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Precursor B-cell lymphoblastic lymphoma. A study of nine cases lacking blood and bone marrow involvement and review of the literature. Am J Clin Pathol 2001 Jun;115(6):868-75

Date

06/08/2001

Pubmed ID

11392884

DOI

10.1309/Q5GV-3K00-WAC6-BBUB

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.

Author List

Maitra A, McKenna RW, Weinberg AG, Schneider NR, Kroft SH

Author

Steven Howard Kroft MD Chair, Professor in the Pathology department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Adult
Aged
Antigens, Differentiation, B-Lymphocyte
B-Lymphocytes
Bone Marrow Neoplasms
Burkitt Lymphoma
Child
Child, Preschool
Diagnosis, Differential
Female
Humans
Immunohistochemistry
Immunophenotyping
Karyotyping
Lymphoma, B-Cell
Male
Precursor Cell Lymphoblastic Leukemia-Lymphoma
Retrospective Studies
Stem Cells
Treatment Outcome