Primary myelofibrosis

Name
Primary myelofibrosis
ICD-O-3 Morphology
9961/3
Effective 2001 and later
Reportable
for cases diagnosed 2001 and later
Primary Site(s)
C421

Help me code for diagnosis year :

Grade
9 - Grade/differentiation unknown, not stated, or not applicable
Module Rule
None
Alternate Names
Agnogenic myeloid metaplasia AMM
Chronic granulocytic-megakaryocytic myelosis
Chronic idiopathic myelofibrosis
Chronic idiopathic myelofibrosis (with extramedullary hematopoiesis)
CIMF
Idiopathic myelofibrosis
Megakaryocytic myelosclerosis
MMM
Myelofibrosis with myeloid metaplasia
Myelofibrosis-osteosclerosis
Myeloid metaplasia
Myeloid metaplasia, NOS
Myelosclerosis with myeloid metaplasia
PMF
Prefibrotic primary myelofibrosis
Definition
PMF is a disorder of the bone marrow in which the marrow is replaced by fibrous tissue. This is a clonal myeloproliferative disease which is characterized by a proliferation of mainly megakaryocytic and granulocytic elements in bone marrow. It is associated with reactive deposition of bone marrow connective tissue and with extra-medullary hematopoiesis (EMH).

Primary myelofibrosis begins with a hypercellular marrow with absence of overt fibrosis and often with early increase in reticulin fibers. This is call the "prefibrotic" stage of primary myelofibrosis.
Abstractor Notes
Primary myelofibrosis is a rare bone marrow disorder characterized by abnormalities in blood cell production and progressive scarring of the bone marrow. Blood forms in sites other than bone marrow such as liver and spleen. The blood and bone marrow are always involved. Blood transfusions are given to correct anemia and should not be listed as treatment. Splenectomy may be done if the patient is symptomatic . About 50% exhibit JAK2 gene mutation, but the test is not specific enough to provide a definitive diagnosis. This disease is diagnosed clinically (a diagnosis of exclusion). The physician correlates information from JAK2 (if available), the equivocal results of the bone marrow and blood work with the clinical symptoms to arrive at a diagnosis of primary myelofibrosis.

In the early stages of disease, CD34 may be slightly increased in the bone marrow but not in the peripheral blood. In the later stages CD34 will appear in large numbers. This is peculiar to Primary myelofibrosis and does not occur in Polycythemia vera or Essential thrombocythemia.
Definitive Diagnostic Methods
Clinical diagnosis
Genetics Data
JAK-2
Immunophenotyping
None
Treatments
Blood thinners, anti-clotting medications, aspirin
Chemotherapy
Endocrine
Immunotherapy
Stem cell transplant
Transformations from
None
Corresponding ICD-9 Codes
238.76 Myelofibrosis with myeloid metaplasia
Corresponding ICD-10 Codes
D47.1 Chronic myeloproliferative disease
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
D47.4 Osteomyelofibrosis
Signs and Symptoms
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None