Mixed-phenotype large granular lymphocytic leukemia: a rare subtype in the large granular lymphocytic leukemia spectrum

Jadee L. Neff, Aruna Rangan, Dragan Jevremovic, Phuong L. Nguyen, April Chiu, Ronald S Go, Dong Chen, William G. Morice, Min Shi

Research output: Contribution to journalArticle

1 Scopus citations

Abstract

Large granular lymphocytic leukemia (LGLL) is a chronic proliferation of cytotoxic lymphocytes in which more than 70% of patients develop cytopenia(s) requiring therapy. LGLL includes T-cell LGLL and chronic lymphoproliferative disorder of natural killer (NK) cells. The neoplastic cells in LGLL usually exhibit a single immunophenotype in a patient, with CD8-positive/αβ T-cell type being the most common, followed by NK-cell, γδ T-cell, and CD4-positive/αβ T-cell types. We investigated a total of 220 LGLL cases and identified 12 mixed-phenotype LGLLs (5%): 7 cases with coexistent αβ T-cell and NK-cell clones and 5 with coexistent αβ and γδ T-cell clones. With a median follow-up of 48 months, the clinicopathological characteristics of these patients seemed similar to those of typical LGLL patients. Treatment was instituted in 9 patients, and 5 patients (55%) attained complete hematologic response or partial response. The therapeutic response rate of this cohort is comparable to the reported overall response rate of 40% to 60% in typical LGLL patients. Three patients who did not receive any treatment had progressive or persistent cytopenias. Interestingly, inverted proportions of 2 clones at disease recurrence were identified in 4 patients (36%) and stable clonal proportions in 7 patients (64%). Mixed-phenotype LGLL is rare, and this study underscores the importance of recognizing this rare type of LGLL in patients who may benefit from LGLL treatment.

Original languageEnglish (US)
Pages (from-to)96-104
Number of pages9
JournalHuman Pathology
Volume81
DOIs
StatePublished - Nov 1 2018

Keywords

  • CLPD-NK
  • Flow cytometry
  • Large granular lymphocytic leukemia
  • Mixed-phenotype
  • T-LGLL

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

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