Hemophagocytic lymphohistiocytosis: Difference between revisions
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==Overview== | ==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. | '''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== | ==Classification== | ||
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[[Image:autorecessive.jpg|thumb|left|Familial hemophagocytic lymphohistiocytosis has an autosomal recessive pattern of inheritance.]] | [[Image:autorecessive.jpg|thumb|left|Familial hemophagocytic lymphohistiocytosis has an autosomal recessive pattern of inheritance.]] | ||
<br clear="left"/> | <br clear="left"/> | ||
==Diagnosis== | ==Diagnosis== | ||
===History=== | ===History=== | ||
Inquiry about family history may help diagnose the familial forms of Hemophagocytic lymphohistiocytosis. | Inquiry about family history may help diagnose the familial forms of Hemophagocytic lymphohistiocytosis. | ||
===Symptoms=== | ===Symptoms=== | ||
* [[Rash]] on the skin | * [[Rash]] on the skin | ||
* [[Abdominal pain]] | * [[Abdominal pain]] | ||
* | * | ||
Revision as of 02:07, 1 August 2012
| 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 |
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
Overview
Hemophagocytic lymphohistiocytosis (HLH)[1] 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:
Diagnosis
History
Inquiry about family history may help diagnose the familial forms of Hemophagocytic lymphohistiocytosis.
Symptoms
- Rash on the skin
- Abdominal pain
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
- http://www.hrtrust.org/library/HLHSymptomsSignsAndDiagnosis.html
- Histiocytosis at eMedicine
- http://www.histio.org
- http://www.jonahchuang.com
- http://www.elyseyu.com
- http://www.ethanmichaelsmith.com
- http://www.savejonah.com