title: B-lymphoblastic leukemia/lymphoma associated with t(8;13)(p11;q12)/ ZMYM2 (ZNF198)-FGFR1 : rare case and review of the literature.
abstract: Myeloid and lymphoid neoplasms with fibroblastic growth factor receptor-1 (FGFR1) abnormalities originate from mutated pluripotent stem cells and have a heterogeneous clinical presentation. There are 12 identified partner genes commonly involved in FGFR1 translocation at an 8p11 breakpoint. In FGFR1-related neoplasms, T-lymphoblastic lymphoma with eosinophilia is the most common clinical scenario, whereas acute B-lymphoblastic leukemia/lymphoma (B-ALL/LBL) is rare. To date, only 7 cases of B-ALL/LBL with FGFR1 abnormalities have been reported. Here, we report an additional case of a 64-year-old gentleman with leukocytosis, eosinophilia and diffuse mediastinal and general lymphadenopathy. Bone marrow examination showed patchy infiltrates of immature precursors/blasts, along with myeloid/eosinophilic hyperplasia. Immunophenotyping confirmed increased B lymphoblasts (30-40%). Karyotyping revealed cytogenetic abnormalities, including t(8;13)(p11;q12)/ZMYM2 (ZNF198)-FGFR1 and trisomy 21. The patient did not respond to hyper-CVAD chemotherapy and within 4 months developed acute myelomonocytic leukemia and expired 11 months after the initial diagnosis. Similar cases from the literature are reviewed.
authors.affiliation: Department of Pathology, College of Medicine, University of South Florida, Tampa, Fla., USA.
authors.forename: Janese
authors.initials: J
authors.lastname: Trimaldi
authors.forename: Estrella M
authors.initials: EM
authors.lastname: Carballido
authors.forename: Jeremy W
authors.initials: JW
authors.lastname: Bowers
authors.forename: Arturo L
authors.initials: AL
authors.lastname: Anguiano
authors.forename: Zhong J
authors.initials: ZJ
authors.lastname: Zhang
authors.forename: Bijal D
authors.initials: BD
authors.lastname: Shah
authors.forename: Salvador
authors.initials: S
authors.lastname: Bruno
authors.forename: Alan F
authors.initials: AF
authors.lastname: List
authors.forename: Lynn C
authors.initials: LC
authors.lastname: Moscinski
authors.forename: Terrence
authors.initials: T
authors.lastname: Grady
authors.forename: Steven J
authors.initials: SJ
authors.lastname: Agosti
authors.forename: Loveleen
authors.initials: L
authors.lastname: Kang
authors.forename: Ling
authors.initials: L
authors.lastname: Zhang
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage; D001402:B-Lymphocytes / Q000378:metabolism / Q000473:pathology; D002882:Chromosomes, Human, Pair 13 / Q000235:genetics*; D002898:Chromosomes, Human, Pair 8 / Q000235:genetics*; D003520:Cyclophosphamide / Q000008:administration & dosage; D004268:DNA-Binding Proteins / Q000235:genetics*; D003907:Dexamethasone / Q000008:administration & dosage; D004314:Down Syndrome; D004317:Doxorubicin / Q000008:administration & dosage; D017809:Fatal Outcome; D006801:Humans; D007948:Leukemia, Monocytic, Acute / Q000188:drug therapy / Q000235:genetics / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009363:Neoplasm Proteins / Q000235:genetics*; D016609:Neoplasms, Second Primary / Q000188:drug therapy / Q000235:genetics / Q000473:pathology; D015452:Precursor B-Cell Lymphoblastic Leukemia-Lymphoma / Q000188:drug therapy / Q000235:genetics* / Q000473:pathology; D051496:Receptor, Fibroblast Growth Factor, Type 1 / Q000235:genetics*; D014157:Transcription Factors / Q000235:genetics*; D014178:Translocation, Genetic; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D004268:DNA-Binding Proteins; D009363:Neoplasm Proteins; D014157:Transcription Factors; C111777:ZMYM2 protein, human; D014750:Vincristine; D003907:Dexamethasone; D004317:Doxorubicin; D003520:Cyclophosphamide; C496348:FGFR1 protein, human; D051496:Receptor, Fibroblast Growth Factor, Type 1