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   MeshID        = D051359 |
   MeshID        = D051359 |
}}
}}
{{SI}}
{{HPL}}
{{CMG}}; {{AE}} {{RT}}
{{CMG}}; {{AE}} {{RT}}


{{SK}} Hemophagocytic syndrome; familial histiocytic reticulosis; hemophagocytic lymphohistiocytosis - familial; erythrophagocytic lymphohistiocytosis -  familial; familial haemophagocytic lymphohistiocytosis; HLH


==Overview==
==[[Hemophagocytic lymphohistiocytosis overview|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 historical perspective|Historical Perspective]]==
 
==[[Hemophagocytic lymphohistiocytosis classification|Classification]]==
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.
==[[Hemophagocytic lymphohistiocytosis pathophysiology |Pathophysiology]]==
 
==[[Hemophagocytic lymphohistiocytosis causes|Causes]]==
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.
==[[Hemophagocytic lymphohistiocytosis differential diagnosis|Differentiating Hemophagocytic lymphohistiocytosis from other Diseases]]==
 
==[[Hemophagocytic lymphohistiocytosis epidemiology and demographics|Epidemiology and Demographics]]==
==Classification==
==[[Hemophagocytic lymphohistiocytosis risk factors|Risk Factors]]==
HLH comprises familial (primary) hemophagocytic lymphohistiocytosis (FHL) and secondary HLH (SHLH), both clinically characterized by the features described above, in particular fever, [[hepatosplenomegaly]], and [[cytopenia]].
==[[Hemophagocytic lymphohistiocytosis screening|Screening]]==
 
==[[Hemophagocytic lymphohistiocytosis natural history, complications and prognosis|Natural History, Complications and Prognosis]]==
===Familial forms===
==Diagnosis==
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.
[[Hemophagocytic lymphohistiocytosis history and symptoms|History and Symptoms]] | [[Hemophagocytic lymphohistiocytosis physical examination|Physical Examination]] | [[Hemophagocytic lymphohistiocytosis laboratory findings|Laboratory Findings]] | [[Hemophagocytic lymphohistiocytosis x ray|X Ray]] | [[Hemophagocytic lymphohistiocytosis CT|CT]] | [[Hemophagocytic lymphohistiocytosis MRI|MRI]] | [[Hemophagocytic lymphohistiocytosis other imaging findings|Other Imaging Findings]] | [[Hemophagocytic lymphohistiocytosis other diagnostic studies|Other Diagnostic Studies]]
 
There are four types, and each is associated with a specific gene:
 
* FHL1 - [[HPLH1]]
* FHL2 - [[PRF1]]
* FHL3 - [[UNC13D]]
* FHL4 - [[STX11]]
 
[[Image:autorecessive.jpg|thumb|left|Familial hemophagocytic lymphohistiocytosis has an autosomal recessive pattern of inheritance.]]
<br clear="left"/>
 
== References ==
{{reflist|2}}
* {{cite journal |author=Fisman DN |title=Hemophagocytic syndromes and infection |journal=Emerging Infect. Dis. |volume=6 |issue=6 |pages=601-608 |year=2000 |pmid=11076718 |doi= |url=http://www.cdc.gov/ncidod/eid/vol6no6/fisman.htm}}
 
* {{cite journal |author=Henter JI, Samuelsson-Horne A, Aricò M, ''et al'' |title=Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation |journal=Blood |volume=100 |issue=7 |pages=2367-2373 |year=2002 |pmid=12239144 |doi=10.1182/blood-2002-01-0172 |url=http://www.bloodjournal.org/cgi/pmidlookup?view=long&pmid=12239144}}
 
==External links==
* http://www.hrtrust.org/library/HLHSymptomsSignsAndDiagnosis.html
* {{eMedicine|ped|1997|Histiocytosis}}
* http://www.histio.org
* http://www.jonahchuang.com
* http://www.elyseyu.com
* http://www.ethanmichaelsmith.com
* http://www.savejonah.com


==Treatment==
[[Hemophagocytic lymphohistiocytosis medical therapy|Medical Therapy]] | [[Hemophagocytic lymphohistiocytosis surgery|Surgery]] | [[Hemophagocytic lymphohistiocytosis primary prevention|Primary Prevention]] | [[Hemophagocytic lymphohistiocytosis Secondary Prevention|Secondary Prevention]] | [[Hemophagocytic lymphohistiocytosis cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Hemophagocytic lymphohistiocytosis future or investigational therapies|Future or Investigational Therapies]]
==Case Studies==
[[Hemophagocytic lymphohistiocytosis case study one|Case #1]]


{{Hematology}}
{{Hematology}}


[[pl:Histiocytoza X]]


[[Category:Disease]]
[[Category:Pediatrics]]
[[Category:Pediatrics]]
[[Category:Hematology]]
[[Category:Hematology]]
[[Category:Blood disorders]]
[[Category:Genetic disorders]]
[[Category:Autosomal recessive disorders]]




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Latest revision as of 13:03, 23 June 2016

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

Hemophagocytic lymphohistiocytosis Microchapters

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Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Hemophagocytic lymphohistiocytosis from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms

Physical Examination

Laboratory Findings

X Ray

CT

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

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

Synonyms and keywords: Hemophagocytic syndrome; familial histiocytic reticulosis; hemophagocytic lymphohistiocytosis - familial; erythrophagocytic lymphohistiocytosis - familial; familial haemophagocytic lymphohistiocytosis; HLH

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Hemophagocytic lymphohistiocytosis from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms | Physical Examination | Laboratory Findings | X Ray | CT | MRI | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case #1

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