Refractory anemia

Name
Refractory anemia
ICD-O-3 Morphology
9980/3
Effective 2001 and later
Reportable
for cases diagnosed 2001 and later
Primary Site(s)
C421

Help me code for dx year :

Grade
9 - Grade/differentiation unknown, not stated, or not applicable
Module Rule
None
Alternate Names
Aregenerative anemia
Primary refractory anemia
RA
Refractory anemia without sideroblasts
Refractory cytopenia with unilineage dysplasia
Definition
Refractory anemia (RA) is a specific type of myelodysplastic syndrome that is characterized mainly by unilineage dysplasia affecting erythroid series. (Diagnosis of exclusion)
Myeloblasts < -1% in blood, <5% in marrow
There should be a period of observation of six months followed by a re-evaluation before a definitive diagnosis of RA is established.

MDS is a group name for a number of specific diseases. As the MDS progresses, it may manifest as several subtypes. This is a part of the disease process. Abstracting each of the subtypes would result in over-counting these diseases. Code the first subtype that is diagnosed. Do not change the histology code or create a new abstract for any subsequent specific MDS subtypes.
Abstractor Notes
RA is any of a group of anemic conditions not associated with another disease and is marked by a persistent, frequently advanced anemia that can only be successfully treated with blood transfusions. RA is only anemia. The early cells that develop into red blood cells have an abnormal appearance (called dysplasia). The number of very early cells (called blasts) is normal (less than 5%). About 5% to 10% of all MDS patients have refractory anemia. This type seldom, if ever, progresses to acute myeloid leukemia and patients with this type typically have long survivals. The peripheral blood smear usually shows normochromic, normocytic, or normochromic macrocytic. Blasts are rarely seen and, if present, account for <1% of the white blood cells. The erythroid precursors in the BM vary from decreased to markedly increased. The BM must show unequivocal evidence of dysplasia (dysplasia must be present in 10% or more erythroid precursors). Ring sideroblasts may be present. The BM biopsy is generally hypercellular.
Definitive Diagnostic Methods
Clinical diagnosis
Bone marrow biopsy
Genetics Data
None
Immunophenotyping
None
Treatments
Bone marrow transplant
Chemotherapy
Other treatment
Transformations from
None
Corresponding ICD-9 Codes
238.72 Low grade myelodysplastic syndrome lesions
Corresponding ICD-10 Codes
D46.4 Refractory anemia, unspecified
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
D46.4 Refractory anemia, unspecified
Signs and Symptoms
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None