/primary myelofibrosis

primary myelofibrosis

Rare Disease

MONDO:0009692

Also Known As

AMMAgnogenic myeloid metaplasiaCIMFaleukemic myelosisbone marrow fibrosischronic idiopathic myelofibrosisidiopathic bone marrow fibrosisidiopathic myelofibrosismegakaryocytic myelosclerosismyelofibrosis with myeloid metaplasia, somaticmyelofibrosis, somaticmyelosclerosismyelosclerosis with myeloid metaplasiaosteomyelofibrosisprimary myelofibrosismyelofibrosis with myeloid metaplasiamyelofibrosismyeloid metaplasia

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.