A 3 year old male child was brought to a
doctor with complaints of bleeding from nose, urinary tract, alimentary tract
and skin after trivial injury. History of pain and swelling of joints
associated with fever was also found. Bleeding was persistent but not profuse.
A careful history revealed similar bleeding tendencies among male relatives. On
Lab investigation coagulation time was prolonged and the bleeding time was normal.
Q1. What is the most likely diagnosis?
Q2. What are the possibilities of boy’s sister being
affected?
Q3. What is the physiological basis for the treatment of
this disorder?
A1. It is Haemophilia.
A2. Boy’s sister will be a carrier.
A3. Fresh blood transfusion. (Factors VIII is lost on storage). Injection of factor VIII & IX prepared from fresh frozen plasma or cryoprecipitate.