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Name
Myelodysplastic neoplasm with ring sideroblasts and multilineage dysplasia (MDS-RD-MLD) (ICD-O-4: 99930/3)
ICD-O-3 Morphology
Effective
2021 and later
Reportable
for cases diagnosed
2021 and later
Primary Site(s)
C421
Primary site must be bone marrow (C421)
Coding Manual:
Hematopoietic Coding Manual (PDF)
Abstractor Notes
(This code is effective for cases diagnosed 2021 and later. For cases diagnosed prior to 2021 see code: 9985/3.)
Myelodysplastic neoplasm with ring sideroblasts and multilineage dysplasia (MDS-RD-MLD) 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)
The presenting symptoms are usually related to anemia. The peripheral blood and bone marrow are the principle sites of involvement. The liver and spleen may show evidence of iron overload.
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.
Myelodysplastic neoplasm with ring sideroblasts and multilineage dysplasia (MDS-RD-MLD) 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)
The presenting symptoms are usually related to anemia. The peripheral blood and bone marrow are the principle sites of involvement. The liver and spleen may show evidence of iron overload.
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
Assign code 1 for histological confirmation WITH or WITHOUT positive genetics
Module Rule
None
Alternate Names
None
Definition
Myelodysplastic syndrome (MDS) with ring sideroblasts (MDS-RS) is an MDS characterized by cytopenias, morphological dysplasia and ring sideroblasts usually constituting greater than or equal to 15% of the bone marrow erythroid precursors.
In MDS with ring sideroblatss and multilineage dysplasia (MDS-RD-MLD), patients present with any number of cytopenias, and significant dysplasia is present in two or three hematopoietic lineages.
In MDS with ring sideroblatss and multilineage dysplasia (MDS-RD-MLD), patients present with any number of cytopenias, and significant dysplasia is present in two or three hematopoietic lineages.
Definitive Diagnostic Methods
Cytogenetics
Genetic testing
Histologic confirmation
Genetics Data
DNMT3A, SRSF2, TET2, U2AF1, ZRSR2 mutations
Immunophenotyping
No specific immunophenotyping profile
Treatments
None
Transformations to
Transformations from
None
Same Primaries
Corresponding ICD-10 Codes (Cause of Death codes only)
D46.7 Other myelodysplastic syndromes
Corresponding ICD-10-CM Codes (U.S. only)
D46.B Refractory cytopenia with multilineage dysplasia and ring sideroblasts (effective October 01, 2015)
Signs and Symptoms
Diagnostic Exams
Progression and Transformation
~8% of cases evolve to AML in 2 years
Epidemiology and Mortality
Age: 60-73 years median age
Incidence: accounts for ~13% of MDS cases
Survival: 28 month median survival
Sources
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 <02/05/2025>. [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
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
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Myelodysplastic syndromes
Pages: 109-111
