Search Database ICD-O-3 Code Lists

Name

Myelodysplastic syndrome with ring sideroblasts and single lineage dysplasia (MDS-RS-SLD) (ICD-O-4: 99820/3)

ICD-O-3 Morphology

9982/3: Myelodysplastic syndrome with ring sideroblasts and single lineage dysplasia
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 syndrome with ring sideroblasts and single lineage dysplasia (MDS-RS-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)

New preferred terminology for this histology is the MDS with low blasts and SF3B1 mutation.

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

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 WITH or WITHOUT immunophenotyping or genetics (diagnostic confirmation codes 1, 2, 4)

Module Rule

None

Alternate Names

Myelodysplastic neoplasm (MDS) with low blasts and ring sideroblasts (ICD-O-4: 99821/3)
Myelodysplastic syndrome with ring sideroblasts, NOS (MDS-RS) (ICD-O-4: 99820/3)
Myelodysplastic /myeloproliferative neoplasm with low blasts and SF3B1 mutation (ICD-O-4: 99821/3)
Myelodysplastic/myeloproliferative neoplasm (MDS/MPN) with ring sideroblasts and thrombocytosis)
Myelodysplastic/myeloproliferative neoplasm (MDS/MPN) with SF3B1 mutation and thrombocytosis (ICD-O-4: 99822/3)
Myelodysplastic/myeloproliferative neoplasm with ring sideroblasts and thrombocytosis (ICD-O-4: 99822/3)
Refractory anemia with ring sideroblasts, NOS (ICD-O-4: 99820/3)
Refractory anemia with sideroblasts (RARS) (ICD-O-4: 99820/3)
Refractory anemia with ring sideroblasts associated with marked thrombocytosis (ICD-O-4: 99822/3)

Definition

Myelodysplastic neoplasm (MDS) with low blasts and SF3B1 mutation (MDS-SF3B1) is a myeloid neoplasm with cytopenia, and dysplasia characterized by SF3B1 mutation and often ring sideroblasts. (WHO 5th edition)

Definitive Diagnostic Methods

Cytogenetics
Genetic testing
Histologic confirmation

Genetics Data

SF3B1 mutation

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)

D46.1 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

Diagnostic Exams

Bone marrow aspiration and biopsy
CT (CAT) Scan
Cytogenetic analysis
Flow cytometry
Immunophenotyping
Molecular analysis
Peripheral blood smear
Physical exam and history

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

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: 78-79

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

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 <02/05/2025>. [PMID: 26389450]
Section: Myelodysplastic Syndromes Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/myeloproliferative/hp/myelodysplastic-treatment-pdq#top
Glossary