Myelodysplastic/myeloproliferative neoplasm, unclassifiable
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For cases prior to 2010, myeloproliferative disease, NOS histology is a generic disease description. DCO cases or path report only cases may stay in this classification. Cases diagnosed in later stages of the disease may mask the histologic features of any specific myeloproliferative disease and will remain in the NOS category.
When the disease is diagnosed very early, it may manifest symptoms of two or more specific myeloproliferative neoplasms. As the disease progresses, it will manifest the symptoms of one of the specific MPN subtypes. When a more specific diagnosis becomes available, change the histology code to the more specific MPN code as directed in the PH rules.
Many times, the NOS is a 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. Specific MPN diagnosis include chronic myelogenous leukemia BCR-ABL1 positive, chronic neutrophilic leukemia, polycythemia vera, primary myelofibrosis, essential thrombocythemia, chronic eosinophilic leukemia, NOS; systemic mastocytosis, mast cell leukemia, and Mast cell sarcoma. When a more specific diagnosis is identified, the histology should be changed to the more specific neoplasm name and code. See the histology tables for more information on NOS and more specific histologies.The designation myeloproliferative disease, NOS should be used only when no other information is available (DCO, path report only). When a more specific myeloproliferative disease is identified, change the histology code from the NOS to the more specific disease. Specific myeloproliferative diseases are: chronic myelogenous leukemia BCR-ABL1 positive, chronic neutrophilic leukemia, polycythemia vera, primary amyelofibrosis, essential thrombocythemia, chronic eosinophilic leukemia, NOS. It also includes the mastocytosis group: Systemic mastocytosis, mast cell leukemia, and mast cell sarcoma. When any of these more specific neoplasms are identified, change the histology code to the more specific name/code. See Histology Tables for more information on NOS and more specific neoplasms.
Early stages of MPN, U will fall into one of three groups: 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) patients with convincing evidence of an MPN in whom a coexisting neoplastic or inflammatory disorder obscures some of the diagnostic clinical and/or morphologic features.
Specific types of myelodysplastic/myeloproliferative neoplasms are: chronic myelomonocytic leukemia; atypical chronic myeloid leukemia BCR-ABL1 negative; juvenile myelomonocytic leukemia; refractory anemia with ringed sideroblasts associated with marked thrombocytosis. When any of these more specific neoplasms are identified, change the histology code to the more specific name/code. See Histology Tables for more information on NOS and more specific neoplasms.