The Haemophilia Society
Question 1. Discuss in brief hemophilia.
Or
Write short note on hemophilia.
Answer:
“Understanding hemophilia: Causes and symptoms”
Hemophilia is a coagulation disorder.
- It occurs due to defiiency of coagulation factors.
- Types of hemophilia:
- Christmas disease or hemophilia B: Inherited deficiency of factor 9. (Christmas Factor or plasma thromboplastin factor) produces Christmas disease or hemophilia B. Hemophilia B is rare than hemophilia A.
What is hemophilia
“Impact of Factor IX deficiency in hemophilia B”
Question 2. Write a short note on Rh factor.
Answer:
Rh factor
- Rh factor is an antigen present in the RBC. The antigen was found in Rhesus monkey, so it was named as Rh factor.
- Rh antigen system has three closely linked gene loci, coding for D antigen (there is no d antigen), C and/or c antigen and E and/or e antigen. Thus, the antigens produced are C, D, E, c and e.
- An individual may have similar or diffrent sets of these three Rh antigens on each chromosome; for example, CDE/cde, cde/cde, or CdE/cdE (each person inherits one trio gene from each parent).
Hemophilia A vs B
“Techniques for managing hemophilia symptoms”
- Individuals who are positive for D antigen are considered Rh-positive (85% of the population) and those who lack it are Rh-negative.
- Individuals with a weak variant of D antigen, called the Du variant, are also considered Rh-positive.
- Alloimmunization, i.e. formation of an antibody against an antigen occurs if a person is exposed to an Rh antigen that is not on the patients RBCs.
- The majority of clinically important antibodies that produce a transfusion reaction are warm-reacting (IgG) antibodies (For Example, anti-D, anti- Kell) rather than cold-reacting (IgM) antibodies.
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