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ICD-O-2 Morphology
9980/1: Refractory anemia, NOS
ICD-O-3 Morphology
Effective
2001 and later
Reportable
for cases diagnosed
2001 and later
Primary Site(s)
C421
Primary site must be bone marrow (C421)
Coding Manual:
Hematopoietic Coding Manual (PDF)
Abstractor Notes
Refractory anemia (RA) is a specific type of myelodysplastic syndrome that is characterized mainly by unilineage dysplasia affecting erythroid series. (Diagnosis of exclusion).
The principle sites of involvement are the peripheral blood and bone marrow.
There should be a period of observation of six months followed by a re-evaluation before a definitive diagnosis of RA is established.
For MDS diseases (9980, 9982, 9983, 9985, 9986, 9989, 9991, 9992, 9993), abstracting each of the subtypes would result in over-counting of the diseases.
1. Code only the first subtype that is diagnosed.
2. Do not change the histology code or create a new abstract for any subsequent specific MDS subtypes.
The principle sites of involvement are the peripheral blood and bone marrow.
There should be a period of observation of six months followed by a re-evaluation before a definitive diagnosis of RA is established.
For MDS diseases (9980, 9982, 9983, 9985, 9986, 9989, 9991, 9992, 9993), abstracting each of the subtypes would result in over-counting of the diseases.
1. Code only the first subtype that is diagnosed.
2. Do not change the histology code or create a new abstract for any subsequent specific MDS subtypes.
Diagnostic Confirmation
This is a histology for which the Definitive Diagnostic Method does not include Genetics Data or Immunophenotyping, thus Diagnostic Confirmation should never be 3. If genetics and/or immunophenotyping are available, re-review to see if a more specific neoplasm can be coded.
Grade
Not Applicable
Module Rule
None
Alternate Names
Aregenerative anemia
MDS-SLD
Primary refractory anemia
RA
Refractory anemia, NOS
Refractory anemia without sideroblasts
Definition
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%).
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.
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%).
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
Bone marrow biopsy
Clinical diagnosis
Genetics Data
None
Immunophenotyping
None
Treatments
Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Immunotherapy
Transformations to
Transformations from
None
Same Primaries
Corresponding ICD-9 Codes
238.72 Low grade myelodysplastic syndrome lesions
Corresponding ICD-10 Codes
D46.0 Refractory anemia without sideroblasts, so stated
D46.4 Refractory anemia, unspecified
Corresponding ICD-10-CM Codes (U.S. only)
D46.0 Refractory anemia without ring sideroblasts, so stated (effective October 01, 2015)
D46.4 Refractory anemia, unspecified (effective October 01, 2015)
Signs and Symptoms
Easy bruising or bleeding
Petechiae (flat, pinpoint spots under the skin caused by bleeding)
Shortness of breath
Skin paler than usual
Weakness or feeling tired
Diagnostic Exams
Blood chemistry studies
Bone marrow and aspiration
Cytogenetic analysis
Flow cytometry
Immunocytochemistry
Immunophenotyping
Peripheral blood smear
Progression and Transformation
1-2% of cases evolve to AML
Epidemiology and Mortality
Age: 65-70 years median age
Incidence: 10-20% of all MDS cases
Sex: no male or female predominance
Survival: 69-108 median survival time
Sources
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Myelodysplastic syndromes
Pages: 106-109
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Myelodysplastic syndromes
Pages: 106-109
International Classification of Diseases for Oncology, Third Edition, Second Revision. Geneva: World Health Organization, 2020.
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577
National Cancer Institute
Section: General Information About Myelodysplastic Syndromes
Pages: https://www.cancer.gov/types/myeloproliferative/patient/myelodysplastic-treatment-pdq
Section: General Information About Myelodysplastic Syndromes
Pages: https://www.cancer.gov/types/myeloproliferative/patient/myelodysplastic-treatment-pdq
