Search Database ICD-O-3 Code Lists

Name

Myelodysplastic neoplasm with low blasts, NOS (MDS-LB)

ICD-O-3 Morphology

9985/3: Myelodysplastic syndrome with multilineage 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 neoplasm with low blasts, NOS (MDS-LB) 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)

Blood and bone marrow are always involved. At least 2 types of blood counts are low and have an abnormal appearance under the microscope (dysplasia). The number of blasts is less than 5%.

This histology code also includes childhood MDS. MDS is very rare in children. Both the peripheral blood and bone marrow are involved.

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

Childhood myelodysplastic neoplasm with low blasts (cMDS-LB)
Childhood myelodysplastic neoplasm with low blasts, hypocellular
Myelodysplastic neoplasm, hypoplastic (hMDS)
Myelodysplastic neoplasm with biallelic TP53 inactivation
Myelodysplastic neoplasm with biallelic TP53 mutation (MDS-biTP53)
Myelodysplastic neoplasm with low blasts and multilineage dysplasia (MDS-LB-MLD)
Myelodysplastic syndrome with multilineage dysplasia (MDS-MLD)
Refractory cytopenia of childhood (RCC)
Refractory cytopenia with multilineage dysplasia (RCMD)

Definition

There are several different types of MDS with low blasts that are covered under this code.

Myelodysplastic neoplasm (MDS) with low blasts (MDS-LB) is a myeloid neoplasm with cytopenia and dysplasia but without defining genetic abnormalities, defined by the presence of < 5% bone marrow blasts and < 2% peripheral blood blasts. (WHO 5th edition). This diagnosis should only be used after excluding MDS with defining genetic abnormalities.

Myelodysplastic neoplasm (MDS) with biallelic TP53 inactivation (MDS-biTP53) is a myeloid neoplasm with cytopenia, dysplasia, < 20% blasts, and < 30% proerythroblasts, characterized by two or more TP53 mutations or one TP53 mutation and evidence of concurrent TP53 copy loss or copy-neutral loss of heterozygosity (LOH). (WHO 5th edition)

Hypoplastic myelodysplastic neoplasm (hMDS) is a myeloid neoplasm with cytopenia and dysplasia, characterized by significantly decreased age-adjusted bone marrow cellularity as determined on a trephine biopsy. (WHO 5th edition)

Childhood myelodysplastic neoplasm with low blasts (cMDS-LB) is a myeloid neoplasm with cytopenia and dysplasia arising in children and adolescents (≤ 18 years of age), defined by < 5% bone marrow blasts and < 2% peripheral blood blasts. (WHO 5th edition)

Definitive Diagnostic Methods

Cytogenetics
Genetic testing
Histologic confirmation
Immunohistochemistry
Immunophenotyping

Genetics Data

ASXL1 mutation
Biallelic TP53 alterations
DNMT3A mutation
SRSF2 mutation
U2AF1 mutation

Immunophenotyping

CD34+ blast percentage
Mutant p53 expression pattern

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)

D46.7 Other myelodysplastic syndromes

Corresponding ICD-10-CM Codes (U.S. only)

D46.A Refractory cytopenia with multilineage dysplasia (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
Cytogenetic analysis
Immunocytochemistry
Immunophenotyping
Peripheral blood smear/flow cytometry
Physical exam and history

Progression and Transformation

~10% of cases evolve to AML in 2 years

Epidemiology and Mortality

Age (Adult): 70 years median age
Age (Childhood): all age groups
Incidence (Adult): accounts for ~30% of MDS cases
Incidence (Childhood): most common MDS of childhood, ~50%
Sex (Adult): slight male predominance
Sex (Childhood): no male or female predominance
Survival: 30 month median survival (~1/2 of patients died within 2 years)

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: 80-86, 90-92

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 <02/06/2025>. [PMID: 26389450]
Section: Myelodysplastic Syndromes Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/myeloproliferative/hp/myelodysplastic-treatment-pdq
Glossary