmina
• Is Part Of: Pediatric and developmental pathology. Volume 25:Number 3(2022)
• Page Start: 345
• Page End: 350
• Publication Date: 2022-05
• Subjects: Follicular dendritic cell-only lymphoid follicles -- immune synapse -- intracerebral hemorrhage -- lymphoid tissue -- Wiskott-Aldrich syndrome -- histopathology; Pediatric pathology -- Periodicals; Children -- Diseases -- Periodicals; Diagnosis, Laboratory -- Periodicals; Abnormalities, Human -- Periodicals; Child development -- Periodicals; Pediatrics -- Periodicals; 616.07
• DOI: 10.1177/10935266211058345
• Languages: English
• ISSNs: 1093-5266

Abstract

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.