PATOGENESIS dan PEMERIKSAAN LABORATOPRIUM MIELOFIBROSIS PRIMER

Abstract

Primary myelofibrosis (PMF) is a neoplastic hematologic disease, characterized by clonal hemapoietic stem cell and collagenaccumulation in bone marrow. PMF is not related with underlying myeloproliferative disorders or other diseases. The features of PMFshow marrow fibrosis, megakaryocytes and granulocytes proliferation, and extramedullary hemapoiesis. PMF is classified as BCR-ABLnegativemyeloproliferative disorders. Diagnosis of PMF is based on clinical symptoms of splenomegaly and myelopthisis; bone marrowbiopsy shows granulocytes/megakaryocytes hyperplasia, megakaryocytes dysmorphic and fibrosis; cytogenic testing for mutation ofJAK2V617F+, MPLW5I5L/K+ and BCR-ABL. The diagnostic criterion according to the 2008 WHO classification considers major andminor crieria. The primary purpose for treatment is to improve the quality of life by paliative tratment. The five year survival of PMFpatients is low.

Primary Myelophybrosis: Recognizing Oncet to Complications

“…Primary myelofibrosis (PMF) is a haematological malignancy in the form of a hemopoitic clonal disorder of stem cells accompanied by excessive accumulation of collagen in the bone marrow (Johanis & Hajat, 2011). As a result of fibrosis that occurs in the bone marrow, it causes damage to healthy bone marrow (Garmezy, Schaefer, Mercer, & Talpaz, 2020).…”

“…It can also cause a low number of blood clotting cells called platelets, which increases the risk of bleeding. In addition, myelofibrosis also often causes enlargement of the spleen (Johanis & Hajat, 2011).…”

“…Together with polycythemia vera and essential thrombocythemia, myelofibrosis is classified as a chronic hematological cancer (Grinfeld et al, 2018). Myeloproliferative disorders, myelofibrosis itself is also reclassified into two, namely primary myelofibrosis and secondary myelofibrosis (Johanis & Hajat, 2011).…”

“…Primary myelofibrosis is myelofibrosis that occurs without being preceded by myeloproliferative disorders or other diseases (Johanis & Hajat, 2011). In the pathogenesis of primary myelofibrosis, increased expression of inflammatory cytokines, lysyl oxidase, transforming growth factor-β, impaired megakaryocyte function, and aberrant JAK-STAT signaling are major factors in the pathogenesis of bone marrow fibrosis (Zahr et al, 2016).…”

“…For the incidence of myelofibrosis cases, based on data from a meta-analysis of studies conducted on populations in Europe, it was found that primary myelofibrosis cases were more common in males (range = 0.32 per 100,000 per year to 0.9 per 100,000 per year) than women (range = 0.32 per 100,000 per year to 0.9 per 100,000 per year) range = 0.2 per 100,000 per year to 0.7 per 100,000 per year (Moulard et al, 2013).Clinical manifestations found in patients with primary myelofibrosis include severe anemia, hepatosplenomegaly, constitutional symptoms (eg fatigue, night sweats). days, fever), cachexia, bone pain, splenic infarction, pruritus, thrombosis, and bleeding (Johanis & Hajat, 2011).…”