Anatomy & Physiology: Current Research
Open Access

Impact of Acquired Hemophilia in Adults

Swetha Kella^{* *}Correspondence: Swetha Kella, Department of Hematology, Newark Beth Israel Medical Center, Newark, United States, Email:

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Description

Acquired Haemophilia A (AHA) is a rare disease resulting from autoantibodies (inhibitors) against endogenous factor VIII (FVIII) that leads to bleeding, which is often spontaneous and severe. Elderly people with illnesses are more likely to develop AHA, which is linked to a high mortality risk due to underlying comorbidities, bleeding, or treatment problems. Treatment, which involves controlling the hemostasis and getting rid of the inhibitors, can be difficult to provide. There is a lack of information to help in managing AHA-related bleeding and eliminating the disease-causing antibodies. An international team of specialists in the AHA, supported by the Hemostasis and Thrombosis Research Society of North America, examined significant issues, studied the literature, considered the evidence, and came to an agreement to update current recommendations [1]. The likelihood of under diagnosis and misdiagnosis of AHA real-world clinical practice. Recommendations for the management of AHA are summarized here based on the available data, integrated with the clinical experience of panel participants.

Hemophiliac new-born are at risk for extra cranial and cerebral bleeding, as well as other problems. An intricate process that can start even before conception and lasts throughout pregnancy, birth and the newborn stage is required for the safe delivery of a healthy newborn with hemophilia. The expectant parents and a wide range of medical specialists are involved in this procedure, including genetic counselors, obstetricians, neonatologists, pediatricians, radiologists, adult and pediatric hematologists and nurses with hemophilia-specific training [2]. There is a lot of variance in practice in this field as a result of the multidisciplinary complexity, the relative rarity of neonates born with hemophilia and the dearth of high-quality evidence to guide.

Retrospective analysis of electronic medical records of pregnant patients at our HTC from January 2007 to October 2021 who had mild hemophilia A or B (Factor VIII (FVIII) or Factor IX [FIX] level 0.4 IU/mL) or were carriers of hemophilia A or B. Demographic information, the cause of the disease, baseline and third trimester FVIII and FIX levels, the bleeding phenotype, and genotype were gathered [3]. A record was kept of the conception method, factor replacement, iron supplements, birth method, an aesthetic type, per partum problems, and child outcomes. A total of 12 women experienced 18 pregnancies (2 with mild hemophilia A, 2 mild hemophilia B, 6 hemophilia A carriers, and 2 hemophilia B carriers). Seven pregnancies (39%) were born using in-vitro fertilization, while eleven (61%) were born spontaneously (IVF). Eight (44.4%) Retrospective analysis of electronic medical records of pregnant patients at our HTC from 2007-2021 who had mild hemophilia A or B (Factor VIII (FVIII) or Factor IX (FIX) level 0.4 IU/mL) or were carriers of hemophilia A or B. Demographic information, the cause of the disease, baseline and third trimester FVIII and FIX levels, the bleeding phenotype, and genotype were gathered. A record was kept of the conception method, factor replacement, iron supplements, birth method, an aesthetic type, per partum problems, and child outcomes [4]. Severe hemophilia A patients are often identified during the first two years of life after oral or soft tissue bleeding, either as a result of operations or because of a known family history of the disease. Without preventive care, people may experience up to two to five incidents of spontaneous bleeding each month, including deep-muscle hematomas, joint bleeds, persistent bleeding, and excruciating pain and swelling from small cuts, procedures, and teeth extractions.

Although it varies from person to person, people with moderate hemophilia A rarely experience spontaneous bleeding; however, they do experience prolonged or delayed bleeding after relatively minor trauma. They are typically diagnosed before the age of five to six years; the frequency of bleeding episodes varies, typically from once a month to once a year. People with moderate hemophilia A do not experience spontaneous bleeding episodes, although abnormal bleeding does happen with surgery or teeth extractions without pre and postoperative care; the frequency of bleeding episodes varies greatly, ranging from once a year to once every ten years. Mild hemophilia A patients sometimes don't get diagnosed until much later in life. 30% of heterozygous females are at risk for bleeding because their factor VIII clotting activity is below 40%. Even if males in the family are only mildly affected. No matter how severe the injury, there will typically be protracted or profuse bleeding after significant trauma or invasive operations. Additionally, 25% of heterozygous females report an increased propensity to bleed even with normal factor VIII clotting activity.

Diagnosis/testing

When a person has normal levels of functional von Wille brand factor and poor factor VIII clotting activity, the diagnosis of hemophilia A is made. The diagnosis is confirmed by the discovery of a homozygous F8 pathogenic mutation in a male proband during molecular genetic testing. The diagnosis is confirmed by the discovery of a heterozygous F8 pathogenic mutation during molecular genetic testing in a symptomatic female.

Treatment for symptoms

Referral to a Hemophilia Treatment Centre (HTC) to facilitate treatment (intravenous infusion of factor VIII concentrate is most effective when infused within one hour of bleeding onset); instruction to facilitate infusions given at home by parents or affected people; immune tolerance therapy. Decompression acetate or factor VIII concentrate should be administered intravenously or topically to patients who have bleeding that has to be treated right away for mild illness [5].

Primary manifestation prevention involves either subcutaneous injection of emicizumab or prophylactic factor VIII concentrate infusions for those with moderate to severe hemophilia A who bleed often. For those with severe or moderate hemophilia A, assessments at an HTC with inhibitor screening are performed every six to twelve months; for those with mild hemophilia A, assessments are performed every one to two years. Additional visits can be necessary due to comorbidities.

Conclusion

Hemophilia A is characterized by a lack of factor VIII clotting activity, which leads to delayed or repeated bleeding before full wound healing and extended leaking following injuries, teeth extractions, or surgery. The degree of factor VIII clotting activity is correlated with the age of diagnosis and the frequency of major bleeding.

References

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