Flow cytometric findings in hemophagocytic lymphohistiocytosis

Chad M. McCall, Shiyama Mudali, Robert J. Arceci, Donald Small, Shirley Fuller, Christopher D. Gocke, Milena Vuica-Ross, Kathleen H. Burns, Michael J. Borowitz, Amy S. Duffield

Research output: Contribution to journalArticle

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is an often fatal hyperinflammatory syndrome. HLH may be inherited, but it more commonly arises secondary to Epstein-Barr virus (EBV) or other infections, hematologic malignancies, or rheumatologic diseases. We identified 17 patients diagnosed with HLH who had flow cytometric analysis of peripheral blood or bone marrow performed at the time of diagnosis. Two patients had primary HLH, and the others had HLH secondary to EBV infection, hematologic malignancies, rheumatologic conditions, or tuberculosis. The marrow typically showed a reactive lymphocytosis and a marked left shift in myelopoiesis regardless of the etiology. Qualitative abnormalities were also found in several cases, including T-cell abnormalities in the majority of the EBV-associated HLH cases. While not specific, flow cytometric findings in HLH are different from the findings in uninvolved marrow samples, and care should be taken not to overinterpret immunophenotypic findings in these cases as indicative of a primary marrow disorder or lymphoma.

Original languageEnglish (US)
Pages (from-to)786-794
Number of pages9
JournalAmerican journal of clinical pathology
Volume137
Issue number5
DOIs
StatePublished - May 1 2012

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Keywords

  • Epstein-Barr virus
  • Flow cytometry
  • Hematology
  • Hematopathology
  • Hemophagocytic lymphohistiocytosis

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

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