2006 ICD-9-CM Volume 1 Diagnosis Codes Home > Diseases Of Blood And Blood-Forming Organs 280-289 > Aplastic Anemia 284.* > 2006 ICD-9-CM Diagnosis 284.0 Constitutional aplastic anemia View the most recent version of ICD-9-CM 284.0 Suppression of erythropoiesis with little or no abnormality of leukocyte or platelet production. An inborn condition characterized by deficiencies of red cell precursors that sometimes also includes LEUKOPENIA and THROMBOCYTOPENIA. 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 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. 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. 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. 284.0 is a specific code that can be used to specify a diagnosis 284.0 contains 52 index entries View the ICD-9-CM Volume 1 284.* hierarchy Alternate Terminology Aplasia, (pure) red cell: congenital of infants primary Blackfan-Diamond syndrome Familial hypoplastic anemia Fanconi's anemia Pancytopenia with malformations