Indonesian Journal of Clinical Pathology and Medical Laboratory
ISSN 0854-4263
Vol. 17 / No. 2 / Published : 2017-11
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Original Article :
Pathogenesis and laboratory examination of primary myelofibrosis
Author :
- johanis*1
- Arifoel Hajat*2
- Mahasiswa Fakultas Kedokteran
- Dosen Fakultas Kedokteran
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-ABL-negative 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.
Keyword :
Phatogenesis, primary myelofibrosis, laboratory examination,
References :
Ciesla B,(2007) Hematology in Practice Washington DC : FA. Davis Company
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