A SYNDROME OF LYMPHOBLASTIC LYMPHOMA, EOSINOPHILIA, AND MYELOID HYPERPLASIA MALIGNANCY ASSOCIATED WITH T(8-13) (P11-Q11) - DESCRIPTION OF ADISTINCTIVE CLINICOPATHOLOGICAL ENTITY

We report two patients with a distinctive biphenotypic hematologic dis order characterized by lymphoblastic lymphoma (LBL), eosinophilia, and myeloid malignancy and/or hyperplasia associated with a t(8;13)(p11;q 11) chromosomal translocation in both bone marrow and lymph node speci mens. Both patients presented with lymphadenopathy pathologically clas sified as LBL with a T-cell immunophenotype, myeloid hyperplasia of th e bone marrow, and peripheral blood eosinophilia. The first patient ac hieved clinical complete remission after receiving several regimens of chemotherapy and remains disease-free 16 months after undergoing allo geneic bone marrow transplantation. The second patient developed progr essive lymphadenopathy despite several courses of chemotherapy directe d against non-Hodgkin's lymphoma. Eight months after his initial prese ntation, he developed acute myelogenous leukemia that was refractory t o therapy. Comparison of these patients with four similar cases recent ly reported in the literature suggests that this constellation of find ings constitutes a distinctive clinicopathologic syndrome. Molecular a nalysis of the t(8;13) translocation breakpoint may identify genes loc ated in this region and provide insight into the pathogenesis of this interesting biphenotypic hematologic malignancy. (C) 1995 by The Ameri can Society of Hematology.