Primary myelofibrosis

Table of Contents

Alternative Names

Idiopathic myelofibrosis; Myeloid metaplasia; Agnogenic myeloid metaplasia; Primary myelofibrosis; Secondary myelofibrosis


Treatment

There is no specific treatment for myelofibrosis. Treatment depends on the symptoms and degree of the low blood counts.

The goal of treatment is to relieve symptoms. Treatment may involve:

  • Blood transfusions to correct anemia
  • Radiation and chemotherapy
  • Medicines called recombinant erythropoietin or androgens to help stimulate red blood cell production
  • Splenectomy (removal of the spleen) if swelling causes symptoms, or to help with anemia

In young people, bone marrow or stem cell transplants appear to improve the outlook, and may cure the disease.


Support Groups


Expectations (prognosis)

This disorder causes slowly worsening bone marrow failure with severe anemia. Low platelet count leads to easy bleeding. Spleen swelling may slowly get worse.

The average survival of people with primary myelofibrosis is about 5 years. However, some people may survive for decades.


Complications
  • Acute myelogenous leukemia
  • Liver failure

Calling your health care provider

Call for an appointment with your health care provider if symptoms of this disorder develop. Uncontrolled bleeding, shortness of breath, or jaundice that gets worse need urgent or emergency care.



Review Date: 06/02/2010
Reviewed By: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org)