Hemophilia

From Anvita Health Wiki

Contents 1 More Specific Terms 2 Classification 3 Genetics 4 Clinical-manifestations 5 Laboratory 6 Differential-diagnosis 7 Management 8 More General Terms 9 References

More Specific Terms

• coagulation factor VII deficiency
• factor XI deficiency; plasma thromboplastin antecedent deficiency; Rosenthal syndrome
• hemophilia A; factor VIII deficiency
• hemophilia B; factor IX deficiency ; Christmas disease
• multiple coagulation factor deficiency 1 (MCFD1)

Classification

• coagulation factor VIII deficiency ( hemophilia A)*
• coagulation factor IX deficiency ( hemophilia B)
• coagulation factor XI deficiency
• multiple coagulation factor deficiency

• * hemophilia A is 10X more common than hemophilia B

Genetics

• family history of spontanous or unexpected bleeding

Clinical-manifestations

• spontanous or unexpected bleeding

Laboratory

• prothrombin time (PT) is normal; INR is normal
• partial thromboplastin time ( PTT) is prolonged
• confirm coagulation factor deficiency by a mixing study with normal plasma;

• factor deficiency will correct with normal plasma
• factor inhibitor will inhibit factor in normal plasma as well & will not correct

Differential-diagnosis

• von Willebrand's disease

• coagulation factor VIII deficiency without family history

Management

• blood transfusions & coagulation factor replacement for life-threateing bleeding

More General Terms

• coagulation factor deficiency

References

1. Medical Knowledge Self Assessment Program (MKSAP) 14, American College of Physicians, Philadelphia 2006
2. Hemophilia [1]

hemophilia