Hemophilia differential diagnosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Simrat Sarai, M.D. [2]

Overview

Hemophilia must be differentiated from other diseases that lead to spontaneous bleeding and bleeding following injuries or surgery such as von Willebrand disease, liver failure-early or end stage, thrombocytopenia, vitamin K deficiency, disseminated intravascular coagulation, uremia, congenital afibrinogenemia, factor V deficiency, factor X deficiency as seen in amyloid purpura, glanzmann's thrombasthenia, bernard-soulier syndrome, factor XII deficiency and C1INH deficiency.

Differentiating Hemophilia with other Diseases

Hemophilia A can be mimicked by von Willebrand Disease

• Von Willebrand Disease type 2A, where decreased levels of von Willebrand Factor can lead to premature proteolysis of Factor VIII. In contrast to haemophilia, vWD type 2A is inherited in an autosomal dominant fashion.
• Von Willebrand Disease type 2N, where von Willebrand factor cannot bind Factor VIII
• Von Willebrand Disease type 3, where lack of von Willebrand factor causes premature proteolysis of Factor VIII. In contrast to haemophilia, vWD type 3 is inherited in an autosomal recessive fashion.

Additionally, severe cases of vitamin K deficiency can present similar symptoms to hemophilia. This is because vitamin K is necessary for the human body to produce several protein clotting factors. This vitamin deficiency is rare in adults and older children but is common in newborns. Infants are born with naturally low levels of vitamin K and do not yet have the symbiotic gut flora to properly synthesise their own vitamin K. Bleeding issues due to vitamin K deficiency in infants is known as "haemorrhagic disease of the newborn", to avoid this complication newborns are routinely injected with vitamin K supplements.

• The other conditions to be considered includes the following:
  • Lupus Anticoagulant
  • Heparin Administration
  • Disseminated Intravascular Coagulation
  • Dysfibrinogenemia

| style="border: 0px; font-size: 90%; margin: 3px; width: 500px;" align=center |valign=top| |+ ! style="background: #4479BA; width: 200px;" | Condition ! style="background: #4479BA; width: 200px;" | Prothrombin time ! style="background: #4479BA; width: 200px;" | Partial Thromboplastin Time ! style="background: #4479BA; width: 200px;" | Bleeding Time ! style="background: #4479BA; width: 200px;" | Platelet Count |- | style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" | Heamophilia A or B | style="padding: 5px 5px; background: #F5F5F5;" |Unaffected | style="padding: 5px 5px; background: #F5F5F5;" |Prolonged | style="padding: 5px 5px; background: #F5F5F5;" |Unaffected | style="padding: 5px 5px; background: #F5F5F5;" |Unaffected

|- | style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" | Von Willebrand Disease | style="padding: 5px 5px; background: #F5F5F5;" |Unaffected | style="padding: 5px 5px; background: #F5F5F5;" |Prolonged or Unaffected | style="padding: 5px 5px; background: #F5F5F5;" |Prolonged | style="padding: 5px 5px; background: #F5F5F5;" |Unaffected |- | style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" | Thrombocytopenia | style="padding: 5px 5px; background: #F5F5F5;" |Unaffected | style="padding: 5px 5px; background: #F5F5F5;" |Unaffected | style="padding: 5px 5px; background: #F5F5F5;" |Prolonged | style="padding: 5px 5px; background: #F5F5F5;" |Decreased |- | style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" | Vitamin K deficiency or Warfarin | style="padding: 5px 5px; background: #F5F5F5;" |Prolonged | style="padding: 5px 5px; background: #F5F5F5;" |Normal or mildly prolonged | style="padding: 5px 5px; background: #F5F5F5;" |Unaffected | style="padding: 5px 5px; background: #F5F5F5;" |Unaffected |- | style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" | Disseminated Intravascular Coagulation | style="padding: 5px 5px; background: #F5F5F5;" |Prolonged | style="padding: 5px 5px; background: #F5F5F5;" |Prolonged | style="padding: 5px 5px; background: #F5F5F5;" |Prolonged | style="padding: 5px 5px; background: #F5F5F5;" |Decreased |- | style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" | Liver failure, early | style="padding: 5px 5px; background: #F5F5F5;" |Prolonged | style="padding: 5px 5px; background: #F5F5F5;" |Unaffected | style="padding: 5px 5px; background: #F5F5F5;" |Unaffected | style="padding: 5px 5px; background: #F5F5F5;" |Unaffected |- | style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" | Aspirin | style="padding: 5px 5px; background: #F5F5F5;" |Unaffected | style="padding: 5px 5px; background: #F5F5F5;" |Unaffected | style="padding: 5px 5px; background: #F5F5F5;" |Prolonged | style="padding: 5px 5px; background: #F5F5F5;" |Unaffected |- | style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" | Liver failure, end-stage | style="padding: 5px 5px; background: #F5F5F5;" |Prolonged | style="padding: 5px 5px; background: #F5F5F5;" |Pronlonged | style="padding: 5px 5px; background: #F5F5F5;" |Prolonged | style="padding: 5px 5px; background: #F5F5F5;" |Decreased |- | style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" | Uremia | style="padding: 5px 5px; background: #F5F5F5;" |Unaffected | style="padding: 5px 5px; background: #F5F5F5;" |Unaffected | style="padding: 5px 5px; background: #F5F5F5;" |Prolonged | style="padding: 5px 5px; background: #F5F5F5;" |Unaffected |- | style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" | Congenital afibrinogenemia | style="padding: 5px 5px; background: #F5F5F5;" |Prolonged | style="padding: 5px 5px; background: #F5F5F5;" |Prolonged | style="padding: 5px 5px; background: #F5F5F5;" |Prolonged | style="padding: 5px 5px; background: #F5F5F5;" |Unaffected |- | style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" | Factor V deficiency | style="padding: 5px 5px; background: #F5F5F5;" |Prolonged | style="padding: 5px 5px; background: #F5F5F5;" |Prolonged | style="padding: 5px 5px; background: #F5F5F5;" |Unaffected | style="padding: 5px 5px; background: #F5F5F5;" |Unaffected |- | style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" | Factor X deficiency as seen in amyloid purpura | style="padding: 5px 5px; background: #F5F5F5;" |Prolonged | style="padding: 5px 5px; background: #F5F5F5;" |Prolonged | style="padding: 5px 5px; background: #F5F5F5;" |Unaffected | style="padding: 5px 5px; background: #F5F5F5;" |Unaffected |- | style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" | Glanzmann's thrombasthenia | style="padding: 5px 5px; background: #F5F5F5;" |Unaffected | style="padding: 5px 5px; background: #F5F5F5;" |Unaffected | style="padding: 5px 5px; background: #F5F5F5;" |Prolonged | style="padding: 5px 5px; background: #F5F5F5;" |Unaffected |- | style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" | Bernard-Soulier syndrome | style="padding: 5px 5px; background: #F5F5F5;" |Unaffected | style="padding: 5px 5px; background: #F5F5F5;" |Unaffected | style="padding: 5px 5px; background: #F5F5F5;" |Prolonged | style="padding: 5px 5px; background: #F5F5F5;" |Decreased |- | style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" | Factor XII deficiency | style="padding: 5px 5px; background: #F5F5F5;" |Unaffected | style="padding: 5px 5px; background: #F5F5F5;" |Unaffected | style="padding: 5px 5px; background: #F5F5F5;" |Prolonged | style="padding: 5px 5px; background: #F5F5F5;" |Decreased or unaffected |- | style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" | C1INH deficiency | style="padding: 5px 5px; background: #F5F5F5;" |Unaffected | style="padding: 5px 5px; background: #F5F5F5;" |Shortened | style="padding: 5px 5px; background: #F5F5F5;" |Unaffected | style="padding: 5px 5px; background: #F5F5F5;" |Unaffected |- | style="padding: 5px 5px; background: #F5F5F5;" colspan=5| Adapted from Wikipedia hemophilia Laboratory Finding> "Wikipedia Hemophilia Laboratory Finding". |}