title: Precursor B-cell lymphoblastic lymphoma. A study of nine cases lacking blood and bone marrow involvement and review of the literature.
abstract: We describe 9 cases of precursor B-cell lymphoblastic lymphoma (LYL) without evidence of marrow or blood involvement. Four patients had superficial nodal disease, 2 cutaneous involvement, and 1 each ovarian, retroperitoneal, or tonsillar primary tumor. Six patients had limited disease; 3 patients were stage III. Immunophenotyping revealed a terminal deoxynucleotidyl transferase (TdT)-positive, immature B-cell population with variable expression of CD10, CD20, and CD45. All patients are in complete clinical remission (median follow-up, 14 months). A literature review yielded 105 patients with a diagnosis of precursor B-cell LYL based on less than 25% marrow involvement. Of these, 64% were younger than 18 years. Skin, lymph nodes, and bone were the most common sites of disease. Mediastinal involvement was uncommon. TdT, CD19, CD79a, CD10, and HLA-DR were the most frequently expressed antigens, while CD45 and CD20 were expressed in only two thirds of the cases. Cytogenetic analysis showed additional 21q material as a recurring karyotypic abnormality. At a median follow-up of 26 months, 74% of patients were alive; the median survival was 19 months for patients dying of disease. Comparison with precursor B-cell acute lymphoblastic leukemia showed several overlapping features, although distinct differences were identified.
authors.affiliation: Dept of Pathology, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX 75235, USA.
authors.forename: A
authors.initials: A
authors.lastname: Maitra
authors.forename: R W
authors.initials: RW
authors.lastname: McKenna
authors.forename: A G
authors.initials: AG
authors.lastname: Weinberg
authors.forename: N R
authors.initials: NR
authors.lastname: Schneider
authors.forename: S H
authors.initials: SH
authors.lastname: Kroft
mesh_terms: D000328:Adult; D000368:Aged; D000944:Antigens, Differentiation, B-Lymphocyte / Q000032:analysis; D001402:B-Lymphocytes / Q000145:classification; D019046:Bone Marrow Neoplasms / Q000175:diagnosis; D002051:Burkitt Lymphoma / Q000175:diagnosis; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D016130:Immunophenotyping; D007621:Karyotyping; D016393:Lymphoma, B-Cell / Q000097:blood / Q000145:classification / Q000175:diagnosis* / Q000235:genetics; D008297:Male; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000097:blood / Q000145:classification / Q000175:diagnosis* / Q000235:genetics; D012189:Retrospective Studies; D013234:Stem Cells / Q000145:classification; D016896:Treatment Outcome
chemical_list: D000944:Antigens, Differentiation, B-Lymphocyte