Contemporary diagnostic methods for hemophagocytic lymphohistiocytic disorders

Theodore S. Johnson, Joyce Villanueva, Alexandra H. Filipovich, Rebecca A. Marsh, Jack J. Bleesing

Research output: Contribution to journalReview article

28 Citations (Scopus)

Abstract

Hemophagocytic lymphohistiocytosis is a life-threatening multi-system hyperinflammatory disorder characterized by dysfunctional cytolytic lymphocyte responses, hypercytokinemia, and widespread lymphohistiocytic tissue infiltration and destruction. Diagnosis and definitive therapy are often delayed as clinical efforts are directed toward treatment of presumed overwhelming infection. Sporadic cases occur in association with underlying immune dysfunction related to autoimmune disease, malignancy, or severe infection. However, familial cases predominate with remarkable associations between underlying genetic defects and dysregulation of immune responses. Here, we review the genetic and immunologic basis of contemporary diagnostic methods for hemophagocytic lymphohistiocytosis.

Original languageEnglish (US)
Pages (from-to)1-13
Number of pages13
JournalJournal of Immunological Methods
Volume364
Issue number1-2
DOIs
StatePublished - Feb 1 2011

Fingerprint

Hemophagocytic Lymphohistiocytosis
Infection
Autoimmune Diseases
Lymphocytes
Neoplasms
Therapeutics

Keywords

  • HLH
  • Hemophagocytic lymphohistiocytosis
  • Hemophagocytosis
  • Histiocytosis
  • Macrophage
  • T cell

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology

Cite this

Contemporary diagnostic methods for hemophagocytic lymphohistiocytic disorders. / Johnson, Theodore S.; Villanueva, Joyce; Filipovich, Alexandra H.; Marsh, Rebecca A.; Bleesing, Jack J.

In: Journal of Immunological Methods, Vol. 364, No. 1-2, 01.02.2011, p. 1-13.

Research output: Contribution to journalReview article

Johnson, Theodore S. ; Villanueva, Joyce ; Filipovich, Alexandra H. ; Marsh, Rebecca A. ; Bleesing, Jack J. / Contemporary diagnostic methods for hemophagocytic lymphohistiocytic disorders. In: Journal of Immunological Methods. 2011 ; Vol. 364, No. 1-2. pp. 1-13.
@article{89dcdfb830d24337ab5d3345b895289f,
title = "Contemporary diagnostic methods for hemophagocytic lymphohistiocytic disorders",
abstract = "Hemophagocytic lymphohistiocytosis is a life-threatening multi-system hyperinflammatory disorder characterized by dysfunctional cytolytic lymphocyte responses, hypercytokinemia, and widespread lymphohistiocytic tissue infiltration and destruction. Diagnosis and definitive therapy are often delayed as clinical efforts are directed toward treatment of presumed overwhelming infection. Sporadic cases occur in association with underlying immune dysfunction related to autoimmune disease, malignancy, or severe infection. However, familial cases predominate with remarkable associations between underlying genetic defects and dysregulation of immune responses. Here, we review the genetic and immunologic basis of contemporary diagnostic methods for hemophagocytic lymphohistiocytosis.",
keywords = "HLH, Hemophagocytic lymphohistiocytosis, Hemophagocytosis, Histiocytosis, Macrophage, T cell",
author = "Johnson, {Theodore S.} and Joyce Villanueva and Filipovich, {Alexandra H.} and Marsh, {Rebecca A.} and Bleesing, {Jack J.}",
year = "2011",
month = "2",
day = "1",
doi = "10.1016/j.jim.2010.11.006",
language = "English (US)",
volume = "364",
pages = "1--13",
journal = "Journal of Immunological Methods",
issn = "0022-1759",
publisher = "Elsevier",
number = "1-2",

}

TY - JOUR

T1 - Contemporary diagnostic methods for hemophagocytic lymphohistiocytic disorders

AU - Johnson, Theodore S.

AU - Villanueva, Joyce

AU - Filipovich, Alexandra H.

AU - Marsh, Rebecca A.

AU - Bleesing, Jack J.

PY - 2011/2/1

Y1 - 2011/2/1

N2 - Hemophagocytic lymphohistiocytosis is a life-threatening multi-system hyperinflammatory disorder characterized by dysfunctional cytolytic lymphocyte responses, hypercytokinemia, and widespread lymphohistiocytic tissue infiltration and destruction. Diagnosis and definitive therapy are often delayed as clinical efforts are directed toward treatment of presumed overwhelming infection. Sporadic cases occur in association with underlying immune dysfunction related to autoimmune disease, malignancy, or severe infection. However, familial cases predominate with remarkable associations between underlying genetic defects and dysregulation of immune responses. Here, we review the genetic and immunologic basis of contemporary diagnostic methods for hemophagocytic lymphohistiocytosis.

AB - Hemophagocytic lymphohistiocytosis is a life-threatening multi-system hyperinflammatory disorder characterized by dysfunctional cytolytic lymphocyte responses, hypercytokinemia, and widespread lymphohistiocytic tissue infiltration and destruction. Diagnosis and definitive therapy are often delayed as clinical efforts are directed toward treatment of presumed overwhelming infection. Sporadic cases occur in association with underlying immune dysfunction related to autoimmune disease, malignancy, or severe infection. However, familial cases predominate with remarkable associations between underlying genetic defects and dysregulation of immune responses. Here, we review the genetic and immunologic basis of contemporary diagnostic methods for hemophagocytic lymphohistiocytosis.

KW - HLH

KW - Hemophagocytic lymphohistiocytosis

KW - Hemophagocytosis

KW - Histiocytosis

KW - Macrophage

KW - T cell

UR - http://www.scopus.com/inward/record.url?scp=78650811211&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=78650811211&partnerID=8YFLogxK

U2 - 10.1016/j.jim.2010.11.006

DO - 10.1016/j.jim.2010.11.006

M3 - Review article

C2 - 21110979

AN - SCOPUS:78650811211

VL - 364

SP - 1

EP - 13

JO - Journal of Immunological Methods

JF - Journal of Immunological Methods

SN - 0022-1759

IS - 1-2

ER -