Search Database ICD-O-3 Code Lists

Name

Primary myelofibrosis, NOS (PMF)

ICD-O-3 Morphology

9961/3: Myelosclerosis with myeloid metaplasia
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

Primary myelofibrosis (PM) is part of the Myeloproliferative neoplasms (MPN) lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B2)

"Post essential thrombocythemia myelofibrosis" is not a diagnosis associated with 9961/3. It is a diagnosis associated with a progression of essential thrombocythemia (ET) (9962/3).

Asymptomatic low-risk patients are monitored with a watching waiting approach. Treatment is not initiated until patient becomes symptomatic.

Profound anemia usually develops with this neoplasm, which is treated with red blood cell transfusion. The transfusion is treating the symptoms, not the cancer (do not record as treatment).

Other treatments include
* Erythropoietic growth factors
* Hormones
* Immunotherapy
* Splenectomy (for enlarged spleen)

JAK2 status is collected in the following SSDI:
* NAACCR# 3862: JAK2

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.

Module Rule

None

Alternate Names

Agnogenic myeloid metaplasia (AMM)
Myelofibrosis with myeloid metaplasia
Myelofibrosis-osteosclerosis
Myelofibrosis as a result of myeloproliferative disease
Myelosclerosis with myeloid metaplasia
Primary myelofibrosis, prefibrotic (pre-PMF)
Primary myelofibrosis, fibrotic

Definition

"Primary myelofibrosis (PMF) is a myeloproliferative neoplasm (MPN) characterized by abnormal proliferation of the megakaryocytic and granulocytic lineages associated with progressive marrow fibrosis, osteosclerosis, and extramedullary haematopoiesis." (WHO 5th edition)

About 1/3 of patients are asymptomatic at diagnosis.

In later stages of disease, hematopoiesis becomes prominent, especially in the spleen. Extramedullary disease may be present in lymph nodes, CNS, skin, pericardium, peritoneum, pleura, ovaries, kidneys, adrenals, gastrointestinal tract, and lungs.

PMF is now defined as prefibrotic (pre-PMF) or fibrotic (MF).
* pre-PMF is characterized by a hypercellular bone marrow, minimal to absent reticulin fibrosis (grade 0 or 1) and more latent disease course.

* MF (or fibrotic MF) (MF-2, MF-3) is characterized by increased megakaryocytes

Morphological features of PMF are variable and different significantly between PMF and pre-PMF. A grading system was developed so that grading would have a reproducible and standard criterion. The grading system is called the "Semiquantitative WHO grading system for MF."

MF grading is required at diagnosis and during follow up. The severity of the MF is measured to determine the response to treatments. The grades are noted as MF-0, MF-1, MF-2, MF-3
- Note: do not record this grade in the Grade fields. Grade is not collected for Heme neoplasms.

Definitive Diagnostic Methods

Clinical diagnosis
Cytogenetics
Genetic testing

Genetics Data

del(13q) (common recurrent abnormality
del(20q) (common recurrent abnormality)
JAK2 V617F
MPL
Trisomy 8 and 9 (common recurrent abnormality)

Immunophenotyping

None

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Immunotherapy
Radiation therapy
Surgery

Transformations to

Transformations from

None

Same Primaries

Corresponding ICD-10 Codes (Cause of Death codes only)

D47.1 Chronic myeloproliferative disease

Corresponding ICD-10-CM Codes (U.S. only)

D47.4 Osteomyelofibrosis (effective October 01, 2015)

Signs and Symptoms

Anemia
Anorexia
Bone pain
Early satiety
Fatigue
Fever
Night sweats
Splenomegaly
Thrombocytosis
Weight loss

Diagnostic Exams

Bone marrow aspiration and biopsy
CT (CAT) Scan
Cytogenetic analysis
Flow cytometry
Immunophenotyping
JAK 2 gene mutation test
Lumbar puncture
Molecular analysis
Peripheral blood smear
Physical exam and history

Progression and Transformation

None

Epidemiology and Mortality

Age: 60-70's years median age
Incidence: 0.44-1.5 per 100,000 persons per year
Sex: no male or female predominance
Survival: 3-7 years for patients diagnosed in fibrotic stage; 10-15 years for patients diagnosed in early prefibrotic phase

Sources

WHO Classification of Tumours Editorial Board. Haematolymphoid tumours. Lyon (France): International Agency for Research on Cancer; 2024. (WHO classification of tumours series, 5th ed.; vol. 11). https://publications.iarc.who.int/637.
Section: Myeloproliferative neoplasms
Pages: Part A: 47-52

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

PDQ® Adult Treatment Editorial Board. PDQ Myeloproliferative Neoplasms Treatment. Bethesda, MD: National Cancer Institute. Updated <09/27/24>. Available at: https://www.cancer.gov/types/myeloproliferative/hp/myeloproliferative-neoplasms-treatment. Accessed <01/22/25>. [PMID: 26389291]
Section: Myeloproliferative Neoplasms Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/myeloproliferative/hp/myeloproliferative-neoplasms-treatment#_5
Glossary