EPSTEIN-BARR VIRUS-ASSOCIATED MALIGNANT-LYMPHOMA WITH MACROAMYLASEMIAAND MONOCLONAL GAMMOPATHY IN A PATIENT WITH WISKOTT-ALDRICH SYNDROME

A 1-year-old boy with Wiskott-Aldrich Syndrome (WAS) who developed mal ignant lymphoma is described. He showed various complications such as atypical lymphocytosis, disseminated intravascular coagulation (DIC), intracranial hemorrhage, macroamylasemia, and monoclonal gammopathy (i mmunoglobulin A kappa chain). Epstein-Barr virus (EBV) DNA was detecte d in the tumor tissue, and the monoclonality of B cells from the tumor tissue was established. EBV-associated lymphoma is frequently observe d in immunocompromised patients including those with WAS. The developm ent of macroamylasemia, which is rare in childhood, is discussed in re lation to lymphoma and monoclonal gammopathy. This case is unique in t hat the EBV-associated malignant lymphoma developed at an early age an d was accompanied by macroamylasemia.