Primary myelofibrosis

Orpha code: 824OMIM code: 254450

Definition

A rare myeloproliferative neoplasm characterized by stem-cell derived clonal over proliferation of mature myeloid lineages, such as erythrocytes, leukocytes, and megakaryocytes, with variable degrees of megakaryocyte atypia, associated with reticulin and/or collagen bone marrow fibrosis, osteosclerosis, ineffective erythropoiesis, angiogenesis, extramedullary hematopoiesis, and abnormal cytokine expression.

Disease data
Classification

Disease

Synonyms
Agnogenic myeloid metaplasia
Agnogenic myeloid metaplasia
Mielofibroza idiopatyczna
Mielofibroza pierwotna
Mieloskleroza z metaplazją szpiku
Idiopathic myelofibrosis
Myelofibrosis with myeloid metaplasia
Osteomyelofibrosis
ORPHA code
824
OMIM code
254450
ICD10 code
D47.4
ICD11 code
2A20.2

No additional description.

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