CD20-positive and CD4/CD8-double-negative peripheral T-cell lymphoma of spleen complicated with severe disseminated intravascular coagulation and enteropathy

J Clin Exp Hematop. 2012;52(2):133-6. doi: 10.3960/jslrt.52.133.

Abstract

A 71-year-old woman presented with massive splenomegaly. Open splenectomy was performed, and the diagnosis of peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS), was made, with a characteristic immunophenotype of CD3(+), CD4(-), CD8(-), T-cell receptor (TCR)αβ(+), and TCRγδ(-). After splenectomy, she suffered abrupt exacerbation of the lymphoma with disseminated intravascular coagulation and enteropathy. Although chemotherapy was started, her medical condition did not improve and she died a week later. Postmortem reevaluation of the pathological specimen confirmed her diagnosis as CD20(+) PTCL-NOS. Although it is a rare disease entity, CD20(+) T-cell lymphoma can demonstrate aggressive clinical behavior.

Publication types

  • Case Reports

MeSH terms

  • Acute Disease
  • Aged
  • Antigens, CD20 / genetics*
  • CD4 Antigens / genetics
  • CD8 Antigens / genetics
  • Disseminated Intravascular Coagulation / complications
  • Disseminated Intravascular Coagulation / immunology
  • Disseminated Intravascular Coagulation / pathology*
  • Fatal Outcome
  • Female
  • Gene Expression
  • Humans
  • Intestinal Diseases / complications
  • Intestinal Diseases / immunology
  • Intestinal Diseases / pathology*
  • Lymphoma, T-Cell, Peripheral / complications
  • Lymphoma, T-Cell, Peripheral / immunology
  • Lymphoma, T-Cell, Peripheral / pathology*
  • Receptors, Antigen, T-Cell / genetics
  • Spleen / immunology
  • Spleen / pathology*
  • Spleen / surgery
  • Splenectomy
  • Splenomegaly / complications
  • Splenomegaly / immunology
  • Splenomegaly / pathology
  • Splenomegaly / surgery

Substances

  • Antigens, CD20
  • CD4 Antigens
  • CD8 Antigens
  • Receptors, Antigen, T-Cell