Search Database ICD-O-3 Code Lists

Name

Myelodysplastic neoplasm with ring sideroblasts and multilineage dysplasia (MDS-RD-MLD)

ICD-O-3 Morphology

9993/3: Myelodysplastic syndrome with ring sideroblasts and multilineage dysplasia
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.

For MDS diseases (9980, 9982, 9983, 9985, 9986, 9989, 9991, 9992, 9993), 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.

Diagnostic Confirmation

This histology can be determined by positive histology (including peripheral blood) with or without genetics and/or immunophenotyping. Review the Definitive Diagnostic Methods, Immunophenotyping and Genetics Data sections below, and the instructions in the Hematopoietic Manual for further guidance on assigning Diagnostic confirmation.

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.

Definitive Diagnostic Methods

Cytogenetics
Genetic testing
Histologic confirmation

Genetics Data

DNMT3A mutation
SRSF2 mutation
U2AF1 mutation
ZRSR2 mutation

Immunophenotyping

None

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

Cytopenia (of two or more myeloid lines)
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
Peripheral blood smear
Physical exam and history
Molecular analysis

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

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

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
Glossary