2008 ICD-9-CM Volume 1 Diagnosis Codes Home > Diseases Of The Blood And Blood-Forming Organs 280-289 > Aplastic Anemia 284.* >  2008 ICD-9-CM Diagnosis 284.0
Constitutional aplastic anemia- A rare and often fatal inherited disease in which the bone marrow fails to produce red blood cells, white blood cells, platelets, or a combination of these cells. The disease may transform into myelodysplastic syndrome or leukemia.
- A rare congenital hypoplastic anemia that usually presents early in infancy. The disease is characterized by a moderate to severe macrocytic anemia, occasional neutropenia or thrombocytosis, a normocellular bone marrow with erythroid hypoplasia, and an increased risk of developing leukemia. (Curr Opin Hematol 2000 Mar;7(2):85-94)
- An inborn condition characterized by deficiencies of red cell precursors that sometimes also includes LEUKOPENIA and THROMBOCYTOPENIA.
- Congenital disorder affecting all bone marrow elements, resulting in anemia, leukopenia, and thrombopenia, and associated with cardiac, renal, and limb malformations as well as dermal pigmentary changes. Spontaneous chromosome breakage is a feature of this disease along with predisposition to leukemia. There are at least 7 complementation groups in Fanconi anemia: FANCA, FANCB, FANCC, FANCD, FANCE, FANCF, FANCG. (from Online Mendelian Inheritance in Man, http://www.ncbi.nlm.nih.gov/Omim, MIM# 227650, April 23, 2001)
- Fanconi anemia (FA) is an autosomal recessive genetic disorder characterised clinically by progressive bone marrow failure, skeletal deformities and a predisposition to neoplasia. Patient cells manifest an extreme chromosomal instability and hypersensitivity to polyfunctional alkylating agents. It is assumed that the basic defect is related to the repair of DNA damage, in particular that of so-called DNA crosslinks. Currently there are eight complementation groups in FA (FA-A-FA-H) which indicates that at least eight independent genes can lead to FA. Three of these genes have been identified: FANCA, FANCC and FANCG. (from PMID 10472548)
- Suppression of erythropoiesis with little or no abnormality of leukocyte or platelet production.
- 284.0 is a non-specific code that cannot be used to specify a diagnosis
- View the ICD-9-CM Volume 1 284.* hierarchy
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