ICD10 code of Blackfan Diamond Anemia DBA and ICD9 code

Diamond-Blackfan Anemia (DBA) is officially classified under the ICD-10 code D61.02 (Diamond-Blackfan anemia). In the older ICD-9-CM classification system, Diamond-Blackfan Anemia was coded as 284.01.

What is Diamond-Blackfan Anemia?

Diamond-Blackfan Anemia is a rare, inherited bone marrow failure syndrome characterized by the failure of the bone marrow to produce red blood cells. Most patients with Diamond-Blackfan Anemia are diagnosed within the first year of life, typically presenting with severe macrocytic anemia, low reticulocyte counts, and a deficiency of red cell precursors in the bone marrow. At DiseaseMaps.org, we currently have 8 members living with Diamond-Blackfan Anemia who share their unique experiences with this condition.

Is Diamond-Blackfan Anemia hereditary?

Yes, Diamond-Blackfan Anemia is a genetic disorder, most commonly inherited in an autosomal dominant pattern. Approximately 45% to 50% of cases are caused by mutations in genes encoding ribosomal proteins (such as RPS19). Because the clinical presentation of Diamond-Blackfan Anemia can be highly variable even within the same family, genetic counseling is strongly recommended for families affected by the condition.

How is Diamond-Blackfan Anemia managed?

Treatment for Diamond-Blackfan Anemia is tailored to the individual's specific needs and may involve several approaches, including:

• Corticosteroids: The first-line therapy for many patients to stimulate red blood cell production.


• Chronic Blood Transfusions: Necessary for patients who do not respond to or cannot tolerate steroids.


• Iron Chelation Therapy: Essential for managing iron overload resulting from frequent transfusions.


• Hematopoietic Stem Cell Transplantation: Currently the only curative option for Diamond-Blackfan Anemia.

Next steps

• Consult a pediatric hematologist-oncologist specializing in bone marrow failure syndromes.


• Consider genetic testing to confirm the specific ribosomal protein mutation associated with your diagnosis.


• Connect with the 8 members on DiseaseMaps.org to share support and practical disease-management tips.


• Monitor for long-term complications, including endocrine issues and secondary malignancies, through regular follow-ups.

Medical disclaimer: This information is for educational purposes only and does not replace professional medical advice, diagnosis, or treatment from a qualified healthcare provider.

References

• NIH Genetic and Rare Diseases Information Center (GARD): Diamond-Blackfan anemia.


• Orphanet: Diamond-Blackfan anemia (ORPHA:120).


• Online Mendelian Inheritance in Man (OMIM): Diamond-Blackfan Anemia 1 (DBA1).


• Diamond Blackfan Anemia Foundation (DBAF): Patient support and clinical resources.