Search Database ICD-O-3 Code Lists

Name

Myelodysplastic neoplasm with increased blasts (MDS-IB) (ICD-O-4: 99830/3)

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.

1. For cases diagnosed 2010-2026, multiple occurrences of MDS (9980, 9982, 9983, 9985, 9986, 9989, 9991, 9992, 9993) will always be one primary.
a. If there are multiple diagnoses of MDS
- 9980/3, 9982/3, 9983/3, 9985/3, 9986/3, 9991/3, 9992/3, 9993/3 take priority over 9989/3
- 9983/3 takes priority over 9980/3, 9982/3, 9985/3, 9986/3, 9991/3, 9992/3, 9993/3
- Remaining combinations: Use the first diagnosis

2. For cases diagnosed 2027 and later, if a diagnosis of MDS with increased (excess) blasts (MDS-IB) (9983/3) follows a previous diagnosis of MDS (9980, 9982, 9985, 9986, 9991-9993), then the MDS-IB is abstracted as a NEW primary.
a. The first diagnosis of MDS may be at any time, but the MDS-IB as a second diagnosis must be 2027 or later.
b. MDS-IB is a more aggressive type of MDS and is a transformation within the MDS diagnoses. Treatment for MDS-IB is the same as acute myeloid leukemia.
c. The chronic/acute rules (M8-M13) will apply to these cases.

3. For cases diagnosed 2027 and later, all other multiple occurrences of MDS are still one primary with the following priority order
- 9980/3, 9982/3, 9985/3, 9986/3, 9991/3, 9992/3, 9993/3 take priority over 9989/3
- Remaining combinations: Use the first diagnosis

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

Diagnostic Confirmation

Assign code 1 for histological confirmation WITH or WITHOUT positive immunophenotyping or genetics

Module Rule

None

Alternate Names

Childhood myelodysplastic neoplasm with excess blasts (ICD-O-4: 99834/3)
Childhood myelodysplastic neoplasm with increased blasts (cMDS) (ICD-O-4: 99834/3)
Myelodysplastic syndrome with increased blasts (ICD-O-4: 99830/3)
Myelodysplastic neoplasm with increased blasts 1 (MDS-EB-1) (ICD-O-4: 99831/3)
Myelodysplastic neoplasm with excess blasts (MDS-EB) (ICD-O-4: 99830/3)
Myelodysplastic neoplasm with increased blasts 2 (MDS-EB-2) (ICD-O-4: 99832/3)
Myelodysplastic neoplasm with increased blasts and fibrosis (MDS-F) (ICD-O-4: 99833/3)
Myelodysplastic syndrome with excess blasts-1 (RAEB I) (ICD-O-4: 99831/3)
Myelodysplastic syndrome with excess blasts-2 (RAEB II) (ICD-O-4: 99832/3)
Refractory anemia with excess blasts in transformation (RAEB-T) (see 9984/3 prior to 2010) (ICD-O-4: 99830/3)
Refractory anemia with excess blasts, NOS (RAEB, NOS) (ICD-O-4: 99830/3)

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

ASXL1, RUNX1, EZH2, NRAS, KRAS, TP53

Immunophenotyping

CD34, CD38, CD117, HLA-DR

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
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