Hemophilia
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Hemophilia
Hematological system
Anemias
Iron deficiency anemia
Beta-thalassemia
Alpha-thalassemia
Sideroblastic anemia
Anemia of chronic disease
Lead poisoning
Hemolytic disease of the newborn
Glucose-6-phosphate dehydrogenase (G6PD) deficiency
Autoimmune hemolytic anemia
Pyruvate kinase deficiency
Paroxysmal nocturnal hemoglobinuria
Sickle cell disease (NORD)
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Aplastic anemia
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Megaloblastic anemia
Folate (Vitamin B9) deficiency
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Fanconi anemia
Diamond-Blackfan anemia
Heme synthesis disorders
Acute intermittent porphyria
Porphyria cutanea tarda
Lead poisoning
Coagulation disorders
Hemophilia
Vitamin K deficiency
Platelet disorders
Bernard-Soulier syndrome
Glanzmann's thrombasthenia
Hemolytic-uremic syndrome
Immune thrombocytopenic purpura
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Mixed platelet and coagulation disorders
Von Willebrand disease
Disseminated intravascular coagulation
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Thrombosis syndromes (hypercoagulability)
Antithrombin III deficiency
Factor V Leiden
Protein C deficiency
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Antiphospholipid syndrome
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Leukemias
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Leukemoid reaction
Leukemoid reaction
Dysplastic and proliferative disorders
Myelodysplastic syndromes
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Essential thrombocythemia (NORD)
Langerhans cell histiocytosis
Mastocytosis (NORD)
Plasma cell dyscrasias
Multiple myeloma
Monoclonal gammopathy of undetermined significance
Waldenstrom macroglobulinemia
Hematological system pathology review
Microcytic anemia: Pathology review
Non-hemolytic normocytic anemia: Pathology review
Intrinsic hemolytic normocytic anemia: Pathology review
Extrinsic hemolytic normocytic anemia: Pathology review
Macrocytic anemia: Pathology review
Heme synthesis disorders: Pathology review
Coagulation disorders: Pathology review
Platelet disorders: Pathology review
Mixed platelet and coagulation disorders: Pathology review
Thrombosis syndromes (hypercoagulability): Pathology review
Lymphomas: Pathology review
Leukemias: Pathology review
Plasma cell disorders: Pathology review
Myeloproliferative disorders: Pathology review
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Hemophilia
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Hemophilia
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Content Reviewers
Rishi Desai, MD, MPHContributors
Tanner Marshall, MS
The word “hemophilia” is a combination of the Greek words for “blood” and “love”, a way of saying that people with hemophilia “love to bleed”, or rather that it’s hard to stop bleeding. This is because the process called hemostasis, literally meaning to stop the flow of blood, is impaired.
Normally, after a cut and damage to the endothelium, or the inner lining of blood vessel walls, there’s an immediate vasoconstriction or narrowing of the blood vessel which limits the amount of blood flow. After that some platelets adhere to the damaged vessel wall, and become activated and then recruit additional platelets to form a plug. The formation of the platelet plug is called primary hemostasis.
After that, the coagulation cascade is activated. First off in the blood there’s a set of clotting factors—most of which are proteins synthesized by the liver, and usually these are inactive and just floating around the blood. The coagulation cascade starts when one of these proteins gets proteolytically cleaved. This active protein then proteolytically cleaves and activates the next clotting factor, and so on. This cascade has a great degree of amplification and takes only a few minutes from injury to clot formation. The final step is activation of the protein fibrinogen to fibrin, which deposits and polymerizes to form a mesh around the platelets. So these steps leading up to fibrin reinforcement of the platelet plug make up the process called secondary hemostasis and results in a hard clot at the site of the injury.
In most cases of hemophilia there is a decrease in the amount or function of one or more of the clotting factors which makes secondary hemostasis less effective and allows more bleeding to happen.
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