Abstract
Approximately 15% of patients with Wiskott-Aldrich syndrome (WAS) develop malignant lymphomas. Both Epstein-Barr (EBV)-specific cellular and humoral immune defects were detected in a 13-year-old male patient who succumbed one year later to a lymphoproliferative disease involving lungs, hilar and mesenteric lymph nodes. A large cell lymphoma was found in the hilar lymph nodes. The lungs and mesenteric lymph nodes were infiltrated by a polymorphic population of lymphoid cells. Autopsy-derived specimens from spleen, liver, heart, lung, mediastinal and abdominal lymph nodes were examined. DNA isolated from tissues was tested for clonal rearrangement of joining region (JH) and constant mu (Cµ) region of immunoglobulin heavy chain and constant kappa (CK) region of immunoglobulin light chain by Southern blot hybridization. Two dominant clones with JH rearranged in different positions were found.KeywordsMesenteric Lymph NodeSouthern Blot HybridizationHilar Lymph NodeImmunoglobulin Heavy ChainAbdominal Lymph NodeThese keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
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