Name

Myelodysplastic syndrome with ring sideroblasts and multilineage dysplasia

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)

Abstractor Notes

(This code is effective for cases diagnosed 2021 and later. For cases diagnosed prior to 2021 see code: 9985/3.)

The presenting symptoms are usually related to anemia. The peripheral blood and bone marrow are the principal 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.

For more information on myelodysplastic syndromes, see the NCI website: http://www.cancer.gov/types/myeloproliferative/hp/myelodysplastic-treatment-pdq#section/_1 or http://www.cancer.gov/types/myeloproliferative/patient/myelodysplastic-treatment-pdq#section/_1

Grade

Not Applicable

Module Rule

None

Alternate Names

MDS-RS-MLD
Refractory cytopenia with multilineage dysplasia and ring sideroblasts

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

Bone marrow biopsy
Genetic testing

Genetics Data

DNMT3A
SF3B1
SRSF2
TET2
U2AF1
ZRSR2

Immunophenotyping

None

Treatments

None

Transformations from

None

Corresponding ICD-9 Codes

None

Corresponding ICD-10 Codes

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

Blood chemistry studies
Cytogenetic analysis
FISH (flourescence in situ hybridzation)
Flow cytometry
Immunocytochemistry
Immunophenotyping
Peripheral blood smear

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

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, Second Revision. Geneva: World Health Organization, 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

National Cancer Institute
Section: General Information About Myelodysplastic Syndromes
Pages: https://www.cancer.gov/types/myeloproliferative/patient/myelodysplastic-treatment-pdq
Glossary