Hemophilia classification: Difference between revisions
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{{Hemophilia}} | {{Hemophilia}} | ||
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==Overview== | ==Overview== | ||
Hemophilia may be classified into three sub-types based on the lack of functional [[Coagulation|clotting factors]]: [[hemophilia A]] , [[hemophilia B]], [[hemophilia C]]. It can also be divided into different categories based on the severity of the condition. Hemophilia can also be [[acquired]] in the setting of [[antibodies]] directed against the [[Coagulation|clotting factors]]. | |||
==Classification== | ==Classification== | ||
* | *Hemophilia may be classified into: | ||
'''1. Hemophilia A:''' A [[recessive]] [[Sex linkage|X-linked]] [[genetic disorder]] involving a lack of functional [[Clotting factor|clotting]] [[factor VIII]] and representing approximately 80% of hemophilia cases.<ref name="pmid24014073">{{cite journal |vauthors=Santagostino E, Fasulo MR |title=Hemophilia a and hemophilia B: different types of diseases? |journal=Semin. Thromb. Hemost. |volume=39 |issue=7 |pages=697–701 |date=October 2013 |pmid=24014073 |doi=10.1055/s-0033-1353996 |url=}}</ref><br>'''2. Hemophilia B:''' A [[recessive]] [[Sex linkage|X-linked]] [[genetic disorder]] involving a lack of functional [[Clotting factor|clotting]] [[factor IX]] and comprising approximately 20% of hemophilia cases.<ref name="pmid24014073">{{cite journal |vauthors=Santagostino E, Fasulo MR |title=Hemophilia a and hemophilia B: different types of diseases? |journal=Semin. Thromb. Hemost. |volume=39 |issue=7 |pages=697–701 |date=October 2013 |pmid=24014073 |doi=10.1055/s-0033-1353996 |url=}}</ref><br>'''3. Hemophilia C:''' An [[autosomal]] [[genetic disorder]] involving a lack of functional [[Clotting factor|clotting]] [[factor XI]]. This condition is not completely [[recessive]], as [[heterozygous]] individuals also show increased [[bleeding]].<ref name="pmid23610841">{{cite journal |vauthors=Shearin-Patterson T, Davidson E |title=Hemophilia C |journal=JAAPA |volume=26 |issue=4 |pages=50 |date=April 2013 |pmid=23610841 |doi= |url=}}</ref><Br>'''4. Acquired Hemophilia:''' An acquired condition resulting from [[Autoantibody|autoantibodies]] directed against the [[Coagulation|clotting factors]]. This usually occurs in the setting of a [[Cancer|malignancy]].<ref name="pmid28960809">{{cite journal |vauthors=Napolitano M, Siragusa S, Mancuso S, Kessler CM |title=Acquired haemophilia in cancer: A systematic and critical literature review |journal=Haemophilia |volume=24 |issue=1 |pages=43–56 |date=January 2018 |pmid=28960809 |doi=10.1111/hae.13355 |url=}}</ref> | |||
===Classification Based on Severity=== | |||
*Hemophilia may be classified as severe, moderate or mild based on the levels of [[factor VIII]] or [[factor IX]].<ref>{{Cite web | title =CDC Hemophilia classification| url =http://www.cdc.gov/ncbddd/hemophilia/diagnosis.html }}</ref> | |||
{| style="border: 0px; font-size: 90%; margin: 3px; width: 500px;" align="center" | |||
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! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Severity}} | |||
! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Levels of Factor VIII | |||
or IX in the blood}} | |||
|- | |||
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" | Normal (as in a person who does not have hemophilia) | |||
| style="padding: 5px 5px; background: #F5F5F5;" |50% to 100% | |||
|- | |||
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" | Mild | |||
| style="padding: 5px 5px; background: #F5F5F5;" |Greater than 5% but less than 50% | |||
|- | |||
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" | Moderate | |||
| style="padding: 5px 5px; background: #F5F5F5;" |1% to 5% | |||
|- | |||
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" | Severe | |||
| style="padding: 5px 5px; background: #F5F5F5;" |Less than 1% | |||
|- | |||
| colspan="2" style="padding: 5px 5px; background: #F5F5F5;" | <small>Adapted from CDC Hemophilia Diagnosis> {{cite web| url=http://www.cdc.gov/ncbddd/hemophilia/diagnosis.html| title=CDC Hemophilia Diagnosis }}</small> | |||
|} | |||
==References== | ==References== | ||
Latest revision as of 22:47, 28 May 2019
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Hemophilia Microchapters |
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Diagnosis |
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Treatment |
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Case Studies |
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Hemophilia classification On the Web |
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American Roentgen Ray Society Images of Hemophilia classification |
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Risk calculators and risk factors for Hemophilia classification |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Sabawoon Mirwais, M.B.B.S, M.D.[2]Simrat Sarai, M.D. [3]
Overview
Hemophilia may be classified into three sub-types based on the lack of functional clotting factors: hemophilia A , hemophilia B, hemophilia C. It can also be divided into different categories based on the severity of the condition. Hemophilia can also be acquired in the setting of antibodies directed against the clotting factors.
Classification
- Hemophilia may be classified into:
1. Hemophilia A: A recessive X-linked genetic disorder involving a lack of functional clotting factor VIII and representing approximately 80% of hemophilia cases.[1]
2. Hemophilia B: A recessive X-linked genetic disorder involving a lack of functional clotting factor IX and comprising approximately 20% of hemophilia cases.[1]
3. Hemophilia C: An autosomal genetic disorder involving a lack of functional clotting factor XI. This condition is not completely recessive, as heterozygous individuals also show increased bleeding.[2]
4. Acquired Hemophilia: An acquired condition resulting from autoantibodies directed against the clotting factors. This usually occurs in the setting of a malignancy.[3]
Classification Based on Severity
- Hemophilia may be classified as severe, moderate or mild based on the levels of factor VIII or factor IX.[4]
| Severity | Levels of Factor VIII
or IX in the blood |
|---|---|
| Normal (as in a person who does not have hemophilia) | 50% to 100% |
| Mild | Greater than 5% but less than 50% |
| Moderate | 1% to 5% |
| Severe | Less than 1% |
| Adapted from CDC Hemophilia Diagnosis> "CDC Hemophilia Diagnosis". | |
References
- ↑ 1.0 1.1 Santagostino E, Fasulo MR (October 2013). "Hemophilia a and hemophilia B: different types of diseases?". Semin. Thromb. Hemost. 39 (7): 697–701. doi:10.1055/s-0033-1353996. PMID 24014073.
- ↑ Shearin-Patterson T, Davidson E (April 2013). "Hemophilia C". JAAPA. 26 (4): 50. PMID 23610841.
- ↑ Napolitano M, Siragusa S, Mancuso S, Kessler CM (January 2018). "Acquired haemophilia in cancer: A systematic and critical literature review". Haemophilia. 24 (1): 43–56. doi:10.1111/hae.13355. PMID 28960809.
- ↑ "CDC Hemophilia classification".