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Name
Myelodysplastic neoplasm, NOS
ICD-O-3 Morphology
9989/3: Myelodysplastic syndrome, unclassifiable
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, NOS 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)
Myelodysplastic syndrome, NOS is a generic disease description. DCO cases or path report only cases may stay in this classification. In most cases, NOS histology is only the provisional diagnosis; the physician will run further diagnostic procedures and look for various clinical presentations to identify a more specific disease.
Further review of the medical record should be done to look for the tests listed as definitive diagnosis. The more specific
myelodysplastic syndromes are:
1. Myelodysplastic syndrome with excess blasts (9983/3)
2. Myelodysplastic syndrome with multilineage dysplasia (9985/3)
3. Myelodysplastic syndrome with ring sideroblasts and single lineage dysplasia (9982/3)
4. Myelodysplastic syndrome with single lineage dysplasia (9980/3)
5. Refractory cytopenia of childhood (9985/3)
6. Refractory neutropenia (9991/3)
7. Refractory thrombocytopenia (9992/3)
8. Myelodysplastic syndrome with ring sideroblasts and multilineage dysplasia (9993/3)
If the characteristics of a specific subtype of MDS develops later in the course of the disease, change the histology code to the more specific diagnosis.
The peripheral blood and bone marrow are the principle sites of involvement.
About 10% of MDS patients have bone marrow that is hypocellular. These cases are referred to as hypoplastic MDS. When considering the diagnosis of hypoplastic MDS, it is important to exclude toxic myelopathy and auto-immune disorders.
This is a clinical diagnosis. When the testing has excluded other diseases, the physician uses the information from the equivocal test results plus the patient's clinical history to make a diagnosis of myelodysplastic syndrome.
The patient receives supportive care. Red blood cells are transfused for anemia.
Hematologic Transplant and/or Endocrine Procedures treatments include bone marrow and stem cell transplants.
Myelodysplastic syndrome, NOS is a generic disease description. DCO cases or path report only cases may stay in this classification. In most cases, NOS histology is only the provisional diagnosis; the physician will run further diagnostic procedures and look for various clinical presentations to identify a more specific disease.
Further review of the medical record should be done to look for the tests listed as definitive diagnosis. The more specific
myelodysplastic syndromes are:
1. Myelodysplastic syndrome with excess blasts (9983/3)
2. Myelodysplastic syndrome with multilineage dysplasia (9985/3)
3. Myelodysplastic syndrome with ring sideroblasts and single lineage dysplasia (9982/3)
4. Myelodysplastic syndrome with single lineage dysplasia (9980/3)
5. Refractory cytopenia of childhood (9985/3)
6. Refractory neutropenia (9991/3)
7. Refractory thrombocytopenia (9992/3)
8. Myelodysplastic syndrome with ring sideroblasts and multilineage dysplasia (9993/3)
If the characteristics of a specific subtype of MDS develops later in the course of the disease, change the histology code to the more specific diagnosis.
The peripheral blood and bone marrow are the principle sites of involvement.
About 10% of MDS patients have bone marrow that is hypocellular. These cases are referred to as hypoplastic MDS. When considering the diagnosis of hypoplastic MDS, it is important to exclude toxic myelopathy and auto-immune disorders.
This is a clinical diagnosis. When the testing has excluded other diseases, the physician uses the information from the equivocal test results plus the patient's clinical history to make a diagnosis of myelodysplastic syndrome.
The patient receives supportive care. Red blood cells are transfused for anemia.
Hematologic Transplant and/or Endocrine Procedures treatments include bone marrow and stem cell transplants.
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
Hypoplastic myelodysplastic syndrome
Myelodysplastic disorder
Myelodysplastic syndrome, NOS (MDS)
Myelodysplastic syndrome, unclassifiable (MDS-U)
Preleukemia [OBS]
Preleukemic syndrome [OBS]
Smoldering leukemia
Definition
The myelodysplastic syndromes (MDS, formerly known as "preleukemia") are a diverse collection of hematological conditions united by ineffective production of blood cells and varying risks of transformation to acute myelogenous leukemia (AML). Anemia requiring chronic blood transfusion is frequently present.
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)
D46.9 Myelodysplastic syndrome, unspecified
Corresponding ICD-10-CM Codes (U.S. only)
D46.9 Myelodysplastic syndrome, unspecified (effective October 01, 2015)
D46.Z Other myelodysplastic syndrome, 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
Progression and Transformation
None
Epidemiology and Mortality
None
Sources
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/patient/myelodysplastic-treatment-pdq
Section: Myelodysplastic Syndromes Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/myeloproliferative/patient/myelodysplastic-treatment-pdq
