Name

ICD-O-2 Morphology

9980/1: Refractory anemia, NOS
9981/1: Refractory anemia without sideroblasts

ICD-O-3 Morphology

9980/3: Refractory anemia
Effective 2001 and later

Reportable

for cases diagnosed 2001 and later

Primary Site(s)

C421
Primary site must be bone marrow (C421)

Grade

Not Applicable

Module Rule

None

Alternate Names

Aregenerative anemia
Myelodysplastic syndrome without ring sideroblasts and single lineage dysplasia
RA

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.

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), 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.

Definitive Diagnostic Methods

Bone marrow biopsy
Clinical diagnosis

Genetics Data

None

Immunophenotyping

None

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Immunotherapy

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 (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

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

International Classification of Diseases for Oncology, Third Edition, First Revision. Geneva: World Health Organization, 2013.
Section: ICD-O-3.1 (2011) Morphological Codes
Pages: http://codes.iarc.fr/codegroup/2

National Cancer Institute
Section: General Information About Myelodysplastic Syndromes
Pages: https://www.cancer.gov/types/myeloproliferative/patient/myelodysplastic-treatment-pdq
Glossary