- Unlike congenital hemophilia, acquired hemophilia is curable.
- It is characterized because it causes such abundant hemorrhages that it can have lethal consequences in patients.
- It affects two out of every million people worldwide, mainly adults over 70 years of age and women during pregnancy or in the postpartum period.
Rare diseases remain poorly researched health problems. Because its incidence is too low, not enough work has been done to learn more about its impact. For this reason, there are few treatments available and in the end the most affected are the patients. Within the list stands out the acquired hemophilia because the majority of the population does not know what it consists of or its effects.
What are rare diseases?
According to the World Health Organization (WHO) are those that occur in less than five people per 10,000 inhabitants. But although it seems like a minor and insignificant problem, in total it is estimated that there are 350 million people with a problem of this type.
With respect to Mexico there is no official statistics. The truth is that it is estimated that there could be between four million 550 thousand and up to more than seven million 670 thousand patients with at least one of the known rare diseases. While only five percent of these types of conditions have an approved treatment option. Hence the importance of any action in favor of patients and their families, as well as doctors and health systems.
About acquired hemophilia, it is a rare disease that it affects only two out of every million people globally. It is characterized because it causes such abundant hemorrhages that it can have lethal consequences in patients. It mainly affects adults over 70 years of age and women during pregnancy or in the postpartum period.
Difference Between Congenital and Acquired
Dr. Angélica Licona, Medical Manager of Rare Diseases for Novo Nordisk Mexico, explains that congenital hemophilia is an inherited genetic coagulation disorder, the result of which is the little or no ability of the body to produce factor VIII (hemophilia A). or factor IX (hemophilia B), which are blood proteins that control bleeding
“The lack of these factors causes the body’s coagulation chain to be incomplete, which leads to bleeding episodes, mainly in joints.”
In order to know how to identify acquired hemophilia, it is necessary to know its differences with congenital hemophilia. Unlike congenital or hereditary hemophilia, acquired hemophilia is an autoimmune disease, where a person with a previously healthy coagulation system develops antibodies or inhibitors against one or more coagulation factors.
The most frequently damaged coagulation factor in acquired hemophilia is Factor VIII, and this decrease in factor concentration results in a significant bleeding tendency.
The bleeding pattern seen in acquired hemophilia is very different from that seen in the congenital form. Joint hemorrhages (hemarthroses) that occur in severe congenital hemophilia are not common in acquired hemophilia, in which the main manifestations are skin hemorrhages (extensive bruises or ecchymoses) and bruises in soft tissues (muscles and organs), the which are usually very intense.
Other manifestations of acquired hemophilia can be gastrointestinal bleeding and prolonged postpartum hemorrhage.
“The cause of inhibitor development is unknown, but there are often other related medical conditions, such as autoimmune disorders, including rheumatoid arthritis, ulcerative colitis, or psoriasis; malignant diseases such as solid tumors, leukemias, lymphomas or multiple myeloma; the use of certain medications and pregnancy. People with acquired hemophilia often go to hospital for the first time because of spontaneous and usually severe bleeding that is difficult to control.”
Curable if detected early
Acquired hemophilia is a really serious health problem, since 94.6% of patients have sudden bleeding and 70.3% have severe bleeding. Dr. Licona warned that in Mexico and the world there are more and more cases of acquired hemophilia, with a mortality rate of up to 28% due to late diagnosis.
“This high mortality rate is associated with the lack of information from doctors to identify the disease. The important thing is that both doctors and the general public learn to identify it and know that there is a way to treat it”.
The treatment of acquired hemophilia includes: eradicating the presence of the antibody or inhibitor and stopping the bleeding, where the use of bridging agents or “by pass” agents is recommended, among which recombinant activated Factor VII stands out for control bleeding episodes.