OUP user menu

Precursor B-Cell Lymphoblastic Lymphoma
A Study of Nine Cases Lacking Blood and Bone Marrow Involvement and Review of the Literature

Anirban Maitra MD, Robert W. McKenna MD, Arthur G. Weinberg MD, Nancy R. Schneider MD, PhD, Steven H. Kroft MD
DOI: http://dx.doi.org/10.1309/Q5GV-3K00-WAC6-BBUB 868-875 First published online: 1 June 2001


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.

Key Words:
  • Lymphoblastic lymphoma
  • Precursor B-cell lymphoma
  • Precursor B-cell ALL
  • Acute lymphoblastic leukemia