Primary myelofibrosis
Primary myelofibrosis
Definition
Myelofibrosis with myeloid metaplasia is a myeloproliferative disease with annual incidence of approximately 1 case per 100,000 individuals and age at diagnosis around 60 (an increased prevalence is noted in Ashkenazi Jews). Clinical manifestations depend on the type of blood cell affected and may include anemia, pallor, splenomegaly, hypermetabolic state, petechiae, ecchymosis, bleeding, lymphadenopathy, hepatomegaly, portal hypertension.
Also known as Agnogenic myeloid metaplasia, AMM, chronic idiopathic myelofibrosis, CIMF, idiopathic bone marrow fibrosis, idiopathic myelofibrosis, myelofibrosis with myeloid metaplasia, somatic, myelofibrosis, somatic, myelosclerosis with myeloid metaplasia, osteomyelofibrosis, primary myelofibrosis — per MONDO
Also identified as
- DOID 4971 per MONDO
- ICD10CM D47.4 per MONDO
- ICD9 238.76 per MONDO
- ICD9 289.83 per MONDO
- MESH D055728 per MONDO
- NCIT C2862 per MONDO
- OMIM 254450 per MONDO
- Orphanet 824 per MONDO
- UMLS C0001815 per MONDO
Affected Organs
| Organ | Involvement | Source |
|---|---|---|
| Bone marrow | Disease Has Associated Anatomic Site | NCIT · CC BY 4.0 |
| Bone marrow | Disease Has Primary Anatomic Site | NCIT · CC BY 4.0 |
Drugs indicated
| Drug | Relation | Source |
|---|---|---|
| Busulfan | may treat | MEDRT · Public domain (U.S. Government work) |
| Fedratinib | may treat | MEDRT · Public domain (U.S. Government work) |
| Momelotinib | may treat | MEDRT · Public domain (U.S. Government work) |
| Pacritinib | may treat | MEDRT · Public domain (U.S. Government work) |
| Ruxolitinib | may treat | MEDRT · Public domain (U.S. Government work) |