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A syndrome of lymphoblastic lymphoma, eosinophilia, and myeloid
hyperplasia/malignancy associated with t(8;13)(p11;q11): description of a
distinctive clinicopathologic entity [see comments]
RC Inhorn, JC Aster, SA Roach, CA Slapak, R Soiffer, R Tantravahi and RM Stone
Department of Medicine, Dana-Farber Cancer Institute, Boston, MA 02115,
USA.
We report two patients with a distinctive biphenotypic hematologic disorder
characterized by lymphoblastic lymphoma (LBL), eosinophilia, and myeloid
malignancy and/or hyperplasia associated with a t(8;13)(p11;q11)
chromosomal translocation in both bone marrow and lymph node specimens.
Both patients presented with lymphadenopathy pathologically classified as
LBL with a T-cell immunophenotype, myeloid hyperplasia of the bone marrow,
and peripheral blood eosinophilia. The first patient achieved clinical
complete remission after receiving several regimens of chemotherapy and
remains disease-free 16 months after undergoing allogeneic bone marrow
transplantation. The second patient developed progressive lymphadenopathy
despite several courses of chemotherapy directed against non-Hodgkin's
lymphoma. Eight months after his initial presentation, he developed acute
myelogenous leukemia that was refractory to therapy. Comparison of these
patients with four similar cases recently reported in the literature
suggests that this constellation of findings constitutes a distinctive
clinicopathologic syndrome. Molecular analysis of the t(8;13) translocation
breakpoint may identify genes located in this region and provide insight
into the pathogenesis of this interesting biphenotypic hematologic
malignancy.
Volume 85,
Issue 7,
pp. 1881-1887,
04/01/1995
Copyright © 1995 by The American Society of Hematology
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