Hemophagocytic lymphohistiocytosis: Difference between revisions

	WikiDoc Resources for Hemophagocytic lymphohistiocytosis
Articles
Most recent articles on Hemophagocytic lymphohistiocytosis Most cited articles on Hemophagocytic lymphohistiocytosis Review articles on Hemophagocytic lymphohistiocytosis Articles on Hemophagocytic lymphohistiocytosis in N Eng J Med, Lancet, BMJ
Media
Powerpoint slides on Hemophagocytic lymphohistiocytosis Images of Hemophagocytic lymphohistiocytosis Photos of Hemophagocytic lymphohistiocytosis Podcasts & MP3s on Hemophagocytic lymphohistiocytosis Videos on Hemophagocytic lymphohistiocytosis
Evidence Based Medicine
Cochrane Collaboration on Hemophagocytic lymphohistiocytosis Bandolier on Hemophagocytic lymphohistiocytosis TRIP on Hemophagocytic lymphohistiocytosis
Clinical Trials
Ongoing Trials on Hemophagocytic lymphohistiocytosis at Clinical Trials.gov Trial results on Hemophagocytic lymphohistiocytosis Clinical Trials on Hemophagocytic lymphohistiocytosis at Google
Guidelines / Policies / Govt
US National Guidelines Clearinghouse on Hemophagocytic lymphohistiocytosis NICE Guidance on Hemophagocytic lymphohistiocytosis NHS PRODIGY Guidance FDA on Hemophagocytic lymphohistiocytosis CDC on Hemophagocytic lymphohistiocytosis
Books
Books on Hemophagocytic lymphohistiocytosis
News
Hemophagocytic lymphohistiocytosis in the news Be alerted to news on Hemophagocytic lymphohistiocytosis News trends on Hemophagocytic lymphohistiocytosis
Commentary
Blogs on Hemophagocytic lymphohistiocytosis
Definitions
Definitions of Hemophagocytic lymphohistiocytosis
Patient Resources / Community
Patient resources on Hemophagocytic lymphohistiocytosis Discussion groups on Hemophagocytic lymphohistiocytosis Patient Handouts on Hemophagocytic lymphohistiocytosis Directions to Hospitals Treating Hemophagocytic lymphohistiocytosis Risk calculators and risk factors for Hemophagocytic lymphohistiocytosis
Healthcare Provider Resources
Symptoms of Hemophagocytic lymphohistiocytosis Causes & Risk Factors for Hemophagocytic lymphohistiocytosis Diagnostic studies for Hemophagocytic lymphohistiocytosis Treatment of Hemophagocytic lymphohistiocytosis
Continuing Medical Education (CME)
CME Programs on Hemophagocytic lymphohistiocytosis
International
Hemophagocytic lymphohistiocytosis en Espanol Hemophagocytic lymphohistiocytosis en Francais
Business
Hemophagocytic lymphohistiocytosis in the Marketplace Patents on Hemophagocytic lymphohistiocytosis
Experimental / Informatics
List of terms related to Hemophagocytic lymphohistiocytosis

	Hemophagocytic lymphohistiocytosis;
	Light microscopic image of bone marrow showing stromal macrophages containing numerous red blood cells in their cytoplasm
ICD-10	D76.1
ICD-9	288.4
OMIM	267700 603552
DiseasesDB	31418
MeSH	D051359

← Older edit Newer edit →

VisualWikitext

Revision as of 22:09, 31 July 2012

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [2]

Overview

Hemophagocytic lymphohistiocytosis (HLH), also known as hemophagocytic syndrome,^[1] is an uncommon hematologic disorder

Classification

HLH comprises

Familial (primary) hemophagocytic lymphohistiocytosis (FHL)

Secondary HLH (SHLH)

Familial forms

FHL, an autosomal recessive disorder, is invariably fatal when untreated. It is associated with defective triggering of apoptosis and reduced cytotoxic activity, resulting in a widespread accumulation of T lymphocytes and activated macrophages.

There are four types, and each is associated with a specific gene:

FHL1 - HPLH1
FHL2 - PRF1
FHL3 - UNC13D
FHL4 - STX11

Familial hemophagocytic lymphohistiocytosis has an autosomal recessive pattern of inheritance.

References

↑ Fisman DN (2000). "Hemophagocytic syndromes and infection". Emerging Infect. Dis. 6 (6): 601–8. doi:10.3201/eid0606.000608. PMC 2640913. PMID 11076718.

Fisman DN (2000). "Hemophagocytic syndromes and infection". Emerging Infect. Dis. 6 (6): 601–608. PMID 11076718.

Henter JI, Samuelsson-Horne A, Aricò M; et al. (2002). "Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation". Blood. 100 (7): 2367–2373. doi:10.1182/blood-2002-01-0172. PMID 12239144.

External links

Template:Hematology

Template:WikiDoc Sources

[1] Fisman DN (2000). "Hemophagocytic syndromes and infection". Emerging Infect. Dis. 6 (6): 601–8. doi:10.3201/eid0606.000608. PMC 2640913. PMID 11076718.

[1]

@@ Line 17: / Line 17: @@
 ==Overview==
-'''Hemophagocytic lymphohistiocytosis''' (HLH) is an unusual syndrome characterized by [[fever]], [[splenomegaly]], [[jaundice]], and the pathologic finding of hemophagocytosis. This is [[phagocytosis]] by [[macrophage]]s of [[erythrocyte]]s, [[leukocyte]]s, [[platelet]]s, and their precursors in [[bone marrow]] and other tissues.
+'''Hemophagocytic lymphohistiocytosis''' (HLH), also known as '''hemophagocytic syndrome''',<ref>{{cite journal |author=Fisman DN |title=Hemophagocytic syndromes and infection |journal=Emerging Infect. Dis. |volume=6 |issue=6 |pages=601–8 |year=2000 |pmid=11076718 |url=http://www.cdc.gov/ncidod/eid/vol6no6/fisman.htm |doi=10.3201/eid0606.000608 |pmc=2640913}}</ref> is an uncommon [[hematologic disorder]]
-HLH may be associated with malignant, genetic, or autoimmune diseases. It is also prominently linked with [[Epstein-Barr virus]] (EBV) infection, however it can also be associated with other viruses as well as fungal infections.
-Hyperproduction of [[cytokine]]s, including interferon-g and tumor necrosis factor-a, by EBV-infected [[T lymphocytes]] may play a role in the pathogenesis of HLH. EBV-associated HLH may mimic [[T-cell lymphoma]] and is treated with cytotoxic chemotherapy. In contrast, hemophagocytic syndromes associated with nonviral pathogens often respond to treatment of the underlying infection.
 ==Classification==


Hemophagocytic lymphohistiocytosis
Light microscopic image of bone marrow showing stromal macrophages containing numerous red blood cells in their cytoplasm
ICD-10	D76.1
ICD-9	288.4
OMIM	267700 603552
DiseasesDB	31418
MeSH	D051359