195:. These signs often prompt blood tests that confirm the presence of haemophilia. In individuals, especially those with moderate or mild haemophilia, any form of trauma can trigger the first significant bleed. Haemophilia substantially elevates the risk of protracted bleeding from ordinary injuries, and in severe cases, bleeding can occur spontaneously without an apparent cause. Bleeding episodes can manifest anywhere in the body. Superficial bleeding resulting from abrasions or shallow lacerations may persist, with scabs easily breaking due to the deficiency of
257:. The symptoms depend on the severity of haemophilia. In the case of severe haemophilia, patients may complain of multiple oral bleeding episodes throughout their life. Haemophilia patients are considered to be a special group of patients as routinely done procedures may be fatal in them. It was seen that almost 14% of all haemophilia patients and 30% of cases with a mild type of haemophilia have been diagnosed early following an episode of severe oral bleeding, of which the most common sites were the labial frenum and the tongue.
331:
323:
266:
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504:
haematoma in the retromolar or pterygoid space. The intraligamental technique or interosseous technique should be considered instead of the mandibular block. Articaine has been used as a buccal infiltration to anaesthetize the lower molar teeth. A lingual infiltration also requires appropriate factor replacement since the injection is into an area with a rich plexus of blood vessels and the needle is not adjacent to bone.
57:
2150:
577:. In the latest study which followed patients from 1992 to 2001, the male life expectancy was 59 years. If cases with known viral infections were excluded, the life expectancy was 72, close to that of the general population. 26% of the cases died from AIDS and 22% from hepatitis C. However, these statistics for prognosis are unreliable as there has been marked improvement of
441:
470:(VWF) independent, recombinant DNA-derived Factor VIII (FVIII) concentrate shown to prevent bleeding in children and adults. Treatment dosing and frequency of plasma concentrate Factor VIII may be variable and individually determined; dosing of efanesoctocog alfa shown effective is an IV injection of once-weekly 50 IU per kilogram of body weight.
303:, while the remainder are affected because they produce factor VIII in insufficient amounts (quantitative deficiency). Of those who have severe deficiency (defined as <1% activity of factor VIII), 45-50% have the same mutation, an inversion within the factor VIII gene that results in total elimination of protein production.
176:
379:
There are numerous different mutations which can cause haemophilia A, due to differences in changes to the factor VIII gene (and the resulting protein). Individuals with haemophilia often have some level of active clotting factor. Individuals with less than 1% active factor are classified as having
358:
has been made available to determine an individual's risk of attaining or passing on haemophilia. Diagnosis of haemophilia A also includes a severity level, which can range from mild to severe based on the amount of active and functioning factor VIII detected in the blood. Factor VIII levels do not
503:
If numbing is necessary for dental procedures, the nerve block (typically to the inferior alveolar nerve) should only be given after raising clotting factor levels by appropriate replacement therapy, as there is a risk of bleeding into the muscles along with potential airway compromise due to a
490:
antibiotics but sometimes the device must be removed, also, there are other studies that show a risk of clots forming at the tip of the catheter, rendering it useless. Some individuals with severe haemophilia, and most with moderate and mild haemophilia, treat only as needed without a regular
183:
Haemophilia A's phenotype has a quite wide range of symptoms encompassing both internal and external bleeding episodes. Individuals with more severe haemophilia tend to experience more intense and frequent bleeding, whereas those with mild haemophilia typically exhibit milder symptoms unless
1109:
von
Drygalski, Annette; Chowdary, Pratima; Kulkarni, Roshni; Susen, Sophie; Konkle, Barbara A.; Oldenburg, Johannes; Matino, Davide; Klamroth, Robert; Weyand, Angela C.; Jimenez-Yuste, Victor; Nogami, Keiji; Poloskey, Stacey; Winding, Bent; Willemze, Annemieke; Knobe, Karin (2023-01-26).
1557:
Malec, Lynn; Peyvandi, Flora; Chan, Anthony K.C.; Königs, Christoph; Zulfikar, Bulent; Yuan, Huixing; Simpson, Mindy; Ălvarez RomĂĄn, Maria Teresa; Carcao, Manuel; Staber, Janice M.; Dunn, Amy L.; Chou, Sheng-Chieh; dâOiron, Roseline; Albisetti, Manuela; Demissie, Marek (2024-07-18).
1149:
Malec, Lynn; Peyvandi, Flora; Chan, Anthony K.C.; Königs, Christoph; Zulfikar, Bulent; Yuan, Huixing; Simpson, Mindy; Ălvarez RomĂĄn, Maria Teresa; Carcao, Manuel; Staber, Janice M.; Dunn, Amy L.; Chou, Sheng-Chieh; d'Oiron, Roseline; Albisetti, Manuela; Demissie, Marek (2024-07-18).
359:
typically change throughout an individual's lifetime. Severe haemophilia A is the most common severity, occurring in the majority of affected people. Individuals with mild haemophilia often experience few or no bleeding episodes except in the case of serious
1690:
Plug, I.; Van Der Bom, J. G.; Peters, M.; Mauser-Bunschoten, E. P.; De Goede-Bolder, A.; Heijnen, L.; Smit, C.; Willemse, J.; Rosendaal, F. R. (2006-03-01). "Mortality and causes of death in patients with hemophilia, 1992â2001: a prospective cohort study1".
659:
Konkle, Barbara A.; Josephson, Neil C.; Nakaya
Fletcher, Shelley (1993-01-01). Pagon, Roberta A.; Adam, Margaret P.; Ardinger, Holly H.; Wallace, Stephanie E.; Amemiya, Anne; Bean, Lora J.H.; Bird, Thomas D.; Fong, Chin-To; Mefford, Heather C. (eds.).
2045:
2030:
2015:
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several times a week are eliminated. However, there are risks involved with their use, the most worrisome being that of infection, studies differ but some show an infection rate that is high. These infections can usually be treated with
887:
Sonu
Acharya, Karishma Rathore, Upasana Mahapatra, Sashikant Sethi, Nikita Sahu (2018). "An unusual oral manifestation of hemophilia in a child". Journal of International Oral Health. Retrieved 2019-12-20.
1480:
Rangarajan, Savita; Walsh, Liron; Lester, Will; Perry, David; Madan, Bella; Laffan, Michael; Yu, Hua; Vettermann, Christian; Pierce, Glenn F.; Wong, Wing Y.; Pasi, K. John (16 December 2017).
2196:
1827:
Collins, Peter; Baudo, Francesco; Huth-KĂŒhne, Angela; Ingerslev, JĂžrgen; Kessler, Craig M; Castellano, Maria E Mingot; Shima, Midori; St-Louis, Jean; LĂ©vesque, HervĂ© (7 June 2010).
593:
Haemophilia A occurs in approximately 1 in 5,000 males, while the incidence of haemophilia B is 1 in 30,000 in the male population, of these, 85% have haemophilia A and 15% have
306:
Since both forms of haemophilia can be caused by a variety of different mutations, initial diagnosis and classification is done by measurement of protein activity rather than by
184:
subjected to surgical procedures or significant trauma. Those with moderate haemophilia may display variable symptoms, falling within the spectrum between severe and mild forms.
148:, thereby resulting in significant susceptibility to bleeding, both internally and externally. This condition occurs almost exclusively in males born to carrier mothers due to
1919:
Kellogg, TF (November 1974). "Steroid balance and tissue cholesterol accumulation in germfree and conventional rats fed diets containing saturated and polyunsaturated fats".
3056:
233:
antibodies against factor VIII due to frequent infusions. These develop as the body recognises the infused factor VIII as foreign, as the body does not produce its own
3112:
2976:
2189:
1413:
Andrew Brewer, Maria Elvira Correa (May 2006). "Guildelines for Dental
Treatment of Patients with Inherited Bleeding Disorders" (PDF). Treatment of Hemophilia.
284:
females (which is only possible in the daughters of a haemophilic male and a carrier or haemophiliac female). However, mild haemophilia A is known to occur in
3012:
2823:
350:. PTT tests are the first blood test done when haemophilia is indicated. However, the diagnosis is made in the presence of very low levels of factor VIII. A
1540:
2872:
2182:
3319:
3167:
612:
545:
recombinant protein, prophylaxis for children with severe hemophilia A could have therapeutic benefit leading to effective bleeding prevention.
3201:
2313:
3238:
3439:
3314:
2981:
2957:
1636:
Triemstra, Mattanja (1995-12-01). "Mortality in
Patients with Hemophilia: Changes in a Dutch Population from 1986 to 1992 and 1973 to 1986".
979:
3434:
3157:
2892:
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of haemophilia is identified. Approximately 30% of patients have no family history; their disease is presumably caused by new mutations.
2488:
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2060:
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1428:
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The oral manifestations are characterized by frequent bleeding of multiple sites, frequently seen as gingival and postextraction
3324:
245:, can be infused as a treatment for haemorrhage in individuals with haemophilia and antibodies against replacement factor VIII.
2943:
1756:
Casana, P.; Cabrera, N.; Cid, A. R.; Haya, S.; Beneyto, M.; Espinos, C.; Cortina, V.; Dasi, M. A.; Aznar, J. A. (2008-07-01).
1531:
3266:
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149:
2556:
1800:
199:, potentially leading to re-bleeding. While superficial bleeding poses challenges, more critical sites of bleeding include:
3348:
2862:
2542:
1544:
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2530:
2493:
27:
This article is about the bleeding disorder with factor VIII deficiency. For the disorder with factor IX deficiency, see
2363:
1882:"Treatment for preventing bleeding in people with haemophilia or other congenital bleeding disorders undergoing surgery"
3404:
3329:
3223:
2877:
2809:
2683:
2259:
473:
In children, an easily accessible intravenous port may have to be inserted to minimise frequent traumatic intravenous
339:
296:
be measured in all known or potential carriers prior to surgery and in the event of clinically significant bleeding.
1880:
Coppola, Antonio; Windyga, Jerzy; Tufano, Antonella; Yeung, Cindy; Di Minno, Matteo Nicola Dario (9 February 2015).
3399:
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746:
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164:
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About 5-10% of people with haemophilia A are affected because they make a dysfunctional version of the
330:
322:
167:. This intervention aims to address and preempt additional bleeding episodes in affected individuals.
3429:
2999:
2925:
2897:
2379:
2274:
2264:
141:
553:
Two Dutch studies have followed haemophilia patients for a number of years. Both studies found that
64:
Protein structure of coagulation factor VIII, of which its deficiency is the cause of haemophilia A.
3196:
2907:
2744:
2618:
2608:
2404:
2284:
2133:
2049:
721:
562:
524:
242:
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3069:
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2426:
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1981:
1734:
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538:
463:
159:
The medical management of individuals with hemophilia A frequently entails the administration of
81:
2384:
2358:
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2409:
2318:
2111:
2082:
2024:
1973:
1965:
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1907:
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153:
99:
69:
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1957:
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1123:
1016:
1008:
929:
911:
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754:
364:
343:
338:
The diagnosis of haemophilia A may be suspected as coagulation testing reveals an increased
265:
3309:
3256:
3132:
3127:
3048:
2854:
2764:
2573:
2279:
360:
355:
307:
277:
213:
2414:
1274:
1219:"Barriers to primary prophylaxis in haemophilic children: the issue of the venous access"
1068:
2155:
1902:
1881:
3389:
3304:
3177:
3142:
3074:
2774:
2476:
2419:
2301:
1863:
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1390:
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1251:
1218:
1021:
996:
934:
899:
289:
2054:
1948:
Armstrong, Elina; Hillarp, Andreas (2014). "Assay discrepancy in mild haemophilia A".
1481:
1085:
3423:
3384:
3147:
3107:
3035:
3022:
2920:
2887:
2784:
2596:
2591:
2343:
2174:
1985:
1704:
1317:
1300:
784:
Lissauer, Tom; Fanaroff, Avroy A.; Miall, Lawrence; Fanaroff, Jonathan (2015-06-10).
627:
622:
594:
554:
421:
405:
347:
28:
2122:
1829:"Consensus recommendations for the diagnosis and treatment of acquired hemophilia A"
1738:
1673:
1342:
856:
839:
2994:
2226:
1893:
513:
492:
481:
in haemophilia much easier for families because the problems of finding a vein for
455:
285:
188:
56:
2076:
1190:
785:
2832:
2769:
2734:
2709:
2581:
2389:
2221:
2210:
2106:
1758:"Severe and moderate hemophilia A: identification of 38 new genetic alterations"
1559:
1151:
1111:
607:
574:
570:
542:
487:
478:
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300:
145:
74:
1598:
2759:
2353:
2335:
2128:
2087:
661:
617:
527:
451:
Most individuals with severe haemophilia require regular supplementation with
311:
281:
238:
121:
1969:
1932:
1854:
1783:
1722:
1657:
1622:
1583:
1381:
1326:
1242:
1175:
1135:
925:
865:
2704:
2668:
2291:
2117:
2039:
1845:
1301:"The risk associated with indwelling catheters in children with haemophilia"
1275:"Guidelines for the Prevention of Intravascular Catheter-Related Infections"
1073:
916:
491:
prophylactic schedule. Mild haemophiliacs often manage their condition with
409:
310:, though genetic tests are recommended for testing of family members once a
293:
192:
17:
1977:
1911:
1872:
1791:
1774:
1757:
1730:
1517:
1508:
1454:
1399:
1334:
1260:
1030:
943:
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671:
1940:
1665:
1575:
1498:
1167:
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2719:
2673:
2658:
2517:
2348:
2206:
1614:
1536:
1373:
1234:
482:
274:
254:
2007:
1358:"The use of desmopressin in acquired haemophilia A: a systematic review"
440:
2678:
759:
368:
1961:
1560:"Efanesoctocog Alfa Prophylaxis for Children with Severe Hemophilia A"
1152:"Efanesoctocog Alfa Prophylaxis for Children with Severe Hemophilia A"
1112:"Efanesoctocog Alfa Prophylaxis for Patients with Severe Hemophilia A"
1012:
512:
In
December 2017, it was reported that doctors had used a new form of
2801:
2663:
2034:
2019:
1713:
208:
196:
187:
One common early indicator of haemophilia is prolonged bleeding from
1086:"Von Willebrand's Disease. About Von Willebrand's Disease | Patient"
495:, a drug which releases stored factor VIII from blood vessel walls.
1189:
Ma, Alice D.; Roberts, Harold R.; Escobar, Miguel A. (2012-10-03).
175:
3280:
530:
has been approved by the FDA in 2017 for therapy of hemophilia A.
439:
431:
329:
321:
218:
203:
174:
3276:
3117:
2065:
1802:
Haemophilia and
Haemostasis: A Case-based Approach to Management
840:"Acquired Factor VIII Inhibitors: Pathophysiology and Treatment"
3346:
2844:
2805:
2178:
1597:
Chowdary, Pratima (2024-07-18). Phimister, Elizabeth G. (ed.).
1044:
392:
have between 5â40% of normal levels of active clotting factor.
1599:"Bioengineered Factor VIII â More Innovation for Hemophilia A"
1192:
Hemophilia and
Hemostasis: A Case-Based Approach to Management
722:"Haemophilia A (Factor VIII Deficiency) information | Patient"
566:
1429:"Groundbreaking gene therapy trial set to cure haemophilia A"
898:
Nair, Preethi S.; Shetty, S.; Ghosh, Kanjaksha (2012-01-01).
974:(19th ed.). Philadelphia: Saunders. pp. 1700â1.
354:
is frequently present, although not essential. Recently,
1217:
Santagostino, Elena; Mancuso, Maria Elisa (2008-09-01).
1482:"AAV5âFactor VIII Gene Transfer in Severe Hemophilia A"
292:, so it is recommended that levels of factor VIII and
997:"Haemophilia A and haemophilia B: molecular insights"
1997:
420:(needed for the proper functioning of Factor VIII);
3297:
3275:
3247:
3210:
3176:
3087:
2966:
2934:
2906:
2853:
2694:
2651:
2572:
2516:
2458:
2451:
2444:
2372:
2334:
2247:
2240:
2097:
2001:
666:. Seattle (WA): University of Washington, Seattle.
152:. Nevertheless, rare isolated cases do emerge from
116:
108:
98:
90:
80:
68:
46:
41:
1356:Franchini, Massimo; Lippi, Giuseppe (2011-10-01).
104:Bleeding time, coagulation screen, genetic testing
557:were common in haemophiliacs due to the frequent
694:"Hemophilia A: MedlinePlus Medical Encyclopedia"
229:One therapeutic conundrum is the development of
3023:Danon disease/glycogen storage disease Type IIb
1417:: 9 â via World Federation of Hemophilia (WFH).
749:(2009). Stuart MC, Kouimtzi M, Hill SR (eds.).
838:Ma, Alice D.; Carrizosa, Daniel (2006-01-01).
753:. World Health Organization. pp. 259â60.
533:In July 2024, a recent study published in the
3128:Color blindness (red and green, but not blue)
3113:Alpha-thalassemia mental retardation syndrome
2817:
2190:
1950:European Journal of Haematology. Supplementum
8:
3013:Glucose-6-phosphate dehydrogenase deficiency
1455:"Haemophilia A trial results 'mind-blowing'"
1045:"OMIM Entry - # 306700 - HEMOPHILIA A; HEMA"
241:, a protein in the extrinsic pathway of the
3343:
2841:
2824:
2810:
2802:
2455:
2448:
2244:
2197:
2183:
2175:
1998:
55:
38:
2873:X-linked severe combined immunodeficiency
1901:
1862:
1844:
1773:
1712:
1507:
1497:
1389:
1316:
1250:
1020:
965:
963:
961:
959:
957:
955:
953:
933:
915:
855:
815:"How Is Hemophilia Treated? - NHLBI, NIH"
758:
1650:10.7326/0003-4819-123-11-199512010-00002
1532:HEMLIBRA- emicizumab injection, solution
264:
112:Hepatitis B vaccine should be considered
3267:X-linked nephrogenic diabetes insipidus
3192:Hypohidrotic ectodermal dysplasia (EDA)
1886:Cochrane Database of Systematic Reviews
640:
613:Contaminated haemophilia blood products
86:Prolonged bleeding from common injuries
3202:X-linked endothelial corneal dystrophy
809:
807:
790:. John Wiley & Sons. p. 135.
3158:CharcotâMarieâTooth disease (CMTX2-3)
2982:Ornithine transcarbamylase deficiency
2958:X-linked adrenal hypoplasia congenita
1693:Journal of Thrombosis and Haemostasis
1685:
1683:
716:
714:
688:
686:
684:
682:
654:
652:
650:
648:
646:
644:
384:, those with 1â5% active factor have
120:Factor VIII, factor VIII inhibitors,
7:
2893:X-linked lymphoproliferative disease
2863:Chronic granulomatous disease (CYBB)
561:which put them at risk of acquiring
3239:EmeryâDreifuss muscular dystrophy 1
2948:Spinal and bulbar muscular atrophy
900:"A homozygous female hemophilia A"
237:. In these individuals, activated
25:
3018:Pyruvate dehydrogenase deficiency
1799:Roberts, Harold R. (2008-04-15).
1541:U.S. National Library of Medicine
340:partial thromboplastin time (PTT)
273:Haemophilia A is inherited as an
3098:X-linked intellectual disability
2551:platelet storage pool deficiency
2503:Heparin-induced thrombocytopenia
2148:
1705:10.1111/j.1538-7836.2006.01808.x
1318:10.1111/j.1365-2141.2007.06703.x
904:Indian Journal of Human Genetics
2944:Androgen insensitivity syndrome
1603:New England Journal of Medicine
1564:New England Journal of Medicine
1486:New England Journal of Medicine
1156:New England Journal of Medicine
1116:New England Journal of Medicine
857:10.1182/asheducation-2006.1.432
585:since these studies were done.
535:New England Journal of Medicine
154:de novo (spontaneous) mutations
3320:SimpsonâGolabiâBehmel syndrome
2270:Activated protein C resistance
1894:10.1002/14651858.cd009961.pub2
1305:British Journal of Haematology
269:X linked recessive inheritance
150:X-linked recessive inheritance
1:
3289:AMELX Amelogenesis imperfecta
3229:Centronuclear myopathy (MTM1)
2926:X-linked sideroblastic anemia
1545:National Institutes of Health
972:Nelson textbook of pediatrics
334:Haemarthrosis on lateral view
3440:X-linked recessive disorders
3315:SmithâFinemanâMyers syndrome
3163:PelizaeusâMerzbacher disease
3057:Purineâpyrimidine metabolism
2364:Trousseau sign of malignancy
280:. It occurs in males and in
3435:Defects of enzyme cofactors
3405:Craniofrontonasal dysplasia
3330:Nasodigitoacoustic syndrome
2878:X-linked agammaglobulinemia
2684:Nonthrombocytopenic purpura
2260:Antithrombin III deficiency
1638:Annals of Internal Medicine
342:in the context of a normal
3461:
3400:Orofaciodigital syndrome 1
3234:ConradiâHĂŒnermann syndrome
2987:Oculocerebrorenal syndrome
2639:Congenital afibrinogenemia
2543:Glanzmann's thrombasthenia
2307:Essential thrombocythaemia
1299:Ljung, Rolf (2007-09-01).
844:ASH Education Program Book
477:. These devices have made
26:
3360:X-linked hypophosphatemia
3355:
3342:
3220:Becker muscular dystrophy
2883:Hyper-IgM syndrome type 1
2840:
2755:Gastrointestinal bleeding
2557:HermanskyâPudlak syndrome
2326:Antiphospholipid syndrome
2217:
1921:Journal of Lipid Research
1805:. John Wiley & Sons.
1195:. John Wiley & Sons.
970:Kliegman, Robert (2011).
747:World Health Organization
416:which is a deficiency in
408:which is a deficiency in
63:
54:
3325:MohrâTranebjĂŠrg syndrome
2868:WiskottâAldrich syndrome
2725:Subconjunctival bleeding
2715:Intracranial haemorrhage
2531:BernardâSoulier syndrome
2494:UpshawâSchulman syndrome
2468:Thrombocytopenic purpura
2297:Sticky platelet syndrome
751:WHO Model Formulary 2008
541:, a bioengineered human
516:to treat haemophilia A.
3365:Focal dermal hypoplasia
3079:Occipital horn syndrome
3008:Carbohydrate metabolism
1846:10.1186/1756-0500-3-161
917:10.4103/0971-6866.96685
787:Neonatology at a Glance
445:Recombinant factor VIII
400:Two of the most common
138:blood clotting disorder
3380:Incontinentia pigmenti
3187:Dyskeratosis congenita
3031:Lipid storage disorder
2953:KAL1 Kallmann syndrome
2629:Factor XIII deficiency
2609:Hypoprothrombinemia/II
2604:von Willebrand disease
2562:Gray platelet syndrome
1775:10.3324/haematol.12344
1433:Barts Health NHS Trust
563:blood borne infections
448:
437:
414:von Willebrand Disease
402:differential diagnoses
396:Differential diagnosis
335:
327:
270:
180:
161:factor VIII medication
94:Factor VIII deficiency
3103:CoffinâLowry syndrome
3044:Mucopolysaccharidosis
2624:Factor XII deficiency
2614:Factor VII deficiency
2432:Renal vein thrombosis
1576:10.1056/NEJMoa2312611
1499:10.1056/NEJMoa1708483
1168:10.1056/NEJMoa2312611
1128:10.1056/NEJMoa2209226
583:anti-retroviral drugs
520:Monoclonal antibodies
499:Dental considerations
468:von Willebrand factor
443:
435:
418:von Willebrand factor
344:prothrombin time (PT)
333:
326:X-Ray- haemoarthritis
325:
268:
178:
165:intravenous injection
3395:LujanâFryns syndrome
3062:LeschâNyhan syndrome
3000:Adrenoleukodystrophy
2898:Properdin deficiency
2380:Deep vein thrombosis
2275:Protein S deficiency
2265:Protein C deficiency
1615:10.1056/NEJMe2313795
1374:10.2450/2011.0113-10
1235:10.2450/2008.0031-08
1229:(Suppl 2): s12âs16.
1207:Google books no page
424:is also considered.
386:moderate haemophilia
3197:X-linked ichthyosis
2745:Pulmonary haematoma
2619:Factor X deficiency
2508:MayâHegglin anomaly
2285:Prothrombin G20210A
1535:drug label/data at
1001:Molecular Pathology
995:Bowen, D J (2002).
525:Monoclonal antibody
447:for self-treatment.
301:factor VIII protein
249:Oral Manifestations
243:coagulation cascade
3370:Fragile X syndrome
3180:and related tissue
2847:X-linked recessive
2652:Signs and symptoms
2427:Pulmonary embolism
2232:Bleeding diathesis
2098:External resources
1833:BMC Research Notes
1461:. 14 December 2017
1435:. 14 December 2017
559:blood transfusions
539:efanesoctocog alfa
537:demonstrated that
464:efanesoctocog alfa
449:
438:
382:severe haemophilia
336:
328:
271:
181:
171:Signs and symptoms
142:genetic deficiency
3417:
3416:
3413:
3412:
3349:X-linked dominant
3338:
3337:
3298:No primary system
2799:
2798:
2795:
2794:
2647:
2646:
2634:Dysfibrinogenemia
2518:Platelet function
2440:
2439:
2319:Purpura fulminans
2143:
2142:
1962:10.1111/ejh.12374
1492:(26): 2519â2530.
1362:Blood Transfusion
1223:Blood Transfusion
1013:10.1136/mp.55.1.1
981:978-1-4377-0755-7
819:www.nhlbi.nih.gov
579:infection control
388:, and those with
127:
126:
100:Diagnostic method
36:Medical condition
16:(Redirected from
3452:
3375:Aicardi syndrome
3344:
2842:
2826:
2819:
2812:
2803:
2740:Haemopericardium
2460:Thrombocytopenia
2456:
2449:
2405:Lowenberg's sign
2255:Clotting factors
2245:
2199:
2192:
2185:
2176:
2152:
2151:
1999:
1989:
1944:
1915:
1905:
1876:
1866:
1848:
1823:
1821:
1819:
1795:
1777:
1768:(7): 1091â1094.
1743:
1742:
1716:
1687:
1678:
1677:
1633:
1627:
1626:
1594:
1588:
1587:
1554:
1548:
1528:
1522:
1521:
1511:
1501:
1477:
1471:
1470:
1468:
1466:
1451:
1445:
1444:
1442:
1440:
1425:
1419:
1410:
1404:
1403:
1393:
1353:
1347:
1346:
1320:
1296:
1290:
1289:
1287:
1285:
1271:
1265:
1264:
1254:
1214:
1208:
1206:
1186:
1180:
1179:
1146:
1140:
1139:
1106:
1100:
1099:
1097:
1096:
1082:
1076:
1065:
1059:
1058:
1056:
1055:
1041:
1035:
1034:
1024:
992:
986:
985:
967:
948:
947:
937:
919:
895:
889:
884:
878:
877:
859:
835:
829:
828:
826:
825:
811:
802:
801:
781:
775:
774:
762:
743:
737:
736:
734:
732:
718:
709:
708:
706:
704:
690:
677:
675:
656:
581:and efficacy of
555:viral infections
390:mild haemophilia
365:tooth extraction
59:
39:
21:
3460:
3459:
3455:
3454:
3453:
3451:
3450:
3449:
3420:
3419:
3418:
3409:
3351:
3334:
3310:McLeod syndrome
3293:
3271:
3257:Alport syndrome
3243:
3206:
3172:
3133:Ocular albinism
3083:
3049:Hunter syndrome
2962:
2930:
2902:
2849:
2836:
2830:
2800:
2791:
2765:Haemoperitoneum
2690:
2643:
2574:Clotting factor
2568:
2512:
2436:
2385:Bancroft's sign
2368:
2359:Virchow's triad
2330:
2280:Factor V Leiden
2236:
2213:
2203:
2173:
2172:
2171:
2153:
2149:
2144:
2139:
2138:
2093:
2092:
2010:
1996:
1947:
1918:
1888:(2): CD009961.
1879:
1826:
1817:
1815:
1813:
1798:
1755:
1752:
1750:Further reading
1747:
1746:
1689:
1688:
1681:
1635:
1634:
1630:
1596:
1595:
1591:
1556:
1555:
1551:
1529:
1525:
1479:
1478:
1474:
1464:
1462:
1453:
1452:
1448:
1438:
1436:
1427:
1426:
1422:
1411:
1407:
1355:
1354:
1350:
1298:
1297:
1293:
1283:
1281:
1273:
1272:
1268:
1216:
1215:
1211:
1203:
1188:
1187:
1183:
1148:
1147:
1143:
1108:
1107:
1103:
1094:
1092:
1084:
1083:
1079:
1066:
1062:
1053:
1051:
1043:
1042:
1038:
994:
993:
989:
982:
969:
968:
951:
897:
896:
892:
885:
881:
837:
836:
832:
823:
821:
813:
812:
805:
798:
783:
782:
778:
771:
745:
744:
740:
730:
728:
720:
719:
712:
702:
700:
698:www.nlm.nih.gov
692:
691:
680:
658:
657:
642:
637:
632:
603:
591:
551:
522:
510:
501:
430:
398:
377:
356:genetic testing
320:
288:females due to
278:recessive trait
263:
251:
227:
214:Digestive tract
173:
37:
32:
23:
22:
15:
12:
11:
5:
3458:
3456:
3448:
3447:
3445:Coagulopathies
3442:
3437:
3432:
3422:
3421:
3415:
3414:
3411:
3410:
3408:
3407:
3402:
3397:
3392:
3390:CHILD syndrome
3387:
3382:
3377:
3372:
3367:
3362:
3356:
3353:
3352:
3347:
3340:
3339:
3336:
3335:
3333:
3332:
3327:
3322:
3317:
3312:
3307:
3305:Barth syndrome
3301:
3299:
3295:
3294:
3292:
3291:
3285:
3283:
3273:
3272:
3270:
3269:
3264:
3262:Dent's disease
3259:
3253:
3251:
3245:
3244:
3242:
3241:
3236:
3231:
3226:
3216:
3214:
3208:
3207:
3205:
3204:
3199:
3194:
3189:
3183:
3181:
3174:
3173:
3171:
3170:
3165:
3160:
3151:
3150:
3145:
3143:Norrie disease
3140:
3130:
3125:Eye disorders:
3121:
3120:
3115:
3110:
3105:
3093:
3091:
3089:Nervous system
3085:
3084:
3082:
3081:
3075:Menkes disease
3065:
3064:
3052:
3051:
3039:
3038:
3026:
3025:
3020:
3015:
3003:
3002:
2990:
2989:
2984:
2972:
2970:
2964:
2963:
2961:
2960:
2955:
2950:
2940:
2938:
2932:
2931:
2929:
2928:
2923:
2918:
2912:
2910:
2904:
2903:
2901:
2900:
2895:
2890:
2885:
2880:
2875:
2870:
2865:
2859:
2857:
2851:
2850:
2845:
2838:
2837:
2831:
2829:
2828:
2821:
2814:
2806:
2797:
2796:
2793:
2792:
2790:
2789:
2788:
2787:
2779:
2778:
2777:
2775:Haematosalpinx
2772:
2767:
2762:
2757:
2749:
2748:
2747:
2742:
2737:
2729:
2728:
2727:
2722:
2717:
2712:
2707:
2698:
2696:
2692:
2691:
2689:
2688:
2687:
2686:
2676:
2671:
2666:
2661:
2655:
2653:
2649:
2648:
2645:
2644:
2642:
2641:
2636:
2631:
2626:
2621:
2616:
2611:
2606:
2601:
2600:
2599:
2594:
2589:
2578:
2576:
2570:
2569:
2567:
2566:
2565:
2564:
2559:
2547:
2546:
2545:
2535:
2534:
2533:
2522:
2520:
2514:
2513:
2511:
2510:
2505:
2499:
2498:
2497:
2496:
2491:
2481:
2480:
2479:
2477:Evans syndrome
2464:
2462:
2453:
2446:
2442:
2441:
2438:
2437:
2435:
2434:
2429:
2424:
2423:
2422:
2417:
2412:
2410:Peabody's sign
2407:
2402:
2397:
2392:
2387:
2376:
2374:
2370:
2369:
2367:
2366:
2361:
2356:
2351:
2346:
2340:
2338:
2332:
2331:
2329:
2328:
2323:
2322:
2321:
2311:
2310:
2309:
2304:
2302:Thrombocytosis
2299:
2289:
2288:
2287:
2282:
2277:
2272:
2267:
2262:
2251:
2249:
2242:
2238:
2237:
2235:
2234:
2229:
2224:
2218:
2215:
2214:
2204:
2202:
2201:
2194:
2187:
2179:
2154:
2147:
2146:
2145:
2141:
2140:
2137:
2136:
2125:
2114:
2102:
2101:
2099:
2095:
2094:
2091:
2090:
2079:
2068:
2057:
2042:
2027:
2011:
2006:
2005:
2003:
2002:Classification
1995:
1994:External links
1992:
1991:
1990:
1945:
1916:
1877:
1824:
1811:
1796:
1751:
1748:
1745:
1744:
1699:(3): 510â516.
1679:
1628:
1609:(3): 277â282.
1589:
1570:(3): 235â246.
1549:
1523:
1472:
1446:
1420:
1405:
1368:(4): 377â382.
1348:
1311:(5): 580â586.
1291:
1266:
1209:
1201:
1181:
1162:(3): 235â246.
1141:
1122:(4): 310â318.
1101:
1077:
1060:
1036:
987:
980:
949:
910:(1): 134â136.
890:
879:
850:(1): 432â437.
830:
803:
796:
776:
769:
738:
710:
678:
639:
638:
636:
633:
631:
630:
625:
620:
615:
610:
604:
602:
599:
590:
587:
550:
547:
521:
518:
509:
506:
500:
497:
429:
426:
397:
394:
376:
373:
352:family history
319:
316:
290:X-inactivation
262:
259:
250:
247:
226:
223:
222:
221:
216:
211:
206:
172:
169:
125:
124:
118:
114:
113:
110:
106:
105:
102:
96:
95:
92:
88:
87:
84:
78:
77:
72:
66:
65:
61:
60:
52:
51:
48:
44:
43:
35:
24:
14:
13:
10:
9:
6:
4:
3:
2:
3457:
3446:
3443:
3441:
3438:
3436:
3433:
3431:
3428:
3427:
3425:
3406:
3403:
3401:
3398:
3396:
3393:
3391:
3388:
3386:
3385:Rett syndrome
3383:
3381:
3378:
3376:
3373:
3371:
3368:
3366:
3363:
3361:
3358:
3357:
3354:
3350:
3345:
3341:
3331:
3328:
3326:
3323:
3321:
3318:
3316:
3313:
3311:
3308:
3306:
3303:
3302:
3300:
3296:
3290:
3287:
3286:
3284:
3282:
3278:
3274:
3268:
3265:
3263:
3260:
3258:
3255:
3254:
3252:
3250:
3246:
3240:
3237:
3235:
3232:
3230:
3227:
3225:
3221:
3218:
3217:
3215:
3213:
3212:Neuromuscular
3209:
3203:
3200:
3198:
3195:
3193:
3190:
3188:
3185:
3184:
3182:
3179:
3175:
3169:
3166:
3164:
3161:
3159:
3156:
3153:
3152:
3149:
3148:Choroideremia
3146:
3144:
3141:
3138:
3134:
3131:
3129:
3126:
3123:
3122:
3119:
3116:
3114:
3111:
3109:
3108:MASA syndrome
3106:
3104:
3101:
3099:
3095:
3094:
3092:
3090:
3086:
3080:
3076:
3073:
3071:
3067:
3066:
3063:
3060:
3058:
3054:
3053:
3050:
3047:
3045:
3041:
3040:
3037:
3036:Fabry disease
3033:
3032:
3028:
3027:
3024:
3021:
3019:
3016:
3014:
3011:
3009:
3005:
3004:
3001:
2998:
2996:
2992:
2991:
2988:
2985:
2983:
2980:
2978:
2974:
2973:
2971:
2969:
2965:
2959:
2956:
2954:
2951:
2949:
2945:
2942:
2941:
2939:
2937:
2933:
2927:
2924:
2922:
2921:Haemophilia B
2919:
2917:
2916:Haemophilia A
2914:
2913:
2911:
2909:
2905:
2899:
2896:
2894:
2891:
2889:
2886:
2884:
2881:
2879:
2876:
2874:
2871:
2869:
2866:
2864:
2861:
2860:
2858:
2856:
2852:
2848:
2843:
2839:
2834:
2827:
2822:
2820:
2815:
2813:
2808:
2807:
2804:
2786:
2785:Haemarthrosis
2783:
2782:
2780:
2776:
2773:
2771:
2768:
2766:
2763:
2761:
2758:
2756:
2753:
2752:
2750:
2746:
2743:
2741:
2738:
2736:
2733:
2732:
2730:
2726:
2723:
2721:
2718:
2716:
2713:
2711:
2708:
2706:
2703:
2702:
2700:
2699:
2697:
2693:
2685:
2682:
2681:
2680:
2677:
2675:
2672:
2670:
2667:
2665:
2662:
2660:
2657:
2656:
2654:
2650:
2640:
2637:
2635:
2632:
2630:
2627:
2625:
2622:
2620:
2617:
2615:
2612:
2610:
2607:
2605:
2602:
2598:
2595:
2593:
2590:
2588:
2585:
2584:
2583:
2580:
2579:
2577:
2575:
2571:
2563:
2560:
2558:
2555:
2554:
2553:
2552:
2548:
2544:
2541:
2540:
2539:
2536:
2532:
2529:
2528:
2527:
2524:
2523:
2521:
2519:
2515:
2509:
2506:
2504:
2501:
2500:
2495:
2492:
2490:
2487:
2486:
2485:
2482:
2478:
2475:
2474:
2473:
2469:
2466:
2465:
2463:
2461:
2457:
2454:
2450:
2447:
2443:
2433:
2430:
2428:
2425:
2421:
2418:
2416:
2413:
2411:
2408:
2406:
2403:
2401:
2400:Louvel's sign
2398:
2396:
2395:Lisker's sign
2393:
2391:
2388:
2386:
2383:
2382:
2381:
2378:
2377:
2375:
2371:
2365:
2362:
2360:
2357:
2355:
2352:
2350:
2347:
2345:
2344:Thrombophilia
2342:
2341:
2339:
2337:
2333:
2327:
2324:
2320:
2317:
2316:
2315:
2312:
2308:
2305:
2303:
2300:
2298:
2295:
2294:
2293:
2290:
2286:
2283:
2281:
2278:
2276:
2273:
2271:
2268:
2266:
2263:
2261:
2258:
2257:
2256:
2253:
2252:
2250:
2246:
2243:
2239:
2233:
2230:
2228:
2225:
2223:
2220:
2219:
2216:
2212:
2208:
2205:Disorders of
2200:
2195:
2193:
2188:
2186:
2181:
2180:
2177:
2169:
2168:
2167:
2166:Haemophilia A
2161:
2157:
2135:
2134:Haemophilia A
2131:
2130:
2126:
2124:
2120:
2119:
2115:
2113:
2109:
2108:
2104:
2103:
2100:
2096:
2089:
2085:
2084:
2080:
2078:
2074:
2073:
2069:
2067:
2063:
2062:
2058:
2056:
2052:
2051:
2047:
2043:
2041:
2037:
2036:
2032:
2028:
2026:
2022:
2021:
2017:
2013:
2012:
2009:
2004:
2000:
1993:
1987:
1983:
1979:
1975:
1971:
1967:
1963:
1959:
1955:
1951:
1946:
1942:
1938:
1934:
1930:
1926:
1922:
1917:
1913:
1909:
1904:
1899:
1895:
1891:
1887:
1883:
1878:
1874:
1870:
1865:
1860:
1856:
1852:
1847:
1842:
1838:
1834:
1830:
1825:
1814:
1812:9780470766439
1808:
1804:
1803:
1797:
1793:
1789:
1785:
1781:
1776:
1771:
1767:
1763:
1762:Haematologica
1759:
1754:
1753:
1749:
1740:
1736:
1732:
1728:
1724:
1720:
1715:
1710:
1706:
1702:
1698:
1694:
1686:
1684:
1680:
1675:
1671:
1667:
1663:
1659:
1655:
1651:
1647:
1644:(11): 823â7.
1643:
1639:
1632:
1629:
1624:
1620:
1616:
1612:
1608:
1604:
1600:
1593:
1590:
1585:
1581:
1577:
1573:
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595:haemophilia B
588:
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422:haemophilia C
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406:haemophilia B
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225:Complications
224:
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179:Joint capsule
177:
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163:through slow
162:
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139:
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131:
130:Haemophilia A
123:
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93:
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83:
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76:
73:
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42:Haemophilia A
40:
34:
30:
29:haemophilia B
19:
3154:
3124:
3096:
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3042:
3029:
3006:
2995:Dyslipidemia
2993:
2975:
2915:
2586:
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2525:
2415:Pratt's sign
2227:coagulopathy
2164:
2163:
2162:profile for
2159:
2127:
2116:
2105:
2081:
2070:
2059:
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2029:
2014:
1953:
1949:
1927:(6): 574â9.
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1816:. Retrieved
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1184:
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1115:
1104:
1093:. Retrieved
1089:
1080:
1069:Hemophilia A
1067:
1063:
1052:. Retrieved
1048:
1039:
1004:
1000:
990:
971:
907:
903:
893:
886:
882:
847:
843:
833:
822:. Retrieved
818:
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741:
729:. Retrieved
725:
701:. Retrieved
697:
663:Hemophilia A
662:
592:
589:Epidemiology
552:
532:
523:
514:gene therapy
511:
508:Gene therapy
502:
493:desmopressin
472:
458:concentrate
450:
436:Desmopressin
399:
389:
385:
381:
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337:
305:
298:
286:heterozygous
272:
255:haemorrhages
252:
234:
230:
228:
189:venepuncture
186:
182:
158:
144:in clotting
140:caused by a
134:hemophilia A
133:
129:
128:
50:Hemophilia A
33:
18:Hemophilia A
3430:Haemophilia
2908:Hematologic
2770:Haematocele
2735:Haemothorax
2710:Haemoptysis
2582:Haemophilia
2538:aggregation
2420:Rose's sign
2390:Homans sign
2222:Coagulation
2107:MedlinePlus
1465:14 December
1439:14 December
1279:www.cdc.gov
1007:(1): 1â18.
760:10665/44053
676:update 2014
608:Haemophilia
575:hepatitis C
571:hepatitis B
543:factor VIII
488:intravenous
479:prophylaxis
475:cannulation
460:factor VIII
453:intravenous
146:factor VIII
75:Haematology
47:Other names
3424:Categories
2977:Amino acid
2760:Haemobilia
2354:Thrombosis
2129:Patient UK
2083:DiseasesDB
1095:2016-07-08
1054:2016-07-08
824:2016-07-08
635:References
618:Ryan White
565:, such as
528:emicizumab
312:known case
282:homozygous
239:factor VII
193:heelpricks
122:emicizumab
109:Prevention
2968:Metabolic
2936:Endocrine
2835:disorders
2705:Epistaxis
2669:Haematoma
2292:Platelets
2123:emerg/239
2118:eMedicine
1986:205100329
1970:0902-4506
1956:: 48â50.
1933:0253-0716
1855:1756-0500
1784:0390-6078
1723:1538-7836
1714:1887/5021
1658:0003-4819
1623:0028-4793
1584:0028-4793
1382:1723-2007
1327:1365-2141
1243:1723-2007
1176:0028-4793
1136:0028-4793
1074:eMedicine
926:0971-6866
866:1520-4391
549:Prognosis
428:Treatment
410:Factor IX
318:Diagnosis
231:inhibitor
117:Treatment
70:Specialty
3249:Urologic
3224:Duchenne
2833:X-linked
2751:abdomen
2720:Hyphaema
2674:Petechia
2659:Bleeding
2526:adhesion
2452:By cause
2445:Bleeding
2349:Thrombus
2248:By cause
2241:Clotting
2211:clotting
2207:bleeding
1978:24957107
1912:25922858
1903:11245682
1873:20529258
1792:18403393
1739:13651790
1731:16460432
1674:22008880
1537:DailyMed
1518:29224506
1400:21839010
1343:25748135
1335:17686052
1261:19105504
1049:omim.org
1031:11836440
944:22754241
874:17124095
672:20301578
601:See also
483:infusion
375:Severity
275:X-linked
261:Genetics
82:Symptoms
3070:Mineral
2695:By site
2679:Purpura
2373:By site
2156:Scholia
2077:D006467
1941:4430880
1864:2896368
1839:: 161.
1666:7486463
1391:3200405
1252:2652218
1090:Patient
1022:1187139
935:3385172
731:24 June
726:Patient
703:24 June
369:surgery
209:Muscles
136:) is a
3155:Other:
2855:Immune
2781:joint
2731:torso
2664:Bruise
2587:A/VIII
2158:has a
2112:000538
2066:306700
1984:
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456:plasma
363:(i.e.
361:trauma
204:Joints
197:fibrin
91:Causes
3281:tooth
3168:SMAX2
2701:head
2336:Clots
2160:topic
2055:286.0
1982:S2CID
1735:S2CID
1670:S2CID
1539:from
1339:S2CID
367:, or
219:Brain
3277:Bone
3178:Skin
3118:PHF8
2888:IPEX
2597:C/XI
2592:B/IX
2209:and
2088:5555
2072:MeSH
2061:OMIM
2050:9-CM
2025:3B10
1974:PMID
1966:ISSN
1937:PMID
1929:ISSN
1908:PMID
1869:PMID
1851:ISSN
1820:2016
1807:ISBN
1788:PMID
1780:ISSN
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1467:2017
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1323:ISSN
1286:2016
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862:ISSN
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792:ISBN
765:ISBN
733:2016
705:2016
668:PMID
573:and
466:, a
412:and
404:are
346:and
235:copy
132:(or
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2472:ITP
2314:DIC
2046:ICD
2040:D66
2031:ICD
2016:ICD
1958:doi
1898:PMC
1890:doi
1859:PMC
1841:doi
1770:doi
1709:hdl
1701:doi
1646:doi
1642:123
1611:doi
1607:391
1572:doi
1568:391
1504:hdl
1494:doi
1490:377
1459:BBC
1386:PMC
1370:doi
1313:doi
1309:138
1247:PMC
1231:doi
1164:doi
1160:391
1124:doi
1120:388
1072:at
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930:PMC
912:doi
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567:HIV
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