S100-positive, T-cell chronic lymphoproliferative disease: An aggressive disorder of an uncommon T-cell subset

C. A. Hanson, P. L. Bockenstedt, B. Schnitzer, D. A. Fox, B. Kueck, D. K. Braun

Research output: Contribution to journalArticle

24 Scopus citations

Abstract

S100-positive T lymphocytes account for less than 3% of peripheral blood T cells. Rare cases of S100-positive T-cell lymphoma have been previously described. We report four such cases of S100-positive T-cell chronic lymphoproliferative disease. In all cases, hepatosplenomegaly was observed, without prominent lymphadenopathy. Central nervous system (CNS) involvement by the leukemic cells was suggested in three cases by physical symptoms and confirmed in two cases by cerebrospinal fluid studies. Despite treatment, three patients died at 3, 6, and 8 months after diagnosis. Although there was a leukemic presentation, only minimal bone marrow infiltration was evident. Splenectomy showed red pulp infiltration. Liver and lymph node biopsies showed sinusoidal leukemic involvement. In all cases, the leukemic cells expressed mature T-cell- and natural killer cell-associated antigens. Cytoplasmic S100 was detected in the leukemic cells in the blood, spleen, liver, and lymph node. Southern blot studies in two cases showed T-β, T-γ, and T-δ gene rearrangements. RNA Northern blots showed T-α and T-β chain transcripts with no T-γ or T-δ RNA identified. Southern blot analysis showed no hybridization to probes specific for Epstein-Barr virus, cytomegalovirus, human immunodeficiency virus-1, or human T-cell lymphotropic virus type-1. These findings show that S100-positive T-cell chronic lymphoproliferative disorder is an aggressive, extramedullary-based disease frequently associated with CNS involvement and characterized by short survivals.

Original languageEnglish (US)
Pages (from-to)1803-1813
Number of pages11
JournalBlood
Volume78
Issue number7
DOIs
StatePublished - 1991

ASJC Scopus subject areas

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology

Fingerprint Dive into the research topics of 'S100-positive, T-cell chronic lymphoproliferative disease: An aggressive disorder of an uncommon T-cell subset'. Together they form a unique fingerprint.

  • Cite this

    Hanson, C. A., Bockenstedt, P. L., Schnitzer, B., Fox, D. A., Kueck, B., & Braun, D. K. (1991). S100-positive, T-cell chronic lymphoproliferative disease: An aggressive disorder of an uncommon T-cell subset. Blood, 78(7), 1803-1813. https://doi.org/10.1182/blood.v78.7.1803.1803