Reactive process common to many malignant and benign disorders.
Primary myelofibrosis is a chronic progressive disorder with a median lifespan (5.5 years) much shorter than that with other myeloproliferative disorders. However, survival can be heterogeneous, ranging from <1 year to >30 years.
Leukoerythroblastosis and splenomegaly are common findings.
Death is usually due to bone marrow failure (haemorrhage, anaemia, or infection), transformation to acute leukaemia, portal or pulmonary HTN, heart failure, cachexia, or myeloid metaplasia with organ failure.
Asymptomatic, low-risk patients without hyperuricaemia or a remedial cause for anaemia require no therapy.
Haematopoietic stem cell transplant is the only treatment option with a potential for cure.
Myelofibrosis is a reactive process common to many malignant and benign disorders. Primary myelofibrosis (PMF) is a chronic myeloproliferative disorder of unknown aetiology. It involves a multipotent haematopoietic progenitor cell, but lacking a specific clonal marker, which results in abnormalities in RBC, WBC, and platelet production in association with marrow fibrosis and extramedullary haematopoiesis.  Leukoerythroblastosis and splenomegaly are the clinical hallmarks of PMF. When fibrosis (scarring) in the bone marrow is due to a known diagnosis such as leukaemia, hypoparathyroidism, or drugs, it is called secondary or reactive myelofibrosis.
Professor of Medicine and Oncology
Division of Hematology
Johns Hopkins University School of Medicine
JLS is an author of a reference cited in this monograph and is a consultant for Incyte.
Professor Jerry Spivak would like to gratefully acknowledge Dr Ashkan Emadi, a previous contributor to this monograph. AE declares that he has no competing interests.
Professor and Consultant in Haematology
Royal Hallamshire Hospital
JTR is an author of a number of references cited in this monograph.
Director of the Laboratory of Clinical Epidemiology
IRCCS Policlinico S. Matteo Foundation
GB declares that he has no competing interests.
Myeloproliferative Disorders Program Specialist
Department of Medicine
Division of Hematology and Medical Oncology
Weill Cornell Medical College
RS is an author of a reference cited in this monograph.
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