Name

ICD-O-2 Morphology

ICD-O-3 Morphology

9982/3: Refractory anemia with ring sideroblasts
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

Myelodysplastic/myeloproliferative neoplasm with ring sideroblasts and thrombocytosis
RARS
Refractory anemia with ring sideroblasts associated with marked thrombocytosis

Definition

The peripheral blood may contain a minor population of hypochromic cells but will not show blasts. The bone marrow aspirate smear shows an increase in erythroid precursors with erythroid lineage dysplasia.

> or equal to 15% ringed sideroblasts in marrow
< 5% myeloblasts in marrow, not in blood
Erythroid dysplasia

Abstractor Notes

People with RA with ring sideroblasts are similar to those with refractory anemia except that many of the red blood cells in the bone marrow contain characteristic ring-shaped iron deposits which are called ring sideroblasts.

The principle sites of involvement are the peripheral blood and bone marrow.

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.

Hematologic Transplant and/or Endocrine Procedures treatments include bone marrow and stem cell transplants.

Definitive Diagnostic Methods

Bone marrow biopsy
Peripheral blood smear

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

D46.1 Refractory anemia with sideroblasts

Corresponding ICD-10-CM Codes (U.S. only)

D46.1 Refractory anemia with sideroblasts (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 acute myeloid leukemia

Epidemiology and Mortality

Age: 60-73 years median age
Incidence: 3-11% of MDS cases
Sex: no male or female predominance
Survival: 69-108 months median survival

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: 109-111

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