Table of ContentsView AllTable of ContentsTypesSymptomsCausesDiagnosisTreatmentCoping
Table of ContentsView All
View All
Table of Contents
Types
Symptoms
Causes
Diagnosis
Treatment
Coping
The symptoms, causes, and treatments depend on the type and severity of the disorder. Diagnosis of each condition involves a similar process, and usually includes a blood test, and possibly abone marrow biopsy. This article will help you understand your risks and what to expect if you have a myeloproliferative disorder.
Verywell / Jessica Olah
Types of Myeloproliferative Disorders
Sometimes myeloproliferative disorders are described as myeloproliferative neoplasms.These conditions are rare, and they typically occur during adulthood, although they can occur during childhood as well.
The types of myeloproliferative disorders include:
These conditions can cause similar symptoms, including fevers and fatigue, although each has its own set of effects and complications. They each have a separate cause, and while there are some similar treatments, they each are also treated with specific therapies.
Myeloproliferative Disorder Symptoms
Often, myeloproliferative disorders cause generalized weakness and persistent tiredness. They might not cause symptoms at early stages, and the obvious effects can begin during late stages of the disease.
The specific symptoms of each type of myeloproliferative disorder are:
You can also be susceptible to getting infections or anemia (low red blood cells), as well as other symptoms with myeloproliferative disorders.
These conditions are associated with noninheritable genetic abnormalities that affect blood cell production. The genetic changes may occur due to environmental influences, but they generally have no specific identifiable cause. Myeloproliferative disorders can also develop without the presence of any specific genetic abnormality.
Smoking is associated with a higher-than-average risk of developing a myeloproliferative disorder, and obesity is associated with a slightly increased risk, but these conditions can develop without any risk factors.Polycythemia may also be secondary to heart problems or some forms of cancer. Secondary polycythemia differs from polycythemia vera.
Sometimes myeloproliferative disorders are diagnosed due to an abnormal number of blood cells noted on a blood test done for another reason. These conditions may also be diagnosed during a symptom evaluation.
Tests that are used to diagnose myeloproliferative disorders include:
The results of these tests differ, depending on which type of myeloproliferative disorder you have. With polycythemia vera, a CBC would show an increasedhematocrit, which is a measure of red blood cells. With essential thrombocythemia, a CBC would show a high platelet count, and a blood smear may show clumps of platelets.
Myelofibrosis may be diagnosed with a bone marrow biopsy. Chronic myeloid leukemia, chronic eosinophilic leukemia, and chronic neutrophilic leukemia can show abnormal numbers of specific white blood cells on a bone marrow aspiration or biopsy.
If you have a myeloproliferative disorder, it’s important to get treatment to manage your symptoms and prevent your condition from progressing.
Chemotherapy can prevent excess cell production, and the medications are sometimes used to treat myeloproliferative disorders. Each disorder is treated with different chemotherapy, as well as specific medication or other therapy to manage the complications.
For example, hydroxyurea is sometimes used totreat polycythemia veraor essential thrombocytopenia.Interferons, which are immunostimulants, affect the immune system, are sometimes used to treat the different types of chronic leukemia and other myeloproliferative disorders.
Jakafi (ruxolitinib), a tyrosine kinase inhibitor, is used to treat polycythemia vera and myelofibrosis. Polycythemia vera can also be treated with medication to reduce the number of red blood cells, and sometimes removal of blood (therapeutic phlebotomy) is necessary.
Vonjo (pacritinib) is a multikinase inhibitor used to treat people with a rare form of a bone marrow disorder known as intermediate- or high-risk primary or secondary myelofibrosis. Vonjo has the distinction of being the first approved therapy to specifically address the needs of people with cytopenic myelofibrosis (people with a platelet count below 50 x 109/L).
Myelofibrosis and chronic myeloid leukemia are sometimes treated with a bone marrow transplant.
What to Expect From a Bone Marrow Transplant
If you have a myeloproliferative disorder, you may need lifelong medical care. This can involve surveillance of your symptoms, diagnostic tests, and possibly treatment.
You may need to be cautious about protecting yourself from infections and injuries. This can involve lifestyle adjustments, such as staying away from anyone you know is sick or avoiding activities such as contact sports.
These conditions can be slowly progressive, and they may sometimes increase your risk of cancer. If you need treatment for your myeloproliferative disorder, you might also have side effects of chemotherapy. These side effects should resolve after your treatment course is complete.
Summary
Myeloproliferative disorders cause excess production of red or white blood cells or platelets. They are chronic and slowly get worse over time.
The different types of myeloproliferative disorders depend on which cells are overproduced. These conditions include chronic myelogenous leukemia, polycythemia vera, primary myelofibrosis, essential thrombocythemia, chronic neutrophilic leukemia, and chronic eosinophilic leukemia.
Symptoms vary, but often they are nonspecific and the condition is sometimes caught during a routine blood test, such as a CBC. A bone marrow biopsy is usually needed to diagnose these conditions. Treatment varies depending on the type of disorder, and may include chemotherapy.
12 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Allahverdi N, Yassin M, Ibrahim M.Environmental factors, lifestyle risk factors, and host characteristics associated with Philadelphia negative myeloproliferative neoplasm: A systematic review.Cancer Control.2021 Jan-Dec;28:10732748211046802. doi:10.1177/10732748211046802El-Sharkawy F, Margolskee E.Pediatric myeloproliferative neoplasms.Clin Lab Med. 2021 Sep;41(3):529-540. doi:10.1016/j.cll.2021.04.010National Cancer Institute.Myeloproliferative neoplasms.National Heart, Lung, and Blood Institute.Polycythemia vera.National Heart, Lung, and Blood Institute.Thrombocythemia and thrombocytosis.Greenberg PL, Attar E, Bennett JM, et al.Myelodysplastic syndromes: clinical practice guidelines in oncology.JNCCN.2013;11(7):838-74. doi:10.6004/jnccn.2013.0104American Cancer Society.Signs and symptoms of chronic myeloid leukemia. Updated June 19, 2018.Canadian Cancer Society.Chronic eosinophilic leukemia.Szuber N, Elliott M, Tefferi A.Chronic neutrophilic leukemia: 2020 update on diagnosis, molecular genetics, prognosis, and management.Am J Hematol. 2020 Feb;95(2):212-224. doi:10.1002/ajh.25688Healy FM, Dahal LN, Jones JRE, Floisand Y, Woolley JF.Recent progress in Interferon therapy for myeloid malignancies.Front Oncol.2021 Oct 29;11:769628. doi:10.3389/fonc.2021.769628Ravid K, Karagianni A.Myeloproliferative disorders and its effect on bone homeostasis: The role of megakaryocytes.Blood.2021 Aug 24:blood.2021011480. doi:10.1182/blood.2021011480CTI BioPharma Corp.VONJO (pacritinib).
12 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Allahverdi N, Yassin M, Ibrahim M.Environmental factors, lifestyle risk factors, and host characteristics associated with Philadelphia negative myeloproliferative neoplasm: A systematic review.Cancer Control.2021 Jan-Dec;28:10732748211046802. doi:10.1177/10732748211046802El-Sharkawy F, Margolskee E.Pediatric myeloproliferative neoplasms.Clin Lab Med. 2021 Sep;41(3):529-540. doi:10.1016/j.cll.2021.04.010National Cancer Institute.Myeloproliferative neoplasms.National Heart, Lung, and Blood Institute.Polycythemia vera.National Heart, Lung, and Blood Institute.Thrombocythemia and thrombocytosis.Greenberg PL, Attar E, Bennett JM, et al.Myelodysplastic syndromes: clinical practice guidelines in oncology.JNCCN.2013;11(7):838-74. doi:10.6004/jnccn.2013.0104American Cancer Society.Signs and symptoms of chronic myeloid leukemia. Updated June 19, 2018.Canadian Cancer Society.Chronic eosinophilic leukemia.Szuber N, Elliott M, Tefferi A.Chronic neutrophilic leukemia: 2020 update on diagnosis, molecular genetics, prognosis, and management.Am J Hematol. 2020 Feb;95(2):212-224. doi:10.1002/ajh.25688Healy FM, Dahal LN, Jones JRE, Floisand Y, Woolley JF.Recent progress in Interferon therapy for myeloid malignancies.Front Oncol.2021 Oct 29;11:769628. doi:10.3389/fonc.2021.769628Ravid K, Karagianni A.Myeloproliferative disorders and its effect on bone homeostasis: The role of megakaryocytes.Blood.2021 Aug 24:blood.2021011480. doi:10.1182/blood.2021011480CTI BioPharma Corp.VONJO (pacritinib).
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
Allahverdi N, Yassin M, Ibrahim M.Environmental factors, lifestyle risk factors, and host characteristics associated with Philadelphia negative myeloproliferative neoplasm: A systematic review.Cancer Control.2021 Jan-Dec;28:10732748211046802. doi:10.1177/10732748211046802El-Sharkawy F, Margolskee E.Pediatric myeloproliferative neoplasms.Clin Lab Med. 2021 Sep;41(3):529-540. doi:10.1016/j.cll.2021.04.010National Cancer Institute.Myeloproliferative neoplasms.National Heart, Lung, and Blood Institute.Polycythemia vera.National Heart, Lung, and Blood Institute.Thrombocythemia and thrombocytosis.Greenberg PL, Attar E, Bennett JM, et al.Myelodysplastic syndromes: clinical practice guidelines in oncology.JNCCN.2013;11(7):838-74. doi:10.6004/jnccn.2013.0104American Cancer Society.Signs and symptoms of chronic myeloid leukemia. Updated June 19, 2018.Canadian Cancer Society.Chronic eosinophilic leukemia.Szuber N, Elliott M, Tefferi A.Chronic neutrophilic leukemia: 2020 update on diagnosis, molecular genetics, prognosis, and management.Am J Hematol. 2020 Feb;95(2):212-224. doi:10.1002/ajh.25688Healy FM, Dahal LN, Jones JRE, Floisand Y, Woolley JF.Recent progress in Interferon therapy for myeloid malignancies.Front Oncol.2021 Oct 29;11:769628. doi:10.3389/fonc.2021.769628Ravid K, Karagianni A.Myeloproliferative disorders and its effect on bone homeostasis: The role of megakaryocytes.Blood.2021 Aug 24:blood.2021011480. doi:10.1182/blood.2021011480CTI BioPharma Corp.VONJO (pacritinib).
Allahverdi N, Yassin M, Ibrahim M.Environmental factors, lifestyle risk factors, and host characteristics associated with Philadelphia negative myeloproliferative neoplasm: A systematic review.Cancer Control.2021 Jan-Dec;28:10732748211046802. doi:10.1177/10732748211046802
El-Sharkawy F, Margolskee E.Pediatric myeloproliferative neoplasms.Clin Lab Med. 2021 Sep;41(3):529-540. doi:10.1016/j.cll.2021.04.010
National Cancer Institute.Myeloproliferative neoplasms.
National Heart, Lung, and Blood Institute.Polycythemia vera.
National Heart, Lung, and Blood Institute.Thrombocythemia and thrombocytosis.
Greenberg PL, Attar E, Bennett JM, et al.Myelodysplastic syndromes: clinical practice guidelines in oncology.JNCCN.2013;11(7):838-74. doi:10.6004/jnccn.2013.0104
American Cancer Society.Signs and symptoms of chronic myeloid leukemia. Updated June 19, 2018.
Canadian Cancer Society.Chronic eosinophilic leukemia.
Szuber N, Elliott M, Tefferi A.Chronic neutrophilic leukemia: 2020 update on diagnosis, molecular genetics, prognosis, and management.Am J Hematol. 2020 Feb;95(2):212-224. doi:10.1002/ajh.25688
Healy FM, Dahal LN, Jones JRE, Floisand Y, Woolley JF.Recent progress in Interferon therapy for myeloid malignancies.Front Oncol.2021 Oct 29;11:769628. doi:10.3389/fonc.2021.769628
Ravid K, Karagianni A.Myeloproliferative disorders and its effect on bone homeostasis: The role of megakaryocytes.Blood.2021 Aug 24:blood.2021011480. doi:10.1182/blood.2021011480
CTI BioPharma Corp.VONJO (pacritinib).
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