hemophilia a vs b

Hemophilia a vs b

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Overview

Hemophilia is a serious, inherited disorder that causes the blood to not clot properly and often causes excessive bleeding for no reason or with minor injuries. Although a person with factor VIII deficiency may not bleed more or faster than normal, they bleed for a long time and continuous bleeding can cause significant damage, especially to their joints.

Being a hereditary disorder, hemophilia is passed from parent to child through the X chromosome, which is carried as an recessive trait. Usually this disease spreads from mother to child. Although women can also develop hemophilia, it is very rare. The majority of the factor VIII deficiency population worldwide is male

hemophilia

The most common type of hemophilia is hemophilia A.2 An estimated 320,000 people worldwide are affected by hemophilia A.3 More than 1,400 cases have been identified in Bangladesh4 and many more cases remain undetected. An essential protein known as factor VIII plays an important role in blood clotting. It is either not present in the body of people with factor VIII deficiency or is present in very low amounts.

When bleeding occurs in a normal healthy person, factor VIII binds to factor IXa and X and plays an important role in clotting and stopping bleeding. But, in people with factor VIII deficiency , the lack of factor VIII inhibits this process and reduces the blood’s ability to clot.

Based on the extent of factor VIII deficiency in the blood5, hemophilia A is divided into three categories – mild, moderate and severe. About 50-60 percent of people with factor VIII deficiency have severe factor VIII deficiency A6 and have frequent bleeding into the joints or muscles, usually once or twice a week7. Such bleeding can often cause significant physical problems such as pain and chronic swelling or disfigurement, loss of mobility, long-term joint damage, etc.8 Besides affecting a person’s quality of life, such bleeding can be life-threatening if it occurs in vital organs such as the brain. Maybe .10,11

Hemophilia a vs b

Symptoms and diagnosis: Due to its hereditary nature, most cases are diagnosed at a very young age. Signs and symptoms of factor VIII deficiency are as follows:

  • wound
  • Bleeding into muscles or joints
  • Bleeding for no reason
  • Prolonged bleeding after minor trauma or surgery12

Treatment

Recombinant (genetically engineered) or plasma-derived factor VIII replacement is the main treatment for factor VIII deficiency in Bangladesh as in other countries of the world. Most formulations typically require frequent intravenous infusions at least several times a week13, which can be inconvenient for patients or their caregivers. Because of this, patients often seek treatment ‘on-demand’ (when bleeding occurs) rather than prophylactic treatment. Preventive treatment can also prevent the patient from bleeding. 14-16

An additional limitation of current treatment is the development of specific antibodies ‘inhibitors’ made by the body’s immune system, which can identify and attack the replaced factor VIII as foreign and harmful to the body.17 The development of inhibitors in the patient’s blood can render factor VIII replacement therapy ineffective. . About one in four (25–30 percent) of people with severe hemophilia A experience this problem.18

hemophilia a vs b

Alternative treatments for patients with factor VIII deficiency due to inhibitor problems are very limited. Such patients may require more frequent infusions as well as immune tolerance induction (ITI), in which the patient is given large amounts of factor VIII over a longer period of time to allow the immune system to take up factor VIII. If ITI fails or the patient is unsuitable for ITI, treatment with ‘bypassing agents’ is given. Like factor VIII replacement, it is also administered intravenously to the patient but has to be given more frequently.19

Roche’s promise

For more than 20 years, Roche has been developing and supplying medicines for people with blood disorders. Today, we are working harder than ever to develop effective treatments for people with hemophilia A, including other blood disorders, and are committed to inventing and developing innovative approaches to hopefully treat such serious disorders. #hemophilia a vs b, #hemophilia a vs b, #hemophilia a vs b.

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