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


{{SK}} Hemophagocytic syndrome, Familial histiocytic reticulosis, Hemophagocytic lymphohistiocytosis - familial, Erythrophagocytic lymphohistiocytosis -  familial, Familial haemophagocytic lymphohistiocytosis
{{SK}} Hemophagocytic syndrome; familial histiocytic reticulosis; hemophagocytic lymphohistiocytosis - familial; erythrophagocytic lymphohistiocytosis -  familial; familial haemophagocytic lymphohistiocytosis; HLH
 
==Overview==
'''Hemophagocytic lymphohistiocytosis''' (HLH)<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]] in which the immune system produces too many activated immune cells ([[lymphocytes]]) called [[T cells]], [[natural killer cells]], [[B cells]], and [[macrophages]] ([[histiocytes]]).  Excessive amounts of immune system proteins called [[cytokines]] are also produced.
 
==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]]
 
[[Image:autorecessive.jpg|thumb|left|Familial hemophagocytic lymphohistiocytosis has an autosomal recessive pattern of inheritance.]]
<br clear="left"/>
 
==Pathophysiology==
Excessive production of too many activated immune cells ([[lymphocytes]]) called [[T cells]], [[natural killer cells]], [[B cells]], and [[macrophages]] ([[histiocytes]]) and [[cytokines]] by the immune system causes symptoms and damages [[liver]] and [[spleen]] and causes these organs to enlarge.  Familial hemophagocytic lymphohistiocytosis also destroys blood-producing cells in the bone marrow, a process called hemophagocytosis. As a result, affected individuals have low numbers of red blood cells ([[anemia]]) and a reduction in the number of blood cells involved in clotting (platelets). A reduction in platelets may cause easy bruising and abnormal [[bleeding]].
 
The [[brain]] may also be affected in familial hemophagocytic lymphohistiocytosis causing various symptoms and complications.


==[[Hemophagocytic lymphohistiocytosis overview|Overview]]==
==[[Hemophagocytic lymphohistiocytosis historical perspective|Historical Perspective]]==
==[[Hemophagocytic lymphohistiocytosis classification|Classification]]==
==[[Hemophagocytic lymphohistiocytosis pathophysiology |Pathophysiology]]==
==[[Hemophagocytic lymphohistiocytosis causes|Causes]]==
==[[Hemophagocytic lymphohistiocytosis differential diagnosis|Differentiating Hemophagocytic lymphohistiocytosis from other Diseases]]==
==[[Hemophagocytic lymphohistiocytosis epidemiology and demographics|Epidemiology and Demographics]]==
==[[Hemophagocytic lymphohistiocytosis risk factors|Risk Factors]]==
==[[Hemophagocytic lymphohistiocytosis screening|Screening]]==
==[[Hemophagocytic lymphohistiocytosis natural history, complications and prognosis|Natural History, Complications and Prognosis]]==
==Diagnosis==
==Diagnosis==
===History===
[[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]]
Inquiry about family history may help diagnose the familial forms of Hemophagocytic lymphohistiocytosis.
 
===Symptoms===
* [[Fever]]
* [[Rash]] on the skin
* [[Abdominal pain]]
* Easy [[bruising]] and abnormal [[bleeding]]
* [[Infections]] <ref>{{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}}</ref>
 
== References ==
{{reflist|2}}
 
* <ref>{{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}}</ref>
 
==External links==
* http://www.hrtrust.org/library/HLHSymptomsSignsAndDiagnosis.html
* 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

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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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