Hemophilia A history and symptoms
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Fahd Yunus, M.D. [2] Vahid Eidkhani, M.D.
Overview
Hemophilia leads to a severely increased risk of bleeding from common injuries. Mild hemophilia is usually asymptomatic, unless the patient experiences a significant injury or undergoes surgery. Moderate Hemophilia patients bleeding episodes occur during mild trauma and surgeries and spontaneous bleeding occurs in severe Hemophilia cases. Bleeding into large joints or muscles is the most common site of bleeding in hemophilia. Hemarthrosis is considered as the hallmark of Hemophilia. Though uncommon, spontaneous or traumatic intracranial hemorrhages are the most catastrophic complication of hemophilia[1][2].
History
A family history of abnormal bleeding is often present and highly suggestive of hemophilia A/ B, though it is not essential for diagnosis.
Acquired forms of Hemophilia usually occur in patients with an underlying diseases or conditions affecting the immune system. These include malignancies, pregnancy, autoimmune disorders, infections and drugs[3].
Symptoms
Mild hemophilia is usually asymptomatic, unless the patient experiences an injury or undergoes surgery. Young males often experience excessive bleeding with circumcision, or following dental procedures.[4] Hemophilia leads to a severely increased risk of bleeding from common injuries. The sites of bleeding are:
Other symptoms include:
- Bruising
- Excessive bleeding following circumcision
- Gastrointestinal tract and urinary tract hemorrhage
- Nosebleeds
- Prolonged bleeding from cuts, tooth extraction, and surgery
- Spontaneous bleeding
The muscle and joint hemorrhages are quite typical of hemophilia, while digestive tract and cerebral hemorrhages are also germane to other coagulation disorders. Bleeding into large joints or muscles is the most common site of bleeding in hemophilia.[5] Most common muscle bleeds include the muscles of the upper arm and forearm, the iliopsoas muscle, the thigh and the calf. Most joint bleeds affect the knees, ankles or elbows. Patients may present with pain in these areas, or they may simply present with incidental bruising or swelling. Repeated episodes of bleeds into joints may result in arthritis, and eventual destruction of cartilage in joint space. Though uncommon, spontaneous or traumatic intracranial hemorrhages are the most catastrophic complication of hemophilia. Patients with intracranial hemorrhages present with typical symptoms, including nausea, headache, vomiting, and altered mental status. This is a medical emergency.
References
- ↑ Zimmerman B, Valentino LA (2013). "Hemophilia: in review". Pediatr Rev. 34 (7): 289–94, quiz 295. doi:10.1542/pir.34-7-289. PMID 23818083.
- ↑ Bolton-Maggs PH, Pasi KJ (2003). "Haemophilias A and B." Lancet. 361 (9371): 1801–9. doi:10.1016/S0140-6736(03)13405-8. PMID 12781551.
- ↑ Knoebl P, Marco P, Baudo F, Collins P, Huth-Kühne A, Nemes L; et al. (2012). "Demographic and clinical data in acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2)". J Thromb Haemost. 10 (4): 622–31. doi:10.1111/j.1538-7836.2012.04654.x. PMID 22321904.
- ↑ Types of Bleeds | National Hemophilia Foundation. Available at https://www.hemophilia.org/Bleeding-Disorders/Types-of-Bleeds . Accessed on Sept 20, 2016
- ↑ What are the signs and symptoms of Hemophilia? – NHLBI, NIH. Available at http://www.nhlbi.nih.gov/health/health-topics/topics/hemophilia/signs. Accessed on Sept 20, 2016