IJSRST162422 | Received : 22 July 2016 | Accepted : 02 August 2016 | July-August 2016 [(2)4: 155-161 ]
© 2016 IJSRST | Volume 2 | Issue 4 | Print ISSN: 2395-6011 | Online ISSN: 2395-602X
Themed Section: Science and Technology
155
Treatment of Haemophilia A by Replacement Therapy using
Factor VIII Inhibitors
Maria Shahid, Sikander Ali
Institute of Industrial Biotechnology (IIB), GC University Lahore, Pakistan
ABSTRACT
Haemophilia A is a very severe disease which is caused due to deficiency of a protein clotting factor VIII.
Haemophilia A is X-linked recessive disorder which can be treated by the replacement therapy or by development of
an inhibitors or antibodies against the FVIII. Hemarthroses is a disease in which bleeding in the joint spaces occur.
This disease occurs approximately 70 to 80% in haemophilic patients. Then here we review about the epidimicity
that deals with the transformation of haemophilia A. Then the process of inhibition stated, in which under the
influence of some chemical substances the specific chemical process may inhibit. So, different replacement therapy
may be ineffective when an inhibitor affect the factor Vǀǀǀ gene. In the end here stated different treatments for the
management & control of disease, so among them the counselling between physician & patient is very effective.
Keywords : Hemarthroses, Haemophilia, Epidimicity, Epidemiology, DNA, AVD, IgG, TCR, Arthropathy,
Prophylaxis
I. INTRODUCTION
There are many different types of bleeding disorders in
which haemophilia A is known as a result of the
deficiency of the protein clotting factor VIII and is
genetically severe bleeding disorder. The deficiency of
factor VIII in 1 in every 5000 male births without racial
majority as X-linked recessive abnormality (Soucie et
al., 1998). Haemophilia A, as the most common disorder
in patients, is intra-articular hemorrhages. The cure of
haemophilia started from the last 40 years. In history,
patients of haemophilia, including adults and children,
had no ability of survival but now days this disease can
be controlled and helps the patients to do their work
activities in efficient way (DiMichele & Neufeld, 1998).
Proper treatment of these patients requires deeply
understanding of AHA path physiology by
anaesthesiologists.
There are different therapeutic treatments for other
bleeding disorders so that haemophilia A also cured by
specific medication and blood products. At present the
best treatment of haemophilia A is to replace the factor
VIII by using the plasma or recombinant FVIII in order
to stable its homeostasis. When a patient is develop an
inhibitor against the factor VIII then the replacement
therapy of haemophilia A becomes hopeless. Now a
day’s development of the inhibitor is the most important
treatment of haemophilic patient (Fig. 1). Although there
is decrease in death rate due to improvement in
homeostatic agent but still there is another complication
that is high rate of bleeding (Gringeri et al., 2003; Darby
et al., 2004; Morfini et al., 2007; Brown et al., 2009; Di
Minno et al., 2010).
Figure 1. Difference between a normal and haemophilic
blood vessel