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Первинний мієлофіброз

ORPHA:824· ICD-10 D47.4· Primary myelofibrosis

Визначення(English summary)

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.

Поширеність
1-9 / 100 000
Успадкування
Not applicable
Вік початку
Adult