HEAMOPHILIA Flashcards
What is haemophilia?
A genetic bleeding disorder caused by a deficiency of clotting factors.
Which clotting factor is deficient in haemophilia A?
Factor VIII.
Which clotting factor is deficient in haemophilia B?
Factor IX.
What is the inheritance pattern of haemophilia?
X-linked recessive.
What are the symptoms of haemophilia?
Prolonged bleeding, spontaneous bleeding into joints (hemarthrosis), muscles, and internal organs.
How is haemophilia diagnosed?
Prolonged activated partial thromboplastin time (aPTT), normal PT, and specific factor assays.
What is the treatment for haemophilia A?
Replacement therapy with Factor VIII concentrates.
What is the treatment for haemophilia B?
Replacement therapy with Factor IX concentrates.
What is the role of desmopressin (DDAVP) in haemophilia?
Used in mild haemophilia A to increase Factor VIII levels.
What are inhibitors in haemophilia?
Antibodies that neutralize clotting factor concentrates, complicating treatment.
What is the role of gene therapy in haemophilia?
A potential curative approach to correct the genetic defect.
What is the difference between mild
moderate
What is acquired haemophilia?
A rare autoimmune disorder where the body produces antibodies against clotting factors.
What are the complications of untreated haemophilia?
Chronic joint damage (hemarthropathy), anemia, and life-threatening bleeding.
What is the importance of prophylactic therapy in haemophilia?
Prevents spontaneous bleeding episodes and long-term complications like joint damage.
