Primary myelofibrosis: Difference between revisions
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{{Infobox medical condition | |||
| name = Primary myelofibrosis | |||
| synonyms = Chronic idiopathic myelofibrosis, agnogenic myeloid metaplasia | |||
| field = [[Hematology]] | |||
| symptoms = [[Fatigue (medical)|Fatigue]], [[splenomegaly]], [[anemia]], [[night sweats]], [[fever]], [[weight loss]] | |||
| complications = [[Acute myeloid leukemia]], [[portal hypertension]], [[thrombosis]] | |||
| onset = Typically in adults over 50 | |||
| duration = Chronic | |||
| causes = [[JAK2 mutation]], [[CALR mutation]], [[MPL mutation]] | |||
| risks = [[Age]], [[genetic predisposition]] | |||
| diagnosis = [[Bone marrow biopsy]], [[complete blood count]], [[genetic testing]] | |||
| differential = [[Polycythemia vera]], [[essential thrombocythemia]], [[chronic myeloid leukemia]] | |||
| treatment = [[JAK inhibitors]], [[hydroxyurea]], [[blood transfusion]], [[stem cell transplantation]] | |||
| prognosis = Variable, depends on risk factors | |||
| frequency = Rare | |||
}} | |||
'''Primary myelofibrosis''' (PMF) is a rare type of [[bone marrow]] cancer that disrupts the body's normal production of [[blood cells]]. It is classified as a [[myeloproliferative neoplasm]] (MPN), a group of diseases that cause an abnormal increase in the number of blood cells. PMF is characterized by the replacement of bone marrow with fibrous scar tissue, leading to severe [[anemia]], weakness, fatigue, and an enlarged [[spleen]] (splenomegaly). | '''Primary myelofibrosis''' (PMF) is a rare type of [[bone marrow]] cancer that disrupts the body's normal production of [[blood cells]]. It is classified as a [[myeloproliferative neoplasm]] (MPN), a group of diseases that cause an abnormal increase in the number of blood cells. PMF is characterized by the replacement of bone marrow with fibrous scar tissue, leading to severe [[anemia]], weakness, fatigue, and an enlarged [[spleen]] (splenomegaly). | ||
== Pathophysiology == | == Pathophysiology == | ||
The exact cause of primary myelofibrosis is not well understood. However, it is associated with mutations in the [[JAK2]], [[CALR]], and [[MPL]] genes. These mutations lead to the abnormal proliferation of [[hematopoietic stem cells]], which results in the overproduction of certain blood cells and the release of cytokines that promote fibrosis in the bone marrow. | The exact cause of primary myelofibrosis is not well understood. However, it is associated with mutations in the [[JAK2]], [[CALR]], and [[MPL]] genes. These mutations lead to the abnormal proliferation of [[hematopoietic stem cells]], which results in the overproduction of certain blood cells and the release of cytokines that promote fibrosis in the bone marrow. | ||
== Symptoms == | == Symptoms == | ||
Common symptoms of primary myelofibrosis include: | Common symptoms of primary myelofibrosis include: | ||
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* Night sweats | * Night sweats | ||
* Bone pain | * Bone pain | ||
== Diagnosis == | == Diagnosis == | ||
Diagnosis of primary myelofibrosis typically involves a combination of: | Diagnosis of primary myelofibrosis typically involves a combination of: | ||
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* Genetic testing for mutations in the JAK2, CALR, and MPL genes | * Genetic testing for mutations in the JAK2, CALR, and MPL genes | ||
* Imaging studies such as [[ultrasound]] or [[MRI]] to assess spleen and liver size | * Imaging studies such as [[ultrasound]] or [[MRI]] to assess spleen and liver size | ||
== Treatment == | == Treatment == | ||
Treatment options for primary myelofibrosis depend on the severity of the disease and the symptoms. They may include: | Treatment options for primary myelofibrosis depend on the severity of the disease and the symptoms. They may include: | ||
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* [[Stem cell transplantation]] | * [[Stem cell transplantation]] | ||
* Supportive care to manage symptoms | * Supportive care to manage symptoms | ||
== Prognosis == | == Prognosis == | ||
The prognosis for primary myelofibrosis varies widely among patients. Factors influencing prognosis include the patient's age, overall health, and specific genetic mutations. Some patients may live many years with the disease, while others may experience a more rapid progression. | The prognosis for primary myelofibrosis varies widely among patients. Factors influencing prognosis include the patient's age, overall health, and specific genetic mutations. Some patients may live many years with the disease, while others may experience a more rapid progression. | ||
== Related Pages == | == Related Pages == | ||
* [[Myeloproliferative neoplasm]] | * [[Myeloproliferative neoplasm]] | ||
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* [[Splenomegaly]] | * [[Splenomegaly]] | ||
* [[Stem cell transplantation]] | * [[Stem cell transplantation]] | ||
== References == | == References == | ||
{{Reflist}} | {{Reflist}} | ||
== External Links == | == External Links == | ||
{{Commons category|Primary myelofibrosis}} | {{Commons category|Primary myelofibrosis}} | ||
[[Category:Myeloproliferative disorders]] | [[Category:Myeloproliferative disorders]] | ||
[[Category:Hematology]] | [[Category:Hematology]] | ||
[[Category:Rare diseases]] | [[Category:Rare diseases]] | ||
[[Category:Oncology]] | [[Category:Oncology]] | ||
{{medicine-stub}} | {{medicine-stub}} | ||
{{No image}} | |||
Latest revision as of 01:03, 4 April 2025
| Primary myelofibrosis | |
|---|---|
| Synonyms | Chronic idiopathic myelofibrosis, agnogenic myeloid metaplasia |
| Pronounce | N/A |
| Specialty | N/A |
| Symptoms | Fatigue, splenomegaly, anemia, night sweats, fever, weight loss |
| Complications | Acute myeloid leukemia, portal hypertension, thrombosis |
| Onset | Typically in adults over 50 |
| Duration | Chronic |
| Types | N/A |
| Causes | JAK2 mutation, CALR mutation, MPL mutation |
| Risks | Age, genetic predisposition |
| Diagnosis | Bone marrow biopsy, complete blood count, genetic testing |
| Differential diagnosis | Polycythemia vera, essential thrombocythemia, chronic myeloid leukemia |
| Prevention | N/A |
| Treatment | JAK inhibitors, hydroxyurea, blood transfusion, stem cell transplantation |
| Medication | N/A |
| Prognosis | Variable, depends on risk factors |
| Frequency | Rare |
| Deaths | N/A |
Primary myelofibrosis (PMF) is a rare type of bone marrow cancer that disrupts the body's normal production of blood cells. It is classified as a myeloproliferative neoplasm (MPN), a group of diseases that cause an abnormal increase in the number of blood cells. PMF is characterized by the replacement of bone marrow with fibrous scar tissue, leading to severe anemia, weakness, fatigue, and an enlarged spleen (splenomegaly).
Pathophysiology[edit]
The exact cause of primary myelofibrosis is not well understood. However, it is associated with mutations in the JAK2, CALR, and MPL genes. These mutations lead to the abnormal proliferation of hematopoietic stem cells, which results in the overproduction of certain blood cells and the release of cytokines that promote fibrosis in the bone marrow.
Symptoms[edit]
Common symptoms of primary myelofibrosis include:
- Severe anemia
- Fatigue
- Weakness
- Splenomegaly
- Hepatomegaly (enlarged liver)
- Easy bruising and bleeding
- Night sweats
- Bone pain
Diagnosis[edit]
Diagnosis of primary myelofibrosis typically involves a combination of:
- Complete blood count (CBC)
- Bone marrow biopsy
- Genetic testing for mutations in the JAK2, CALR, and MPL genes
- Imaging studies such as ultrasound or MRI to assess spleen and liver size
Treatment[edit]
Treatment options for primary myelofibrosis depend on the severity of the disease and the symptoms. They may include:
- JAK inhibitors (e.g., ruxolitinib)
- Hydroxyurea
- Blood transfusions
- Stem cell transplantation
- Supportive care to manage symptoms
Prognosis[edit]
The prognosis for primary myelofibrosis varies widely among patients. Factors influencing prognosis include the patient's age, overall health, and specific genetic mutations. Some patients may live many years with the disease, while others may experience a more rapid progression.
Related Pages[edit]
References[edit]
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External Links[edit]
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