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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) 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 primarily categorized as
* 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.
 
 
===Genetics===
 
 
 
===Microscopic Pathology===
 
[[Image:Hemophagocytic_syndrome.jpg‎|thumb|center|Micrograph showing red blood cells within macrophages. H&E stain]]
 
==Epidemiology and Demographics==
*  Familial hemophagocytic lymphohistiocytosis occurs in approximately 2 in 100,000 individuals worldwide.
* Signs and symptoms of familial hemophagocytic lymphohistiocytosis usually become apparent during infancy, although occasionally they appear later in life.  They usually occur when the immune system launches an exaggerated response to an infection, but may also occur in the absence of infection. 
 
==Natural History, Complications and Prognosis==
*  Without treatment, most people with familial hemophagocytic lymphohistiocytosis survive only a few months.


==[[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
 


==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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