Haemophilia B

Haemophilia is a group of inherited blood disorders in which blood does not clot properly. Bleeding disorders are due to defect in blood vessels, the coagulation mechanism or the blood platelets. Blood coagulation mechanism is a process which transforms blood from a liquid into solid and involves different clotting factors that generates fibrin fibers through extrinsic or intrinsic coagulation pathway which along with platelet plug stops bleeding. When coagulation factors are deficient, the blood does not clot properly and bleeding continues. An affected individual may bleed spontaneously or for longer than a healthy person after injury or surgery. Haemophilia is a X-linked recessive disorder that affects males, and females are protected by normal gene on other X-chromosome. Queen Victoria was a carrier of haemophilia and passed the mutation to her son Leopold and through her daughters to members of Royal families of Spain, Russia and Germany. JMS 2016; 19(1):32-33