Diamond blackfan anemia steroid treatment

Diamond Blackfan Anemia (DBA) was first recognized as a distinct entity in 1938, although it was called “congenital hypoplastic anemia” at that time. Diamond Blackfan Anemia (“DBA”) is a rare inherited bone marrow failure syndrome, characterized by a failure of the bone marrow (the center of the bone where blood cells are made) to produce red blood cells. This failure causes DBA patients to become severely anemic. It is important to note that this anemia is not the result of a deficiency in iron, vitamin B-12, folate, or erythropoietin, which is a blood cell stimulating factor. Although a number of theories regarding the cause of DBA have been proposed, it is now widely accepted that DBA is a ribosomal protein disease. The disorder results from an intrinsic progenitor cell defect in which erythroid progenitors and precursors are highly sensitive to death by apoptosis (self-destruction).

A medical approach that is not currently part of standard practice. Complementary medicine is used along with standard medicine. Alternative medicine is used in place of standard medicine. Example of CAM therapies are acupuncture, chiropractic, homeopathic, and herbal medicines. There is no complementary or alternative therapy that effectively treats bone marrow failure. Some CAM therapies may even hinder the effectiveness of standard medical care. <strong>Patients should talk with their doctor if they are currently using or considering using a complementary or alternative therapy.</strong>

Blackfan-Diamond anemia is usually treated with adrenal corticosteroid drugs beginning as early as possible. Red blood cell transfusions may be used in conjunction with steroid treatments. Multiple blood transfusions can be associated with heart and liver problems, and excessive accumulations of iron in body tissues. Multiple transfusions (every 4-8 weeks) are ordered when the anemia is particularly severe and when the response to treatment with steroids is lee than expected. Infections must be carefully guarded against since they can cause worsening of the blood condition.

Diamond blackfan anemia steroid treatment

diamond blackfan anemia steroid treatment

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