Acquired Hemophilia - NORD (National Organization for Rare Disorders) (2022)

Acquired Hemophilia

NORD gratefully acknowledges Graça D. Almeida-Porada, MD, PhD, Professor of Regenerative Medicine, Director Fetal Research and Therapy Program, Wake Forest Institute for Regenerative Medicine, Wake Forest School of Medicine and Francesco Baudo, MD, Hematology Department, Ospedale Niguarda, Milan, Italy, for assistance in the preparation of this report.

Synonyms of Acquired Hemophilia

  • acquired hemophilia A (AHA)
  • acquired hemophilia B (AHB)

General Discussion

Summary

Acquired hemophilia (AH) is a rare autoimmune disorder characterized by bleeding that occurs in patients with no personal or family history of diseases related to clotting/coagulation. Autoimmune disorders occur when the body’s immune system mistakenly attacks healthy cells or tissue. In AH, the body produces antibodies (known as inhibitors) that attack clotting factors, most often factor VIII. Clotting factors are specialized proteins required for the blood to clot normally. Consequently, affected individuals develop complications associated with abnormal, uncontrolled bleeding into the muscles, skin and soft tissue that can occur spontaneously, during surgery or following trauma. Specific symptoms can include nosebleeds (epistaxis), bruising throughout the body, solid swellings of congealed blood (hematomas), blood in the urine (hematuria) and gastrointestinal or urogenital bleeding. AH can potentially cause life-threatening bleeding complications in severe cases. In approximately 50% of patients, there is an identifiable underlying clinical condition; in the other 50%, no cause is known (idiopathic).

Introduction

AH is different from congenital hemophilia, a group of rare genetic disorders caused by mutations in the genes encoding certain clotting factors. The main form of hemophilia is hemophilia A (classic hemophilia), which is an X-linked disorder that mostly affects males but can also affect females. It is caused by deficiency or inactivation of factor VIII, the same clotting factor that is affected in most individuals with AH. Although both disorders involve deficiency of the same clotting factor, the bleeding pattern is quite different. The reason the bleeding patterns differ between these disorders is not fully understood.

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Signs & Symptoms

The symptoms of AH develop because the blood cannot clot properly. Clotting is the process by which blood clumps together to plug the site of a wound. Clotting factors, such as factor VIII, are specialized proteins that are essential for the blood to clot properly.

Although about 1/3 of patients do not require therapy to control bleeds, bleeding severity varies and more than 1/3 of patients suffer multiple bleeding episodes. Subcutaneous bleeding (ecchymoses) is the most common manifestation of AH followed by muscle bleeding (hematoma), gastrointestinal (melena), genitourinary (hematuria) and retroperitoneal. Intracranial hemorrhage is rare, but can be fatal. In contrast to congenital hemophilia A, joint bleeding (hemarthrosis) is infrequent.

Bleeding often occurs without cause (spontaneously).Bleeding episodes are often severe and can become life-threatening. In some patients, delayed diagnosis and the presence of additional medical issues are often contributing factors to the overall severity of AH. Bleeding into the soft tissues can progress rapidly, potentially causing compartment syndrome, a potentially serious, painful condition characterized by increased pressure on muscles, nerves and blood vessels most often within the arms and legs, with damage due to compression of these structures.

Affected Individuals are also at risk of excessive bleeding during surgery or following trauma, even trivial. Genital heavy bleeding in pregnant women may occur especially after childbirth (postpartum period).

Causes

AH is an autoimmune disorder. It occurs when the immune system produces antibodies that mistakenly attack healthy tissue, specifically specialized proteins known as clotting factors, most often clotting factor VIII.

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The immune system normally responds to a foreign substance by producing specialized proteins called antibodies. Antibodies work by destroying foreign substances directly or by coating them with a substance that marks them for destruction by white blood cells. When antibodies target healthy tissue they may be referred to as autoantibodies. Researchers believe that a triggering event (such as an infection or underlying disorder) may induce the immune system to produce autoantibodies. Autoantibodies in AH are termed inhibitors because they inhibit the function of the affected clotting factor.

AH is predominantly a disease of the elderly. In approximately 50% of the patients, no underlying disorder or triggering event can be identified (idiopathic form). The remaining 50% have coexisting disorders or conditions including autoimmune disorders such as lupus, rheumatoid arthritis, multiple sclerosis, Sjogren syndrome, and temporal arteritis; inflammatory bowel disease or ulcerative colitis; infections; diabetes; hepatitis; respiratory or dermatological diseases; blood (hematological) cancer or certain solid tumors. AH has also been associated with drugs such as penicillin or interferon and an association with pregnancy has also been reported, mainly in the post-partum period.

Affected Populations

AH develops in individuals with no previous history of bleeding disorder with approximately equal numbers of males and females affected. In the United States, the disorder is estimated to affect approximately .2-1 individuals per 1,000,000/year in the general population. In the United Kingdom, the disorder is estimated to affect 1.4 per 1,000,000/year. However, affected individuals may go undiagnosed or misdiagnosed, making it difficult to determine the true frequency of the disorder in the general population.

Individuals of any age can be affected, although AH is extremely rare in children. The incidence increases with age and mostly affects elderly individuals (between 60-80 years of age). A small increase in incidence occurs in pregnant woman between the ages of 20-40. AH affects individuals of all ethnic groups and has been reported worldwide. The majority of cases involve deficiency of factor VIII (acquired hemophilia A). A handful of cases have been described that involve deficiency of factor IX (acquired hemophilia B). Although extremely rare, acquired hemophilia involving other clotting factors has also been reported.

Diagnosis

AH should be suspected by the clinical picture and confirmed by an abnormal coagulation test. A diagnosis should be considered in patients with a recent onset of abnormal bleeding and an isolated prolongation of the activated partial thromboplastin time (aPTT), especially the elderly and peri- and post-partum women.

Clinical Testing and Work-Up

Routine first line coagulation tests include activated partial thromboplastin time (aPTT) and prothrombin time (PT). The two tests measure coagulation time of plasma, triggered by two different tissue factors (in aPTT partial thromboplastin). aPTT is sensitive mainly to FVIII, FIX, FXI and XII, whereas PT is sensitive to coagulation proteins synthesized by the liver (FII, FVII, FIX, the so called “prothrombin complex” with synthesis depending on vitamin K).

Individuals with AH have an isolated prolonged aPTT, with normal PT. Tests to rule out other causes of isolated prolonged aPTT such as non-specific inhibitors (e.g., lupus anticoagulant) or heparin therapy are also performed.

aPTT mixing tests, carried out by mixing patient’s plasma with normal plasma, can further confirm the diagnosis. A mixing study differentiates genetic factor deficiencies from factor inhibitors. A sample of blood is taken and mixed with blood from a control subject. In individuals with a factor deficiency the normal plasma restores the test value to normal; in individuals with a factor inhibitor it does not.

Once a factor inhibitor is established, an assay will be done to measure the activity of coagulation factors (in the majority of cases FVIII) and the titer of the inhibitor. In individuals with AHA, this will demonstrate factor VIII deficiency and can ascertain the severity (titer count) as well.

Standard Therapies

Treatment
Because AH is a rare disorder, most therapies used to treat affected individuals are based upon anecdotal reports or small cases series. There are few studies directly comparing the efficacy of specific treatments. Consequently, treatment is highly individualized.

The specific therapeutic procedures and interventions will vary, depending upon numerous factors including the specific symptoms present; the natural course of the disorder including underlying cause (if known); age and overall health (e.g., concomitant disease), tolerance of certain medications or procedures, and personal preference; and other factors. Decisions concerning the use of particular therapeutic interventions should be made by physicians and other members of the healthcare team in careful consultation with the patient and/or parents based upon the specifics of his or her case; a thorough discussion of the potential benefits and risks including possible side effects and long-term effects; patient preference; and other appropriate factors.

Spontaneous remission has been reported; in general, it may occur in postpartum cases (within a few months of delivery) and, in the cases secondary to an allergic drug reaction, usually within a few months of stopping the offending medication. Spontaneous remission can also occur in other affected individuals such as those who have low titer inhibitors. The exact percentage of patients that undergo spontaneous resolution is unknown.

The management of AH focuses on the following goals: controlling and preventing bleeding (if present or significant), eradication of the inhibitor, and treatment of the underlying disease (if applicable).

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Controlling Bleeding Episodes

Bleeding may be very severe and may have a sudden onset. Therefore, prompt hemostatic control is mandatory in order to reduce morbidity and mortality. The International Recommendations state that anti-hemorrhagic treatment should be started in patients with severe bleeding in which a diagnosis of AH is confirmed, irrespective of inhibitor titer and factor VIII activity. Two approaches are available: the use of bypassing agents (concentrates of factors that bypass the acquired deficiency) or strategies to increase FVIII levels. The choice between these two options is based on the site and the severity of bleeding and the characteristics of each individual patient. Since hemostatic agents do not have a predictable effect, treatment of bleeding should be supervised by an expert in this field and proper laboratory tests. Imaging techniques and a skillful clinical evaluation are necessary to confirm hemostatic control and bleeding resolution. Fibrin glue or antifibrinolytic agents may be useful in the control of local bleeding.

Bypassing agents are the recommended first-line therapy due to their rapid action and high level of effectiveness. The dosage is largely based on experience with the management of patients with FVIII inhibitors in congenital hemophilia and is generally based on the clinical assessment.

The bypassing agents presently available are recombinant activated factor VII (rFVIIa, NovoSeven® RT, or Sevenfact) or activated prothrombin complex concentrate (aPCC or FEIBA®). None of these therapies are effective in all individuals.

NovoSeven® RT is a genetically engineered (recombinant) version of factor VII. Because it is artificially created in a lab, it does not contain human blood or plasma and consequently, there is no risk of blood-borne viruses or other such pathogens. NovoSeven has been well-tolerated and associated with few side effects. Risk of thrombotic adverse effects (thrombosis) is below 1% for individuals with AH. NovoSeven has been approved by the Food and Drug Administration (FDA) for use as a bypassing agent for the treatment of individuals with acquired hemophilia.

In 2020, the FDA approved Sevenfact (recombinant human coagulation factor VIIa expressed in the mammary gland of genetically engineered rabbits and secreted into the rabbits’ milk) for treatment and control of bleeding in adults and adolescents age 12 and older with hemophilia A or B with inhibitors. It is presumed that Sevenfact could also be used in the treatment of acquired hemophilia A, but studies have not yet been published to corroborate this assumption.

Activated prothrombin complex concentrate (aPCC) is a plasma-derived, anti-inhibitor complex that contains various activated clotting factors. These factors allow the drug to bypass certain steps in the formation of blood clots (including the steps that require factor VIII). aPCC is treated to inactivate any potential viruses or similar pathogens and adverse thrombotic events are rare. The only form of aPCC currently available in the United States is FEIBA® (Factor eight inhibitor bypassing activity).

Therapeutic modalities that allow an increase of FVIII, such as infusion of FVIII concentrate or DDAVP, that induces release of FVIII by the endothelial cells, are usually considered inadequate unless the inhibitor titer is very low (i.e. < 5 Bethesda units [BU]) and bypassing agents are not available. In 2014 The US FDA approved Obizur [Antihemophilic Factor (Recombinant), Porcine Sequence] for the treatment of bleeding episodes in adults with acquired hemophilia A (acquired Factor VIII [FVIII] deficiency).

Inhibitor Eradication

Although in some cases inhibitor can disappear spontaneously, as long as the inhibitor is present, bleeding-related morbidity and mortality is significant. Therefore, therapy to eradicate the inhibitor (immunosuppression therapy) in adults is recommended to start immediately after the diagnosis of AH unless clearly contraindicated. Recommendations are largely derived from observation made in registries that have collected real life data.

In general, corticosteroids alone or in combination with cyclophosphamide are the first line therapy. No clear difference in long-term survival was observed between these two modalities. However, individuals respond differently to immunosuppressive drugs, and what is effective in one individual may be ineffective in another. A variety of additional immunosuppressive agents have been used to treat acquired hemophilia including cyclosporine A, azathioprine, vincristine, mycophenolate mofetil, and 2-chlorodeoxyadenosine.

Criteria for the response to treatment have not been established; nevertheless, high inhibitor titer and low FVIII level seem to be the best predictor of the response to therapy.

Relapse of AH can occur in individuals who achieve remission once immunosuppressive therapy is stopped or if the dose is reduced. Unfortunately, because of the associated side effects, long-term immunosuppressive therapy is not recommended.

Individuals with AH are encouraged to avoid activities that have a significant risk of trauma until after inhibitor eradication.

Patients with AH will benefit from referral to a federally-funded hemophilia treatment center. These specialized centers can provide comprehensive care for individuals with hemophilia and related disorders including the development of specific treatment plans, monitoring and follow-up of affected individuals, and state-of-the-art medical care. Treatment at a hemophilia treatment center ensures that individuals and their family members will be cared for by a professional healthcare team (physicians, nurses, physical therapists, social workers and genetic counselors) experienced at treating individuals with hemophilia.

Investigational Therapies

Researchers have been studying the drug rituximab, an anti-CD20 monoclonal antibody, as a potential therapy for individuals with AH. This drug attacks the autoantibodies that cause the disease and has shown promising results in eradicating inhibitors in AH. The current consensus is that rituximab should be considered in patients who are resistant to first-line therapy or who cannot tolerate standard immunosuppressive therapy. However, some clinicians have further fine-tuned recommended use of rituximab to allow its inclusion as first-line therapy in combination with prednisone for patients whose inhibitor titers are higher than 5 Bethesda units (BU) but lower than 30 BU and in combination with prednisone and cyclophosphamide for patients whose titers are higher than 30. More research is necessary to determine the long-term safety and effectiveness of rituximab for the treatment of AH.

High-dose intravenous immunoglobulin has been explored as a means to eradicate inhibitors in AH. However, most reports in the medical literature detail disappointing results. Some researchers believe that this therapy best holds promise as an adjunct treatment to other drugs or procedures that eradicate inhibitors.

Some individuals who have high titers of inhibitors and severe bleeding may undergo a procedure called plasmapheresis or immunoabsorption. These procedures are usually reserved for patients who have not responded to other treatment options and are experiencing life-threatening bleeding episodes. Plasmapheresis involves removing unwanted substances from the blood. Blood is removed from the patient and the solid blood cells are separated from the liquid plasma. The patient’s plasma is then replaced with donor human plasma or albumin, which is re-transfused, along with the patient’s original blood cells. The modified Bonn-Malmö Protocol (MBMP) combines immunoadsorption on columns that specifically link immunoglobulins (FVIII inhibitor) with FVIII replacement and immunosuppression and can achieve rapid and safe control of acute bleeding. It is mainly implemented in Europe since immunoadsorption columns are not available in the US.

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Complications of immunosuppressive therapy (IST)

IST increases risk for infection in the already elderly, fragile population. Neutropenia (decrease of white blood cells) and sepsis are frequently reported and contributed to death. Other well-recognized complications of steroid therapy have been reported including raised blood sugar levels, gastrointestinal ulcers, muscle disorders, and psychiatric disorders (3%).

Conclusions: Research and Other Needs

AH is more frequently encountered by the internist or emergency room doctor who is most often the first to evaluate the patient. It is likely that AH is underdiagnosed and misdiagnosed in real-world clinical practice, suggesting the need to raise awareness of this disease among healthcare practitioners and to encourage referral to specialists in the management of AH. For most, AH is eminently treatable, with clear benefits from prompt recognition and appropriate management. Both hemostatic and immune therapy imposes considerable risks and requires close monitoring to ensure safety. The highest priority in AH research is to develop safer immunosuppressive regimens that improve survival by better balancing inhibitor eradication with the side effects of treatment.

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov . All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Toll-free: (800) 411-1222
TTY: (866) 411-1010
Email:[emailprotected]

Some current clinical trials also are posted on the following page on the NORD website:
https://rarediseases.org/for-patients-and-families/information-resources/news-patient-recruitment/

For information about clinical trials sponsored by private sources, in the main, contact:
www.centerwatch.com

For information about clinical trials conducted in Europe, contact:
https://www.clinicaltrialsregister.eu/

NORD Member Organizations

  • Hemophilia Federation of America
  • Hemophilia Foundation of Southern California
  • National Hemophilia Foundation
    • 7 Penn Plaza
    • Suite 1204
    • New York, NY 10001 USA
    • Phone: (212) 328-3700
    • Toll-free: (800) 424-2634
    • Email: [emailprotected]
    • Website: http://www.hemophilia.org

Other Organizations

  • Canadian Hemophilia Society
    • 301-666 Sherbrooke Street West
    • Montreal, QC H3A 1E7 Canada
    • Phone: (514) 848-0503
    • Toll-free: (800) 668-2686
    • Email: [emailprotected]
    • Website: http://www.hemophilia.ca
  • Genetic and Rare Diseases (GARD) Information Center
  • Irish Haemophilia Society
  • NIH/National Heart, Lung and Blood Institute
  • World Federation of Hemophilia

References

JOURNAL ARTICLES
Tiede A, Hofbauer CJ, Werwitzke S, et al. Anti-factor VIII IgA as a potential marker of poor prognosis in acquired hemophilia A: results from the GTH-AH 01/2010 Study. Blood. 2016;127:2289-2297. http://www.ncbi.nlm.nih.gov/pubmed/26912467

Kruse-Jarres R, St-Louis J, Greist A, et al. Efficacy and safety of OBI-1, an antihaemophilic factor VIII (recombinant), porcine sequence, in subjects with acquired haemophilia A. Haemophilia. 2015;21:162-170. http://www.ncbi.nlm.nih.gov/pubmed/25623166

Tiede A, Klamroth R, Scharf RE, et al. Prognostic factors for remission of and survival in acquired hemophilia A (AHA): Results from the GTH-AH 01/2010 study. Blood. 2015;125:1091-1097. http://www.ncbi.nlm.nih.gov/pubmed/25525118

Borg JY, Guillet B, Le Cam-Duchez V, Goudemand J, Levesque H, Group SS. Outcome of acquired haemophilia in France: the prospective SACHA (Surveillance des Auto antiCorps au cours de l’Hemophilie Acquise) registry. Haemophilia. 2013;19:564-570. http://www.ncbi.nlm.nih.gov/pubmed/23574453

Seita I AK, Higasa S, Sawada A, Kuwahara M, Shima M. . Treatment of acute bleeding episodes in acquired haemophilia with recombinant activated factor VII (rFVIIa): analysis from 10-year Japanese post-marketing surveillance. J Thromb Haemost. 2013;11:119.

Baudo F, Collins P, Huth-Kuhne A, et al. Management of bleeding in acquired hemophilia A: Results from the European Acquired Haemophilia (EACH2) registry. Blood. 2012;120:39-46. http://www.ncbi.nlm.nih.gov/pubmed/22618709

Collins P, Baudo F, Knoebl P, et al. Immunosuppression for acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2). Blood. 2012;120:47-55. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3390961/

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Kempton CL, Abshire TC, Deveras RA, et al. Pharmacokinetics and safety of OBI-1, a recombinant B domain-deleted porcine factor VIII, in subjects with haemophilia A. Haemophilia. 2012;18:798-804. http://www.ncbi.nlm.nih.gov/pubmed/22512291

Knoebl P, Marco P, Baudo F, et al. Demographic and clinical data in acquired hemophilia A: results from the European Acquired Haemophilia (EACH2) Registry. J Thromb Haemost. 2012;[Epub ahead of print]. http://www.ncbi.nlm.nih.gov/pubmed/22321904

Baudo F, Caimi T, de Cataldo F. Diagnosis and treatment of acquired haemophilia. Haemophilia. 2010;16:102-106. http://www.ncbi.nlm.nih.gov/pubmed/20536992

Kelesidis T, Raphael J, Blanchard E, Parameswaran R. Acquired hemophilia as the cause of life-threatening hemorrhage in a 94-year-old man: a case report. J Med Case Reports. 2010:4:231. http://www.ncbi.nlm.nih.gov/pubmed/20670435

Huth-Kunhe A, Baudo F, Collins P, et al. International recommendations on the diagnosis and treatment of patients with acquired hemophilia A. Haematologica. 2009;94:566-575. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2663620/?tool=pubmed

Franchini M, Lippi G. Acquired factor VIII inhibitors. Blood. 2008;112:250-255. http://bloodjournal.hematologylibrary.org/content/112/2/250.full

Ma AD, Carrizosa D. Acquired factor VIII inhibitors: pathophysiology and treatment. Hematology Am Soc Hematol Educ Program. 2006:432-437. http://www.ncbi.nlm.nih.gov/pubmed/17124095

Maillard H, Launay D, Hachulla E, Goudemand J, Lambert M, Morell-Dubois S, et al. Rituximab in postpartum-related acquired hemophilia. Am J Med. 2006 Jan. 119(1):86-8.

Aggarwal A, Grewal R, Green RJ, Boggio L, Green D, Weksler BB, et al. Rituximab for autoimmune haemophilia: a proposed treatment algorithm. Haemophilia. 2005 Jan. 11(1):13-9.

Franchini M, Gandini G, di Paolantonio T, Mariani G. Acquired hemophilia A: a concise review. Am J Hematol. 2005;80:55-63. http://onlinelibrary.wiley.com/doi/10.1002/ajh.20390/pdf

Franchini M, Girelli D, Olivieri O, et al. Clinical heterogeneity of acquired hemophilia A: a description of 4 cases. Haematologica. 2005;90:e46-e49. http://www.haematologica.org/content/90/3/ECR16.long

Freedman J, Garvey MB. Immunoadsorption of factor VIII inhibitors. Curr Opin Hematol. 2004;11:327-333. http://www.ncbi.nlm.nih.gov/pubmed/15666656

Kain S, Copeland TS, Leahy MF. Treatment of refractory autoimmune (acquired) haemophilia with anti-CD20 (rituximab). Br J Haematol. 2002 Nov. 119(2):578.

Wiestner A, Cho HJ, Asch AS, Michelis MA, Zeller JA, Peerschke EI, et al. Rituximab in the treatment of acquired factor VIII inhibitors. Blood. 2002 Nov 1. 100(9):3426-8.

INTERNET
Grethlein SJ. Acquired Hemophilia Treatment & Management. Medscape. Last update January 10, 2020. https://emedicine.medscape.com/article/211186-treatment. Accessed April 26, 2021.

Years Published

2012, 2016, 2021

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FAQs

How rare is acquired haemophilia? ›

Acquired hemophilia is an extremely rare condition affecting around one in a million. It's usually diagnosed when patients present prolonged bleeding, or extensive bruising on the truck, legs and arms which looks very different from usual injury-related bruising.

Why is it called Christmas disease? ›

Hemophilia B is also known as Christmas disease. It is named after the first person to be diagnosed with the disorder in 1952, Stephen Christmas. As the second most common type of hemophilia, it occurs in about 1 in 25,000 male births and affects about 4,000 individuals in the United States.

What is the rarest form of hemophilia? ›

Hemophilia A and B are the most common types, while hemophilia C is relatively rare. Additionally, there is an acquired form of the disease, which results from a fault in the body's immune system that causes it to attack and destroy certain blood clotting proteins, called clotting factors.

How is acquired hemophilia diagnosed? ›

Diagnosis includes screening tests and clotting factor tests. Screening tests are blood tests that show if the blood is clotting properly. Clotting factor tests, also called factor assays, are required to diagnose a bleeding disorder. This blood test shows the type of hemophilia and the severity.

Can acquired hemophilia A be cured? ›

Treatment. Once diagnosed, acquired hemophilia can be successfully treated. The first step in treating it includes controlling immediate bleeding, followed by eliminating FVIII antibodies from the body.

Is acquired hemophilia hereditary? ›

Hemophilia A and B are inherited as X-linked recessive genetic disorders, while hemophilia C is inherited as an autosomal recessive genetic disorder. Hemophilia A and B are mostly expressed in males, but females can also be affected. Hemophilia C affects males and females in equal numbers.

How long do people with hemophilia live? ›

Estimated median life expectancy of patients with hemophilia was 77 years, 6 years lower than the median life expectancy of the general Dutch male population (83 years).

Can a woman with hemophilia have a baby? ›

If the mother is a hemophilia carrier, there is a chance that the baby will be born with hemophilia. In families with a known history of hemophilia, or in those with a prenatal genetic diagnosis of hemophilia, one can plan special testing for hemophilia before the baby's delivery.

What are 5 symptoms of hemophilia? ›

Symptoms
  • Unexplained and excessive bleeding from cuts or injuries, or after surgery or dental work.
  • Many large or deep bruises.
  • Unusual bleeding after vaccinations.
  • Pain, swelling or tightness in your joints.
  • Blood in your urine or stool.
  • Nosebleeds without a known cause.
  • In infants, unexplained irritability.
7 Oct 2021

What organs does hemophilia affect? ›

Hemophilia can result in: Bleeding within joints that can lead to chronic joint disease and pain. Bleeding in the head and sometimes in the brain which can cause long term problems, such as seizures and paralysis. Death can occur if the bleeding cannot be stopped or if it occurs in a vital organ such as the brain.

What are the 3 types of hemophilia? ›

Types of Hemophilia
  • Hemophilia A: Caused by a lack of the blood clotting factor VIII; approximately 85% of hemophiliacs have type A disease.
  • Hemophilia B: Caused by a deficiency of factor IX.
  • Hemophilia C: Some doctors use this term to refer to a lack of clotting factor XI.

How is acquired hemophilia blood treated? ›

Bypassing agents are currently the most used first-line treatment, and both the recombinant activated factor VII (rFVIIa) and the activated prothrombin complex concentrate (aPCC) factor 8 inhibitor bypassing activity (FEIBA) have been proven effective in the treatment of acquired hemophilia A.

What is the most common acquired bleeding disorder? ›

Platelet disorders are the most common cause of bleeding disorder and are usually acquired rather than inherited.

At what age do symptoms of hemophilia appear? ›

Based on CDC data, the median age at diagnosis is 36 months for people with mild hemophilia, 8 months for those with moderate hemophilia, and 1 month for those with severe hemophilia. In about two thirds of cases, there is a family history of hemophilia.

Is hemophilia an autoimmune disease? ›

Acquired hemophilia is a spontaneous autoimmune disorder in which patients with previously normal hemostasis develop autoantibodies against clotting factors, most frequently FVIII.

What causes hemophilia later in life? ›

What causes hemophilia? In most cases, hemophilia is an inherited disease. This means a person who has hemophilia inherited the genetic mutation that causes it from one or both parents. In rare cases, a person develops hemophilia late in life, sometimes because of an underlying autoimmune disease.

How does hemophilia affect everyday life? ›

The most frequent difficulties in everyday life for people living with hemophilia were indicated to be mobility problems, unexpected bleeding, pain, and uncertainties in daily activities impacting the choice of hobbies.

What race is most affected by hemophilia? ›

Prevalence was higher among whites (15.1) than blacks (12.4) or Hispanics of either race (12.4). State-specific prevalence varied from 1.6 to 23.3 cases per 100 000. Based on 9587 males born during the index period, the average haemophilia incidence was 1 case per 4334 live male births.

Can a father pass hemophilia to his daughter? ›

Haemophilia is an inherited condition that occurs in families. The haemophilia gene is passed down from parent to child through generations. Mainly males have haemophilia. Men with haemophilia will pass the gene on to their daughters.

What is the most common cause of hemophilia? ›

The primary cause of all types of hemophilia is a mutation in the genes that control and regulate the development of clotting factors. Clotting factors help the blood form clots that seal up wounds. For both hemophilia A and B, about two-thirds of the mutations come from a parent.

What is the quality of life for a person with hemophilia? ›

The mean total Hemophilia-Specific Quality of Life score was 35.55. 'Sports and leisure'and 'Physical health'were the most impaired dimensions and the dimension 'Relationship and partners'was the least impaired.

Can you drink alcohol with hemophilia? ›

Think things through, make your choices wisely, and consider the following: Drinking even small amounts of alcohol can affect blood clotting. Drinking alcohol is similar to taking aspirin—it acts like a blood thinner. If you have a bleeding disorder, drinking alcohol worsens your clotting issues.

Can people with hemophilia have kids? ›

When a hemophilia carrier female is pregnant, there is a 50/50 chance that the hemophilia gene will be passed on to the baby. If the gene is passed on to a son, he will have the disease. If the gene is passed on to a daughter, she will be a carrier.

Does vitamin K help hemophilia? ›

Supplemental vitamin K may be indicated for hemophilia patients under medical supervision. Careful consideration should be taken when cooking and dining out. Certain herbs can make bleeding more severe and can affect clotting. Examples include ginkgo, garlic, ginseng, ginger, horse chestnut, turmeric, and white willow.

Do hemophiliacs have periods? ›

Thus, the diseases primarily affect the body's soft tissues, and patients can suffer complications including frequent and severe nose bleeds, extremely heavy menstrual periods and bleeding gums.

Can a father pass hemophilia to his son? ›

A father who has hemophilia passes his only X chromosome down to all of his daughters, so they will always get his hemophilia allele and be heterozygous (carriers). A father passes down his Y chromosome to his sons; thus, he cannot pass down a hemophilia allele to them.

Is hemophilia A disability? ›

Fortunately, hemophilia is included in the Social Security Administration's (SSA) Blue Book of impairment listings, which means it may be possible to obtain benefits if you meet the requirements of the listing. This condition is included in section 7.08 for disorders of thrombosis and hemostasis.

Is hemophilia A fatal disease? ›

Even though the disorder is rare, acquired hemophilia is a serious condition in which severe bleeding can occur in a significant number (70%) of cases and it is fatal in about five to ten percent of the cases [8,16].

How do I know I'm internally bleeding? ›

The signs and symptoms that suggest concealed internal bleeding depend on where the bleeding is inside the body, but may include:
  1. pain at the injured site.
  2. swollen, tight abdomen.
  3. nausea and vomiting.
  4. pale, clammy, sweaty skin.
  5. breathlessness.
  6. extreme thirst.
  7. unconsciousness.

What percent of females have hemophilia? ›

Women and girls were less likely than men and boys to have severe hemophilia or moderate hemophilia (only 8% of women and girls had severe or moderate disease, compared to 70% of men and boys). 92% of women and girls had mild hemophilia, compared to 30% of men and boys.

Can blood thinners cause hemophilia? ›

Side effects of blood thinners

uncontrolled high blood pressure. stomach ulcers or other issues that put you at high risk for internal bleeding. hemophilia or other bleeding disorders.

Why is it called hemophilia? ›

The word hemophilia derives from two Greek words: haima, meaning blood, and philia, meaning affection. Hemophilia is an hereditary condition. This means that it is passed on from mother to child at the time of conception. The blood of a person with hemophilia does not clot normally.

What vitamins are good for hemophilia? ›

Iron, vitamins, and dietary supplements

The main nutrients involved in red blood cell production are iron, protein, copper, vitamin C, vitamin B12, vitamin B6 and folic acid — vitamin E is also involved in red blood cell production, but it can increase the risk of bleeding in high doses.

What can make hemophilia worse? ›

High-fat foods

In turn, this can make your hemophilia symptoms worse. People who struggle with their weight often eat too many foods with saturated fat, such as butter, milk, cheese, fatty proteins and processed foods.

What do you put on hemophilia medical bracelets? ›

What to engrave for bleeding disorders?
  • Your Name.
  • Medical Conditions: list the bleeding disorder. ...
  • Any allergies to medication, food, insect, metals, etc.
  • Any medication you take for your condition.
  • Emergency contact information (ICE “In Case of Emergency”) such as a phone number.

What is another name for hemophilia? ›

The major types of this condition are hemophilia A (also known as classic hemophilia or factor VIII deficiency) and hemophilia B (also known as Christmas disease or factor IX deficiency).

Is hemophilia A or B worse? ›

Haemophilia B is clinically less severe than haemophilia A: further evidence - PMC.

What is severe hemophilia? ›

Severe hemophilia A occurs in individuals with less than 1% of normal factor VIII clotting activity. In contrast to the mild or moderate forms of the disease, severe hemophilia A is associated with spontaneous bleeding episodes.

How common is haemophilia? ›

Hemophilia is a rare disorder. It can occur in all races and ethnic groups. Hemophilia A affects 1 in 5,000 to 10,000 males. Hemophilia B is less common, affecting 1 in 25,000 to 30,000 males.

What is the most common acquired bleeding disorder? ›

Platelet disorders are the most common cause of bleeding disorder and are usually acquired rather than inherited.

What causes hemophilia later in life? ›

What causes hemophilia? In most cases, hemophilia is an inherited disease. This means a person who has hemophilia inherited the genetic mutation that causes it from one or both parents. In rare cases, a person develops hemophilia late in life, sometimes because of an underlying autoimmune disease.

How is acquired hemophilia treated? ›

Currently, the recommended hemostatic treatment for clnically relevant bleeding in patients with acquired hemophilia is with recombinant porcine FVIII (rpFVIII) or with an agent that bypasses FVIII: recombinant activated factor VIIa (rFVIIa) or an activated prothrombin complex concentrate (APCC).

How long can you live with hemophilia? ›

Life expectancy in hemophilia varies, depending on whether patients receive appropriate treatment. Many patients still die before adulthood due to inadequate treatment. With proper treatment, life expectancy is only about 10 years less than healthy men.

What are 5 symptoms of hemophilia? ›

Symptoms
  • Unexplained and excessive bleeding from cuts or injuries, or after surgery or dental work.
  • Many large or deep bruises.
  • Unusual bleeding after vaccinations.
  • Pain, swelling or tightness in your joints.
  • Blood in your urine or stool.
  • Nosebleeds without a known cause.
  • In infants, unexplained irritability.
7 Oct 2021

What are the 3 types of hemophilia? ›

Types of Hemophilia
  • Hemophilia A: Caused by a lack of the blood clotting factor VIII; approximately 85% of hemophiliacs have type A disease.
  • Hemophilia B: Caused by a deficiency of factor IX.
  • Hemophilia C: Some doctors use this term to refer to a lack of clotting factor XI.

What causes acquired bleeding disorders? ›

Acquired bleeding disorders are the most common causes of bleeding seen in the practice of hematology. These entities can be due to interference with the platelet plug formation, interference with blood coagulation proteins, and interference with both.

What is the difference between hereditary bleeding disorders and acquired bleeding disorders? ›

Bleeding disorders fall into two main categories: inherited and acquired. Inherited bleeding disorders have a genetic predisposition and involve a deficiency of coagulation factors. Acquired bleeding disorders can be caused by conditions that an individual may develop at any point during their lifetime.

How do you know if you have a bleeding disorder? ›

Signs and symptoms of a bleeding disorder:

Bleeding that lasts longer than 7 days from the time bleeding starts until the time it ends; Flooding or gushing of blood that limits daily activities, such as work, school, exercise, or social activities; Passing clots that are bigger than a grape; and.

What part of the body does hemophilia affect? ›

Signs and Symptoms

Common signs of hemophilia include: Bleeding into the joints. This can cause swelling and pain or tightness in the joints; it often affects the knees, elbows, and ankles. Bleeding into the skin (which is bruising) or muscle and soft tissue causing a build-up of blood in the area (called a hematoma).

Is hemophilia A disability? ›

Fortunately, hemophilia is included in the Social Security Administration's (SSA) Blue Book of impairment listings, which means it may be possible to obtain benefits if you meet the requirements of the listing. This condition is included in section 7.08 for disorders of thrombosis and hemostasis.

Who is most at risk for hemophilia? ›

Who Is at Risk? The Hemophilia Federation of America reports a family history in 70% of hemophilia cases, with inherited genes passed between parent and child. A mother who carries the gene has a 50% chance of having a son or daughter with hemophilia, making the daughter a carrier herself if inherited.

Is hemophilia an autoimmune disease? ›

Acquired hemophilia is an autoimmune condition in which there is sudden production of autoantibody inhibitors in an individual without any personal or family history of bleeding [1]. The body produces autoantibodies against factor VIII in hemophila A.

How does hemophilia affect everyday life? ›

The most frequent difficulties in everyday life for people living with hemophilia were indicated to be mobility problems, unexpected bleeding, pain, and uncertainties in daily activities impacting the choice of hobbies.

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