title: A syndrome of lymphoblastic lymphoma, eosinophilia, and myeloid hyperplasia/malignancy associated with t(8;13)(p11;q11): description of a distinctive clinicopathologic entity.
abstract: 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.
authors.affiliation: Department of Medicine, Dana-Farber Cancer Institute, Boston, MA 02115, USA.
authors.forename: R C
authors.initials: RC
authors.lastname: Inhorn
authors.forename: J C
authors.initials: JC
authors.lastname: Aster
authors.forename: S A
authors.initials: SA
authors.lastname: Roach
authors.forename: C A
authors.initials: CA
authors.lastname: Slapak
authors.forename: R
authors.initials: R
authors.lastname: Soiffer
authors.forename: R
authors.initials: R
authors.lastname: Tantravahi
authors.forename: R M
authors.initials: RM
authors.lastname: Stone
mesh_terms: D000208:Acute Disease; D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001853:Bone Marrow / Q000473:pathology*; D016026:Bone Marrow Transplantation; D016190:Carboplatin / Q000008:administration & dosage; D002882:Chromosomes, Human, Pair 13 / Q000648:ultrastructure*; D002898:Chromosomes, Human, Pair 8 / Q000648:ultrastructure*; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000008:administration & dosage; D003561:Cytarabine / Q000008:administration & dosage; D003630:Daunorubicin / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D004802:Eosinophilia / Q000235:genetics*; D005047:Etoposide / Q000008:administration & dosage; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use; D006965:Hyperplasia; D007069:Ifosfamide / Q000008:administration & dosage; D007951:Leukemia, Myeloid / Q000235:genetics; D008198:Lymph Nodes / Q000473:pathology; D008297:Male; D008875:Middle Aged; D008942:Mitoxantrone / Q000008:administration & dosage; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000235:genetics* / Q000628:therapy; D011241:Prednisone / Q000008:administration & dosage; D011247:Pregnancy; D011252:Pregnancy Complications, Neoplastic / Q000188:drug therapy* / Q000628:therapy; D012074:Remission Induction; D013577:Syndrome; D014178:Translocation, Genetic; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D003561:Cytarabine; D014750:Vincristine; D005047:Etoposide; D004317:Doxorubicin; D003520:Cyclophosphamide; D016190:Carboplatin; D008942:Mitoxantrone; D007069:Ifosfamide; D011241:Prednisone; D006918:Hydroxyurea; D003630:Daunorubicin