Search Database ICD-O-3 Code Lists

Name

Myelodysplastic neoplasm with low blasts and single-lineage dysplasia (MDS-LB-SLD)

ICD-O-3 Morphology

9980/3: Myelodysplastic syndrome with 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 neoplasm with low blasts and single-lineage dysplasia (MDS-LB-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)

Refractory anemia (RA) is a specific type of myelodysplastic syndrome that is characterized mainly by unilineage dysplasia affecting erythroid series. (Diagnosis of exclusion).

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

There should be a period of observation of six months followed by a re-evaluation before a definitive diagnosis of RA is established.

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 is a histology for which the Definitive Diagnostic Method does not include Genetics Data or Immunophenotyping, thus Diagnostic Confirmation should never be 3. If genetics and/or immunophenotyping are available, re-review to see if a more specific neoplasm can be coded.

Module Rule

None

Alternate Names

Myelodysplastic syndrome without ring sideroblasts and single lineage dysplasia
(Primary) refractory anemia, NOS (RA)
Refractory anemia without sideroblasts
Refractory cytopenia with unilineage dysplasia (RCUD)
Refractory neutropenia [OBS] (2021+, for cases 2010-2020, see 9991/3)
Refractory thrombocytopenia [OBS] ((2021+, for cases 2010-2020, see 9992/3)

Definition

RA is any of a group of anemic conditions not associated with another disease and is marked by a persistent, frequently advanced anemia that can only be successfully treated with blood transfusions. RA is only anemia.

The early cells that develop into red blood cells have an abnormal appearance (called dysplasia). The number of very early cells (called blasts) is normal (less than 5%).

The peripheral blood smear usually shows normochromic, normocytic, or normochromic macrocytic. Blasts are rarely seen and, if present, account for <1% of the white blood cells.

The erythroid precursors in the BM vary from decreased to markedly increased. The BM must show unequivocal evidence of dysplasia (dysplasia must be present in 10% or more erythroid precursors). Ring sideroblasts may be present. The BM biopsy is generally hypercellular.

Definitive Diagnostic Methods

Clinical diagnosis
Histologic confirmation

Genetics Data

None

Immunophenotyping

None

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)

D46.0 Refractory anemia without sideroblasts, so stated
D46.4 Refractory anemia, unspecified

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

D46.0 Refractory anemia without ring sideroblasts, so stated (effective October 01, 2015)
D46.4 Refractory anemia, unspecified (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 and aspiration
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 AML

Epidemiology and Mortality

Age: 65-70 years median age
Incidence: 10-20% of all MDS cases
Sex: no male or female predominance
Survival: 69-108 median survival time

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: 83-84

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/patient/myelodysplastic-treatment-pdq
Glossary