Surface immunoglobulin light chain-positive acute lymphoblastic leukemia of FAB L1 or L2 type: a report of 6 cases in adults

Am J Clin Pathol. 1998 Aug;110(2):143-9. doi: 10.1093/ajcp/110.2.143.

Abstract

Acute lymphoblastic leukemia (ALL) of B-cell lineage may be classified using the French-American-British (FAB) classification as L1, L2, or L3 type. L1 and L2 ALLs characteristically express terminal deoxynucleotidyl transferase (TdT) and are surface immunoglobulin (sIg)-negative. In contrast, L3 ALL is typically TdT-negative and sIg-positive. However, in a few large studies of children with ALL, sIg expression has been reported in less than 2% of L1 and L2 neoplasms. In these sIg-positive cases, IgM usually has been detected, with Ig light chain in a subset of tumors. Surface Ig expression in L1 or L2 ALL in adults is extremely rare; we found only 1 case report in the English literature. We report 6 cases of L1 or L2 ALL with an unusual immunophenotype arising in adults. In each tumor, the neoplastic cells expressed monotypic sIg light chain (4 lambda, 2 kappa) and TdT. Three tumors expressed CD34. Cytogenetic studies in 4 cases at diagnosis or relapse revealed no evidence of chromosomal translocations involving the c-myc locus, such as the t(8;14), t(2;8), or t(8;22). Three patients responded completely to chemotherapy and are alive; follow-up ranges from 18 to 57 months. Three patients died at 3, 13, and 14 months after diagnosis.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Adult
  • Female
  • Follow-Up Studies
  • Humans
  • Immunoglobulin Light Chains / metabolism*
  • Immunohistochemistry
  • Immunophenotyping
  • Karyotyping
  • Male
  • Polymerase Chain Reaction
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma / genetics
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma / metabolism*
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma / pathology
  • Surface Properties

Substances

  • Immunoglobulin Light Chains