title: Epstein-Barr virus polymorphic B-cell lymphoma associated with leukemia and with congenital immunodeficiencies.
abstract: Polymorphic B-cell lymphoma seen in four patients with congenital immunodeficiencies and in two patients with leukemia receiving chemotherapy was associated with the Epstein-Barr virus (EBV). The tumors had characteristic histologic features: they were polymorphic consisting of a mixture of lymphoblasts and differentiated cells including plasma cells, and areas of hemorrhagic necrosis were prominent. The tumors were either polyclonal, monoclonal, or multiclonal. Patients with congenital immunodeficiencies who developed these tumors died despite radiotherapy, corticosteroids plus acyclovir, or a combination of intravenous (IV) immunoglobulins and alpha 2 interferon. Patients with leukemia recovered when immunosuppressive drugs were discontinued and leukemia has not recurred over a period of 2 and 4 years, respectively, in the two patients.
authors.affiliation: Department of Microbiology, Hôpital Sainte-Justine, Montreal, Quebec, Canada.
authors.forename: J H
authors.initials: JH
authors.lastname: Joncas
authors.forename: P
authors.initials: P
authors.lastname: Russo
authors.forename: P
authors.initials: P
authors.lastname: Brochu
authors.forename: P
authors.initials: P
authors.lastname: Simard
authors.forename: J
authors.initials: J
authors.lastname: Brisebois
authors.forename: J
authors.initials: J
authors.lastname: Dubé
authors.forename: D
authors.initials: D
authors.lastname: Marton
authors.forename: J M
authors.initials: JM
authors.lastname: Leclerc
authors.forename: H
authors.initials: H
authors.lastname: Hume
authors.forename: G E
authors.initials: GE
authors.lastname: Rivard
mesh_terms: D000970:Antineoplastic Agents / Q000008:administration & dosage; D001402:B-Lymphocytes; D001932:Brain Neoplasms / Q000150:complications* / Q000473:pathology; D002051:Burkitt Lymphoma / Q000150:complications* / Q000473:pathology; D002675:Child, Preschool; D005260:Female; D004854:Herpesvirus 4, Human; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications / Q000151:congenital*; D007223:Infant; D007945:Leukemia, Lymphoid / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D008297:Male
chemical_list: D000970:Antineoplastic Agents