An official website of the United States government
Home
Name
Myelodysplastic/myeloproliferative neoplasm, unclassifiable
ICD-O-1 Morphology
9960/1: Chronic myeloproliferative disease
Effective
1978 - 1991
ICD-O-2 Morphology
9960/1: Chronic myeloproliferative disease
Effective
1992 - 2000
ICD-O-3 Morphology
9975/3: Myelodysplastic/myeloproliferative neoplasm, unclassifiable
Effective
2010 and later
Reportable
for cases diagnosed
2010 and later
Primary Site(s)
C421
Primary site must be bone marrow (C421). Blood and bone marrow are the major sites of involvement. In advanced stages, the spleen an dliver may also be affected.
Coding Manual:
Hematopoietic Coding Manual (PDF)
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 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.
In patients with MPNs that are initially unclassifiable, follow-up studies performed at intervals of 6-12 months can often provide sufficient information for a more precise classification. If a more specific MPN is diagnosed, change the ICD-O-3 histology code to the more specific MPN. Do not abstract a new primary.
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).
For more information, see the NCI website: https://www.cancer.gov/types/myeloproliferative/patient/mds-mpd-treatment-pdq#section/_307
For cases prior to 2010, Chronic myeloproliferative disease, NOS (9960/3) was used for the generic 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.
In patients with MPNs that are initially unclassifiable, follow-up studies performed at intervals of 6-12 months can often provide sufficient information for a more precise classification. If a more specific MPN is diagnosed, change the ICD-O-3 histology code to the more specific MPN. Do not abstract a new primary.
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).
For more information, see the NCI website: https://www.cancer.gov/types/myeloproliferative/patient/mds-mpd-treatment-pdq#section/_307
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.
Grade
Not Applicable
Module Rule
None
Alternate Names
Chronic myelodysplastic/myeloproliferative disease
Chronic myeloproliferative disease
Chronic myeloproliferative disorder
Chronic myeloproliferative neoplasm
CMD
CMPD-U
Mixed myeloproliferative/myelodysplastic syndrome, unclassifiable
MPN, U
MPN-U
Myelodysplastic/myeloproliferative neoplasm, unclassifiable
Myeloid neoplasm, NOS
Myeloproliferative disease, NOS
Myeloproliferative disorder, NOS
Myeloproliferative neoplasm, unclassifiable
Myeloproliferative syndrome [OBS]
Overlap syndrome, unclassifiable
Undifferentiated myeloproliferative disease
Definition
The designation of myeloproliferative neoplasm, unclassifiable (MPN-U) should be applied only to cases that have definite clinical, laboratory, molecular, and morphological features of a myeloproliferative neoplasm (MPN) but fail to meet the diagnostic criteria for any of the specific MPN entities, or that present with features that overlap between two or more of the MPN categories.
In early unclassifiable disease, organomegaly may be minimal or absent, but splenomegaly and hepatomegaly can be massive in advanced cases in which bone marrow specimens are characterized by marked myelofibrosis and/or increased numbers of blasts.
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. Polycythemia vera (9950/3)
6. Primary myelofibrosis (9961/3)
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. Myelodysplastic/myeloproliferative neoplasm with ring sideroblasts and thrombocytosis (9982/3)
In early unclassifiable disease, organomegaly may be minimal or absent, but splenomegaly and hepatomegaly can be massive in advanced cases in which bone marrow specimens are characterized by marked myelofibrosis and/or increased numbers of blasts.
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. Polycythemia vera (9950/3)
6. Primary myelofibrosis (9961/3)
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. Myelodysplastic/myeloproliferative neoplasm with ring sideroblasts and thrombocytosis (9982/3)
Definitive Diagnostic Methods
Bone marrow biopsy
Clinical diagnosis
Genetics Data
Immunophenotyping
None
Treatments
Chemotherapy
Transformations to
Transformations from
None
Same Primaries
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)
D47.1 Chronic myeloproliferative disease (effective October 01, 2015)
C94.6 Myelodysplastic disease, not classified (effective October 01, 2015)
Signs and Symptoms
Diagnostic Exams
Progression and Transformation
None
Epidemiology and Mortality
Sources
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Myeloproliferative neoplasms
Pages: 57-59
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Myeloproliferative neoplasms
Pages: 57-59
International Classification of Diseases for Oncology, Third Edition, Second Revision. Geneva: World Health Organization, 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
National Cancer Institute
Section: General Information About Myelodysplastic/Myeloproliferative Neoplasms
Pages: https://www.cancer.gov/types/myeloproliferative/patient/mds-mpd-treatment-pdq
Section: General Information About Myelodysplastic/Myeloproliferative Neoplasms
Pages: https://www.cancer.gov/types/myeloproliferative/patient/mds-mpd-treatment-pdq
