A γ/δ T-cell Receptor Prolymphocytic Leukemia and CD4-/CD8- Double-negative Immunophenotype in a Pediatric Patient

Asher M. Moser, Abed A. Quider, Jeremiah A. Groen, George Shubinsky, Joseph Kapelushnik

Research output: Contribution to journalArticlepeer-review

4 Scopus citations

Abstract

T-cell prolymphocytic leukemia is a very rare neoplasm, peaking in the seventh decade. An extensive search failed to find any report of this malignancy in the pediatric population. The malignant cell is morphologically characterized by a high nucleocytopasmic ratio, condensed chromatin, a single nucleolus, and nongranular basophilic cytoplasm. Cells are usually positive for the α/β and only rarely to the γ/δ T-cell receptors. Most patients follow an aggressive clinical course, only some respond to anti-CD52. We present a 6-year-old boy with T-cell prolymphocytic leukemia. The malignant cells expressed a postthymic immunophenotype (CD4-/CD8-) and positivity for the γ/δ T-cell receptors. The child died after 8 months despite aggressive chemotherapy, anti-CD52, and an allogeneic bone marrow transplant.

Original languageEnglish
Pages (from-to)e218-e219
JournalJournal of Pediatric Hematology/Oncology
Volume37
Issue number4
DOIs
StatePublished - 1 Dec 2015

Keywords

  • T-cell prolymphocytic leukemia
  • double-negative CD4/CD8
  • pediatric
  • γ/δ T-cell receptor

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

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