title: CD7+ stem cell leukemia/lymphoma. Features of a subgroup without circulating blast cells.
abstract: Recent advances in immunology have clarified the cellular origin of hematopoietic neoplasms. Blast cells with a CD7+ CD4- CD8- phenotype are demonstrated to originate from malignant pluripotent hematopoietic stem cells. In this article, the authors describe three rare cases, designated as a lymphoma type of CD7+ stem cell leukemia/lymphoma, with clinical features described below. All three patients were admitted with non-Hodgkin lymphoma with a 2-month to 4-month history of lymphadenopathy. Histologic examination of lymph nodes showed lymphoblastic lymphoma (LBL) in all patients. Bone marrow blast cells had an immunophenotype consistent with CD7+ CD4- CD8- acute leukemia, although abnormal cells were not observed in the peripheral blood during the course of the disease. One patient had a recurrence in the bone marrow, with myeloperoxidase-positive blast cells expressing myeloid differentiation antigens. Chromosomal analysis detected a common abnormal karyotype initially and at relapse. Furthermore, the same T-cell receptor gene rearrangement was found initially and at relapse, suggesting that these blast cells originated from the same pluripotent leukemic clone. Additional studies on more patients are required to determine the clinical significance of this group, including the difference from CD7+ stem cell leukemia/lymphoma with circulating blast cells (leukemic type) or LBL.
authors.affiliation: Third Department of Internal Medicine, Faculty of Medicine, Kyushu University, Fukuoka, Japan.
authors.forename: M
authors.initials: M
authors.lastname: Katsuno
authors.forename: Y
authors.initials: Y
authors.lastname: Abe
authors.forename: F
authors.initials: F
authors.lastname: Taguchi
authors.forename: Y
authors.initials: Y
authors.lastname: Yufu
authors.forename: S
authors.initials: S
authors.lastname: Sadamura
authors.forename: T
authors.initials: T
authors.lastname: Goto
authors.forename: H
authors.initials: H
authors.lastname: Takatsuki
authors.forename: J
authors.initials: J
authors.lastname: Nishimura
authors.forename: J
authors.initials: J
authors.lastname: Hirata
authors.forename: T
authors.initials: T
authors.lastname: Akiyoshi
mesh_terms: D000208:Acute Disease; D000328:Adult; D015703:Antigens, CD / Q000032:analysis; D019011:Antigens, CD7; D000945:Antigens, Differentiation, T-Lymphocyte / Q000032:analysis; D001853:Bone Marrow / Q000276:immunology / Q000473:pathology*; D001856:Bone Marrow Examination; D005260:Female; D015326:Gene Rearrangement, B-Lymphocyte, Heavy Chain; D015329:Gene Rearrangement, T-Lymphocyte; D006801:Humans; D016130:Immunophenotyping; D007621:Karyotyping; D007938:Leukemia / Q000235:genetics / Q000276:immunology / Q000473:pathology*; D008228:Lymphoma, Non-Hodgkin / Q000235:genetics / Q000276:immunology / Q000473:pathology; D008297:Male; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000235:genetics / Q000276:immunology / Q000473:pathology
chemical_list: D015703:Antigens, CD; D019011:Antigens, CD7; D000945:Antigens, Differentiation, T-Lymphocyte