Myelodysplastic/myeloproliferative neoplasm, unclassifiable

Myelodysplastic/myeloproliferative neoplasm, unclassifiable
ICD-O-3 Morphology
9975/3: Myelodysplastic/myeloproliferative neoplasm, unclassifiable
Effective 2010 and later
for cases diagnosed 2010 and later
Primary Site(s)
Primary site must be bone marrow (C421)

Not Applicable
Module Rule
Alternate Names
Chronic myeloproliferative disease
Chronic myeloproliferative disorder
Chronic myeloproliferative neoplasm
Myeloproliferative disease, NOS
Myeloproliferative disorder, NOS
Myeloproliferative neoplasm, unclassifiable
Myeloproliferative syndrome [OBS]
Undifferentiated myeloproliferative disease
Clinical laboratory and morphological features of MPN but fail to met the criteria for a specific MPN; or presents with features that overlap two or more MPN neoplasms.
Abstractor Notes
(This neoplasm is reportable for cases diagnosed 2010 and later.)

For cases prior to 2010, Chronic myeloproliferative disease, NOS (9960/3) was used for the generic disease description.

For cases diagnosed 2010+, code 9975/3 is used for MPN, NOS as well as a new NOS for myeloproliferative neoplasm, unclassifiable and myelodysplastic/myeloproliferative syndrome unclassifiable (MPN, U) that became effective for cases diagnosed 1/1/2010.

The NOS is usually a provisional diagnosis and the physician will run further diagnostic procedures to identify specific 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.

When the disease is diagnosed very early, it may manifest symptoms of two or more specific myeloproliferative neoplasms. A specific MPN will manifest when the disease progresses and symptoms appear for that disease. When a more specific diagnosis becomes available, change the histology code to the more specific MPN code as directed in the Hematopoietic manual.

There are three groups of early stage MPN, U:
1) Early stages of polycythemia vera, primary myelofibrosis, or essential thrombocythemia in which the characteristic features are not yet fully developed.

2) Advanced disease in which pronounced myelofibrosis, osteosclerosis, or transformation to a more aggressive stage masks the underlying disorder.

3) Evidence of an MPN where a coexisting neoplastic or inflammatory disorder obscures some of the diagnostic clinical and/or morphologic features.

Specific myeloproliferative neoplasms (MPN) include:
1. Chronic eosinophilic leukemia, NOS (9964/3)
2. Chronic myelogenous leukemia BCR-ABL1 positive (9875/3)
3. Chronic neutrophilic leukemia (9963/3)
4. Essential thrombocythemia (9962/3)
5. Mast cell leukemia (9742/3)
6. Mast cell sarcoma (9740/3)
7. Polycythemia vera (9950/3)
8. Primary myelofibrosis (9961/3)
9. Systemic mastocytosis (9741/3)

Specific myelodysplastic/myeloproliferative neoplasms include (MDS/MPN):
1. Atypical chronic myeloid leukemia BCR-ABL1 negative (9876/3)
2. Chronic myelomonocytic leukemia (9945/3)
3. Juvenile myelomonocytic leukemia (9946/3)
4. Refractory anemia with ringed sideroblasts associated with marked thrombocytosis (9982/3)

Aspirin was previously documented as treatment for MPN, NOS. This was found to be incorrect. Treatment has been updated based on the NCI website. Aspirin is given to patients with MPN, NOS to reduce risk of blood clots. The aspirin is not used to manage the cancer. Treatment has been updated based on the NCI website (updated 6/12/15).
Definitive Diagnostic Methods
Bone marrow biopsy
Clinical diagnosis
Genetics Data
Transformations from
Corresponding ICD-9 Codes
238.79 Other lymphatic and hematopoietic tissues
Corresponding ICD-10 Codes
D47.1 Chronic myeloproliferative disease
Corresponding ICD-10-CM Codes (U.S. only)
C94.6 Myelodysplastic disease, not classified (effective October 01, 2015)
D47.1 Chronic myeloproliferative disease (effective October 01, 2015)
Signs and Symptoms
Easy bruising or bleeding
Frequent infections
Pain or a feeling of fullness below the rib
Pale skin
Shortness of breath
Weight loss
Progression and Transformation
Epidemiology and Mortality
Incidence: ~10-15% of all cases of MPN
Survival: Depends on what stage diagnosed at. Early stage MPN will have the same survival as some of the more specified MPN's, while late stage has a poor prognosis