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Name
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
Myelodysplastic neoplasm with low blasts and single-lineage dysplasia (MDS-LB-SLD) is part of the Myelodysplastic neoplasm's lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B4)
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.
1. For cases diagnosed 2010-2026, multiple occurrences of MDS (9980, 9982, 9983, 9985, 9986, 9989, 9991, 9992, 9993) will always be one primary.
a. If there are multiple diagnoses of MDS
- 9980/3, 9982/3, 9983/3, 9985/3, 9986/3, 9991/3, 9992/3, 9993/3 take priority over 9989/3
- 9983/3 takes priority over 9980/3, 9982/3, 9985/3, 9986/3, 9991/3, 9992/3, 9993/3
- Remaining combinations: Use the first diagnosis
2. For cases diagnosed 2027 and later, if a diagnosis of MDS with increased (excess) blasts (MDS-IB) (9983/3) follows a previous diagnosis of MDS (9980, 9982, 9985, 9986, 9991-9993), then the MDS-IB is abstracted as a NEW primary.
a. The first diagnosis of MDS may be at any time, but the MDS-IB as a second diagnosis must be 2027 or later.
b. MDS-IB is a more aggressive type of MDS and is a transformation within the MDS diagnoses. Treatment for MDS-IB is the same as acute myeloid leukemia.
c. The chronic/acute rules (M8-M13) will apply to these cases.
3. For cases diagnosed 2027 and later, all other multiple occurrences of MDS are still one primary with the following priority order
- 9980/3, 9982/3, 9985/3, 9986/3, 9991/3, 9992/3, 9993/3 take priority over 9989/3
- Remaining combinations: Use the first diagnosis
Hematologic Transplant and/or Endocrine Procedures treatments include bone marrow and stem cell transplants.
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.
1. For cases diagnosed 2010-2026, multiple occurrences of MDS (9980, 9982, 9983, 9985, 9986, 9989, 9991, 9992, 9993) will always be one primary.
a. If there are multiple diagnoses of MDS
- 9980/3, 9982/3, 9983/3, 9985/3, 9986/3, 9991/3, 9992/3, 9993/3 take priority over 9989/3
- 9983/3 takes priority over 9980/3, 9982/3, 9985/3, 9986/3, 9991/3, 9992/3, 9993/3
- Remaining combinations: Use the first diagnosis
2. For cases diagnosed 2027 and later, if a diagnosis of MDS with increased (excess) blasts (MDS-IB) (9983/3) follows a previous diagnosis of MDS (9980, 9982, 9985, 9986, 9991-9993), then the MDS-IB is abstracted as a NEW primary.
a. The first diagnosis of MDS may be at any time, but the MDS-IB as a second diagnosis must be 2027 or later.
b. MDS-IB is a more aggressive type of MDS and is a transformation within the MDS diagnoses. Treatment for MDS-IB is the same as acute myeloid leukemia.
c. The chronic/acute rules (M8-M13) will apply to these cases.
3. For cases diagnosed 2027 and later, all other multiple occurrences of MDS are still one primary with the following priority order
- 9980/3, 9982/3, 9985/3, 9986/3, 9991/3, 9992/3, 9993/3 take priority over 9989/3
- Remaining combinations: Use the first diagnosis
Hematologic Transplant and/or Endocrine Procedures treatments include bone marrow and stem cell transplants.
Diagnostic Confirmation
This neoplasm can be diagnosed clinically (diagnostic confirmation codes 5, 6, 8) or histologically (diagnostic confirmation codes 1, 2, 4)
Module Rule
None
Alternate Names
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
Clinical diagnosis
Histologic confirmation
Genetics Data
No specific molecular genetic profile
Immunophenotyping
No specific immunophenotyping profile
Treatments
Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Immunotherapy
Transformations to
Transformations from
None
Same Primaries
Corresponding ICD-10 Codes (Cause of Death codes only)
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
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
WHO Classification of Tumours Editorial Board. Haematolymphoid tumours. Lyon (France): International Agency for Research on Cancer; 2024. (WHO classification of tumours series, 5th ed.; vol. 11). https://publications.iarc.who.int/637.
Section: Myelodysplastic neoplasms
Pages: Part A: 83-84
Section: Myelodysplastic neoplasms
Pages: Part A: 83-84
International Classification of Diseases for Oncology, 3rd edition (including revisions). Geneva: World Health Organization, 2001, 2011, 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
PDQ® Adult Treatment Editorial Board. PDQ Myelodysplastic Syndromes Treatment. Bethesda, MD: National Cancer Institute. Updated <09/19/2024>. Available at: https://www.cancer.gov/types/myeloproliferative/hp/myelodysplastic-treatment-pdq. Accessed <02/06/2025>. [PMID: 26389450]
Section: Myelodysplastic Syndromes Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/myeloproliferative/patient/myelodysplastic-treatment-pdq
Section: Myelodysplastic Syndromes Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/myeloproliferative/patient/myelodysplastic-treatment-pdq
