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Name
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.
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.
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.
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
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)
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, DNMT3A, SRSF2, TET2, TP53, U2AF1 mutations
Immunophenotyping
CD34, p53
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
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
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
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
Section: Myelodysplastic Syndromes Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/myeloproliferative/hp/myelodysplastic-treatment-pdq
