PATOGENESIS dan PEMERIKSAAN LABORATOPRIUM MIELOFIBROSIS PRIMER

Abstract

Primary myelofibrosis (PMF) is a neoplastic hematologic disease, characterized by clonal hemapoietic stem cell and collagen
accumulation in bone marrow. PMF is not related with underlying myeloproliferative disorders or other diseases. The features of PMF
show marrow fibrosis, megakaryocytes and granulocytes proliferation, and extramedullary hemapoiesis. PMF is classified as BCR-ABLnegative
myeloproliferative disorders. Diagnosis of PMF is based on clinical symptoms of splenomegaly and myelopthisis; bone marrow
biopsy shows granulocytes/megakaryocytes hyperplasia, megakaryocytes dysmorphic and fibrosis; cytogenic testing for mutation of
JAK2V617F+, MPLW5I5L/K+ and BCR-ABL. The diagnostic criterion according to the 2008 WHO classification considers major and
minor crieria. The primary purpose for treatment is to improve the quality of life by paliative tratment. The five year survival of PMF
patients is low.