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Hemophilia AAka: Hemophilia, Factor VIII Deficiency

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  1. Epidemiology
    1. Most common hereditary coagulation disorder (1:10,000)
  2. Pathophysiology
    1. Sex linked recessive deficiency of factor VIII
  3. Symptoms
    1. Excessive bleeding from slight trauma
      1. Dental extraction
      2. Surgery
    2. Visceral bleeding
    3. Hemarthrosis
      1. Contractures
      2. Degenerative arthritis
  4. Labs
    1. Partial Thromboplastin Time (PTT) elevated
    2. ProTime normal
  5. Management: Factor VIII replacement
    1. Indications
      1. Acute Bleeding
      2. Before surgical procedure
    2. Dosing
      1. Mild Bleeding
        1. Give Factor VIII to obtain 15% Factor VIII level
      2. Severe Bleeding
        1. Give Factor VIII to obtain 50% Factor VIII level

Hemophilia A (C0019069)

Definition (MSH)The classic hemophilia resulting from a deficiency of factor VIII. It is an inherited disorder of blood coagulation characterized by a permanent tendency to hemorrhage.
Definition (CSP)classic hemophilia resulting from a deficiency of factor VIII; an inherited disorder of blood coagulation characterized by a permanent tendency to hemorrhage.
Definition (NCI)An inherited deficiency of coagulation Factor VIII characterized by the tendency to spontaneous or exaggerated post-traumatic hemorrhage. Inherited as an x-linked recessive disease, hemophilia A is the most common hemophilia, occurring in approximately 1 in 10,000 male births. --2004
ConceptsDisease or Syndrome (T047)
ICD9286.0, 286.0
MSHD006467
EnglishAHG DEFICIENCY, AHG deficiency disease, ANTIHEMOPHILIC FACTOR A DEFICIENCY, ANTIHEMOPHILIC GLOBULIN DEFICIENCY, AUTOSOMAL HEMOPHILIA A, Classical haemophilia, Classical hemophilia, CONG FACTOR VIII DIORD, Congenital factor VIII deficiency, Congenital factor VIII deficiency disease, Congenital factor VIII disorder, DEFIC FACTOR VIII, FACTOR VIII DEFIC, Factor VIII Deficiencies, Factor VIII Deficiency, Functional factor VIII deficiency, Haemophilia, Haemophilia A, Hemophilia, Hemophilia A, Hemophilia As, Hereditary factor VIII deficiency disease, Sex-linked factor VIII deficiency, Subhemophilia
Spanishdeficiencia de factor VIII, deficiencia hereditaria de factor VIII, deficiencia hereditaria de factor VIII ligada al sexo, enfermedad de deficiencia del factor congenito VIII, hemofilia A, hemofilia clasica
Parent ConceptsBlood Coagulation Disorders (C0005779), Hereditary Diseases (C0019247), Hemorrhagic Disorders (C0019087), Coagulation Protein Disorders (C0600503), Blood Coagulation Disorders, Inherited (C0852077), Hemophilia, NOS (C0684275), Hemophilia A (C0019069), Coagulation factor deficiency syndrome (C0272315), Hereditary coagulation factor deficiency (C0272316), Genetic Diseases, X-Linked (C1138434)
SourcesCSP, DXP, ICD9CM, MSH, MTH, MTHICD9, NCI, NDFRT, OMIM, QMR, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)



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