Search Database ICD-O-3 Code Lists

Name

Myelodysplastic neoplasm with increased blasts (MDS-IB)

ICD-O-3 Morphology

9983/3: Myelodysplastic syndrome with excess blasts
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 increased blasts (MDS-IB) 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 principle sites of involvement are the peripheral blood and bone marrow. Any one of the cell types can be low and look abnormal under the microscope.

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

Hematologic Transplant and/or Endocrine Procedures treatments include bone marrow and stem cell transplants.

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 excess blasts
Childhood myelodysplastic neoplasm with increased blasts (cMDS)
Myelodysplastic syndrome with increased blasts
Myelodysplastic neoplasm with increased blasts 1 (MDS-EB-1)
Myelodysplastic neoplasm with excess blasts (MDS-EB)
Myelodysplastic neoplasm with increased blasts 2 (MDS-EB-2)
Myelodysplastic neoplasm with increased blasts and fibrosis (MDS-F)
Refractory anemia with excess blasts-1 (RAEB I)
Refractory anemia with excess blasts-2 (RAEB II)
Refractory anemia with excess blasts in transformation (RAEB-T) (see 9984/3 prior to 2010)
Refractory anemia with excess blasts, NOS (RAEB, NOS)

Definition

Myelodysplastic neoplasm (MDS) with increased blasts (MDS-IB) is a myeloid neoplasm with cytopenia and dysplasia but without defining genetic abnormalities, defined by increased blasts (≥ 5% and < 20% in bone marrow and/or ≥ 2% and < 20% in peripheral blood). (WHO 5th edition)

Childhood myelodysplastic neoplasm (cMDS) with increased blasts (cMDS-IB) is a myeloid neoplasm with cytopenia and dysplasia arising in children and adolescents (≤ 18 years of age), defined by increased blasts (≥ 5% and < 20% in bone marrow and/or ≥ 2% and < 20% in peripheral blood).

Definitive Diagnostic Methods

Cytogenetics
Genetic testing
Histologic confirmation
Immunohistochemistry
Immunophenotyping

Genetics Data

ASLX1 mutation
RUNX1 mutation

Immunophenotyping

CD13+
CD33+
CD34+
CD38+
CD117+

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)

D46.2 Refractory anemia with excess of blasts

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

D46.20 Refractory anemia with excess of blasts, unspecified (effective October 01, 2015)
D46.21 Refractory anemia with excess of blasts 1 (effective October 01, 2015)
D46.22 Refractory anemia with excess of blasts 2 (effective October 01, 2015)

Signs and Symptoms

Anemia
Easy bruising or bleeding
Neutropenia
Petechiae (flat, pinpoint spots under the skin caused by bleeding)
Shortness of breath
Skin paler than usual
Thrombocytopenia
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

~25% of RAEB-1 and 33% of RAEB-2 transform to AML

Epidemiology and Mortality

Age: > 50 years of age
Incidence: ~40% of all MDS
Sex: no male or female predominance
Survival: ~16 months for RAEB-1 and ~3-12 months for RAEB-2

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: 87-93

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