Diffuse Large B-Cell Lymphoma Associated with Wiskott-Aldrich Syndrome: A Case Report
Archives of Advances in Biosciences,
Vol. 16 No. 1 (2025),
2 March 2025
,
Page 1-3
https://doi.org/10.22037/aab.v16i1.47683
Abstract
Context: Wiskott-Aldrich syndrome (WAS) is an X-linked primary immunodeficiency characterized by microthrombocytopenia, eczema, recurrent infections, and a significant predisposition to autoimmune diseases and malignancies, particularly B-cell lymphomas.
Case presentation: We report the case of a 14-year-old male with a confirmed diagnosis of WAS since infancy, who later developed Crohn’s disease. He presented with vegetative lesions in the rectum and transverse colon, which were diagnosed as diffuse large B-cell lymphoma (DLBCL) based on immunohistochemistry and positive EBV-encoded small RNAs (EBER). The patient received 12 cycles of chemotherapy, including Rituximab and Etoposide, along with intravenous immunoglobulin (IVIG) support.
Conclusion: This is a rare case of EBV-associated DLBCL in a WAS patient from Iran. The case highlights the importance of early and routine malignancy screening in WAS patients, even in the absence of significant symptoms. Molecular and immunophenotypic assessments, including PCR and immunohistochemistry, are essential for accurate diagnosis and management.
- DLBCL
- Wiskott Aldrich syndrome
How to Cite
References
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