Flow cytometric findings in hemophagocytic lymphohistiocytosis

Chad M. McCall, Shiyama Mudali, Robert J. Arceci, Donald Small, Shirley Fuller, Christopher Gocke, Milena Vuica-Ross, Kathleen 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 2012

Fingerprint

Hemophagocytic Lymphohistiocytosis
Bone Marrow
Hematologic Neoplasms
Human Herpesvirus 4
Myelopoiesis
Lymphocytosis
Epstein-Barr Virus Infections
Lymphoma
Tuberculosis
T-Lymphocytes
Infection

Keywords

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

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

Cite this

Flow cytometric findings in hemophagocytic lymphohistiocytosis. / McCall, Chad M.; Mudali, Shiyama; Arceci, Robert J.; Small, Donald; Fuller, Shirley; Gocke, Christopher; Vuica-Ross, Milena; Burns, Kathleen; Borowitz, Michael J; Duffield, Amy S.

In: American Journal of Clinical Pathology, Vol. 137, No. 5, 05.2012, p. 786-794.

Research output: Contribution to journalArticle

McCall, Chad M. ; Mudali, Shiyama ; Arceci, Robert J. ; Small, Donald ; Fuller, Shirley ; Gocke, Christopher ; Vuica-Ross, Milena ; Burns, Kathleen ; Borowitz, Michael J ; Duffield, Amy S. / Flow cytometric findings in hemophagocytic lymphohistiocytosis. In: American Journal of Clinical Pathology. 2012 ; Vol. 137, No. 5. pp. 786-794.
@article{2a26c4c6c2f3490eb2a0847a4508a927,
title = "Flow cytometric findings in hemophagocytic lymphohistiocytosis",
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.",
keywords = "Epstein-Barr virus, Flow cytometry, Hematology, Hematopathology, Hemophagocytic lymphohistiocytosis",
author = "McCall, {Chad M.} and Shiyama Mudali and Arceci, {Robert J.} and Donald Small and Shirley Fuller and Christopher Gocke and Milena Vuica-Ross and Kathleen Burns and Borowitz, {Michael J} and Duffield, {Amy S}",
year = "2012",
month = "5",
doi = "10.1309/AJCPP40MEXWYRLPN",
language = "English (US)",
volume = "137",
pages = "786--794",
journal = "American Journal of Clinical Pathology",
issn = "0002-9173",
publisher = "American Society of Clinical Pathologists",
number = "5",

}

TY - JOUR

T1 - Flow cytometric findings in hemophagocytic lymphohistiocytosis

AU - McCall, Chad M.

AU - Mudali, Shiyama

AU - Arceci, Robert J.

AU - Small, Donald

AU - Fuller, Shirley

AU - Gocke, Christopher

AU - Vuica-Ross, Milena

AU - Burns, Kathleen

AU - Borowitz, Michael J

AU - Duffield, Amy S

PY - 2012/5

Y1 - 2012/5

N2 - 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.

AB - 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.

KW - Epstein-Barr virus

KW - Flow cytometry

KW - Hematology

KW - Hematopathology

KW - Hemophagocytic lymphohistiocytosis

UR - http://www.scopus.com/inward/record.url?scp=84862588686&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84862588686&partnerID=8YFLogxK

U2 - 10.1309/AJCPP40MEXWYRLPN

DO - 10.1309/AJCPP40MEXWYRLPN

M3 - Article

C2 - 22523218

AN - SCOPUS:84862588686

VL - 137

SP - 786

EP - 794

JO - American Journal of Clinical Pathology

JF - American Journal of Clinical Pathology

SN - 0002-9173

IS - 5

ER -