Idiopathic myelofibrosis; Myeloid metaplasia; Agnogenic myeloid metaplasia; Primary myelofibrosis; Secondary myelofibrosis; Bone marrow - myelofibrosis DefinitionMyelofibrosis is a disorder of the bone marrow in which the marrow is replaced by fibrous scar tissue. CausesBone marrow is the soft, fatty tissue inside your bones. Stem cells are immature cells in the bone marrow that develop into all of your blood cells. Your blood is made of:
When the bone marrow is scarred, it cannot make enough blood cells. Anemia, bleeding problems, and a higher risk for infections may occur. As a result, the liver and spleen try to make some of these blood cells. This causes these organs to swell. The cause of myelofibrosis is often unknown. There are no known risk factors. When it occurs, it often develops slowly in people over age 50. Women and men are equally affected. There is an increased occurrence of this condition in Ashkenazi Jews. Blood and bone marrow cancers, such as myelodysplastic syndrome, leukemia, and lymphoma, may also cause bone marrow scarring. This is called secondary myelofibrosis. SymptomsSymptoms may include any of the following:
Exams and TestsThe health care provider will perform a physical exam and ask about the symptoms. Tests that may be done include:
TreatmentBone marrow or stem cell transplant may improve symptoms, and may cure the disease. This treatment is usually considered for younger people. Other treatment may involve:
Outlook (Prognosis)As the disease worsens, the bone marrow slowly stops working. Low platelet count leads to easy bleeding. Spleen swelling may get worse along with anemia. Survival of people with primary myelofibrosis is about 5 years. But some people survive for decades. Possible ComplicationsComplications may include:
When to Contact a Medical ProfessionalMake an appointment with your provider if you have symptoms of this disorder. Seek medical care right away for uncontrolled bleeding, shortness of breath, or jaundice (yellow skin and whites of the eyes) that gets worse. ReferencesGotlib J. Polycythemia vera, essential thrombocythemia, and primary myelofibrosis. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 26th ed. Philadelphia, PA: Elsevier; 2020:chap 157. Long NM, Kavanagh EC. Myelofibrosis. In: Pope TL, Bloem HL, Beltran J, Morrison WB, Wilson DJ, eds. Musculoskeletal Imaging. 2nd ed. Philadelphia, PA: Elsevier Saunders; 2015:chap 76. Mascarenhas J, Najfeld V, Kremyanskaya M, Keyzner A, Salama ME, Hoffman R. Primary myelofibrosis. In: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Basic Principles and Practice. 7th ed. Philadelphia, PA: Elsevier; 2018:chap 70. Sive J, Foggo V. Haematological oncology. In: Feather A, Randall D, Waterhouse M, eds. Kumar and Clark's Clinical Medicine. 10th ed. Philadelphia, PA: Elsevier; 2021:chap 17. | |
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Review Date: 4/29/2022 Reviewed By: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. No warranty of any kind, either expressed or implied, is made as to the accuracy, reliability, timeliness, or correctness of any translations made by a third-party service of the information provided herein into any other language. © 1997- A.D.A.M., a business unit of Ebix, Inc. Any duplication or distribution of the information contained herein is strictly prohibited. | |
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