Myelofibrosis (patient information): Difference between revisions
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{{Myelofibrosis (patient information)}} | {{Myelofibrosis (patient information)}} | ||
{{CMG}}; {{AOEIC}} {{LG}} | {{CMG}}; {{AOEIC}}{{Sab}} {{LG}} | ||
{{SK}} Idiopathic myelofibrosis; Myeloid metaplasia; Agnogenic myeloid metaplasia; Primary myelofibrosis; Secondary myelofibrosis | {{SK}} Idiopathic myelofibrosis; Myeloid metaplasia; Agnogenic myeloid metaplasia; Primary myelofibrosis; Secondary myelofibrosis | ||
==Overview== | ==Overview== | ||
Myelofibrosis is a disorder of the bone marrow, in which the marrow is replaced by scar (fibrous) tissue. | [[Myelofibrosis]] is a disorder of the [[bone marrow]], in which the [[Bone marrow|marrow]] is replaced by [[Scar tissue|scar (fibrous) tissue]]. | ||
==What are the symptoms of Myelofibrosis?== | ==What are the symptoms of Myelofibrosis?== | ||
*Abdominal fullness related to an enlarged spleen | *[[Abdomen|Abdominal]] fullness related to an enlarged [[spleen]] | ||
*Bone pain | *[[Bone pain]] | ||
*Bruising | *[[Bruise|Bruising]] | ||
*Easy bleeding | *Easy [[bleeding]] | ||
*Fatigue | *[[Fatigue]] | ||
*Increased likelihood of getting an infection | *Increased likelihood of getting an [[infection]] | ||
*Pallor | *[[Pallor]] | ||
*Shortness of breath with exercise | *Shortness of breath with [[Physical exercise|exercise]] | ||
*Weight loss | *[[Weight loss]] | ||
*Loss of appetite | *Loss of [[appetite]] | ||
*Night sweats | *Night sweats | ||
*Fever | *[[Fever]] | ||
*Early satiety | *[[Satiety|Early satiety]] | ||
*Bleeding | *[[Bleeding]] | ||
*Peripheral | *[[Pedal edema|Peripheral swelling]] | ||
*Frequent infections | *Frequent [[Infection|infections]] | ||
*Dry cough | *[[Cough|Dry cough]] | ||
*Skin nodules | *[[Skin nodules]] | ||
*Itchy skin | *[[Pruritis|Itchy skin]] | ||
==What causes Myelofibrosis?== | ==What causes Myelofibrosis?== | ||
*Bone marrow is the soft, fatty tissue inside your bones. Stem cells are immature cells in the bone marrow that give rise to all of your blood cells. Your blood is made of: | *[[Bone marrow]] is the soft, fatty [[Tissue (biology)|tissue]] inside your [[Bone|bones]]. [[Stem cell|Stem cells]] are immature [[Cell (biology)|cells]] in the [[bone marrow]] that give rise to all of your [[Blood cell|blood cells]]. Your [[blood]] is made up of: | ||
:*Red blood cells (which carry oxygen to your tissues) | :*[[Red blood cell|Red blood cells]] (which carry [[oxygen]] to your [[Tissue (biology)|tissues]]) | ||
:*White blood cells (which fight infection) | :*[[White blood cells]] (which fight [[infection]]) | ||
:*Platelets (which help your blood clot) | :*[[Platelet|Platelets]] (which help your [[blood]] [[Coagulation|clot]]) | ||
*Scarring of the bone marrow means the marrow is not able to make enough blood cells. Anemia, bleeding problems, and a higher risk of infections may | *[[Scar|Scarring]] of the [[bone marrow]] means the [[Bone marrow|marrow]] is not able to make enough [[Blood cell|blood cells]]. [[Anemia]], [[Bleeding diathesis|bleeding problems]], and a higher risk of [[Infection|infections]] may develop as a result. | ||
* | *The [[liver]] and [[spleen]] may try to compensate and make some of these [[Blood cell|blood cells]], a process called [[Extramedullary hematopoiesis|'extramedullary hematopoiesis']]. This causes these [[Organ (anatomy)|organs]] to [[Edema|swell]]. | ||
* | *The disorder usually develops slowly in people over age 50. | ||
*Diseases such as leukemia and lymphoma may also cause bone marrow scarring. This is called secondary myelofibrosis. | *Diseases such as [[leukemia]] and [[lymphoma]] may also cause [[bone marrow]] [[Scar|scarring]]. This is called [[Myelofibrosis|secondary myelofibrosis]]. | ||
==Diagnosis== | ==Diagnosis== | ||
*Physical examination shows swelling of the spleen. Later in the disease, it may also show an enlarged liver. | *[[Physical examination]] shows [[Edema|swelling]] of the [[spleen]]. Later in the [[disease]], it may also show an enlarged [[liver]]. | ||
*Tests that may be done include | *Tests that may be done, include [[CBC|CBC (complete blood count)]], [[Bone marrow examination|bone marrow biopsy]], and [[Genetic testing|genetic testing.]] | ||
*An examination of the blood shows teardrop-shaped red blood cells. Bone marrow biopsy may be done to rule out other causes of the symptoms. | *An examination of the [[blood]] shows teardrop-shaped [[Red blood cell|red blood cells]]. [[Bone marrow examination|Bone marrow biopsy]] may be done to rule out other causes of the [[Symptom|symptoms]]. | ||
==When to seek urgent medical care?== | ==When to seek urgent medical care?== | ||
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. | Call for an appointment with your health care provider if [[Symptom|symptoms]] of this disorder develop. Uncontrolled [[bleeding]], [[Dyspnea|shortness of breath]], or [[jaundice]] that gets worse, need urgent or emergency care. | ||
==Treatment options== | ==Treatment options== | ||
*There is no specific treatment for myelofibrosis. Treatment depends on the symptoms and degree of the low blood counts. | *There is no specific treatment for [[myelofibrosis]]. Treatment depends on the [[Symptom|symptoms]] and degree of the low [[blood cell]] counts. | ||
*The goal of treatment is to | *The goal of treatment is to alleviate the [[Symptom|symptoms]]. Treatment may involve: | ||
:*Blood transfusions to correct anemia | :*[[Blood transfusion|Blood transfusions]] to correct [[anemia]] | ||
:*Radiation and chemotherapy | :*[[Radiation therapy|Radiation]] and [[chemotherapy]] | ||
*: | :*[[Medication|Medications]] such as [[Recombinant Human Erythropoietin (rHuEpo): Lack of Effect on Response to ASA or Clopidogrel|recombinant erythropoietin]] or [[Androgen|androgens]] to help stimulate [[red blood cell]] production | ||
:*Splenectomy (removal of the spleen) if swelling causes symptoms, or to help with anemia | :*[[Splenectomy]] (removal of the spleen), if [[Edema|swelling]] causes [[Symptom|symptoms]], or to help with [[anemia]] | ||
*In young people, [[bone marrow]] or [[stem cell transplantation]] can improve the outlook, and may cure the [[disease]]. | |||
*In young people, bone marrow or stem cell | |||
==Where to find medical care for Myelofibrosis?== | ==Where to find medical care for Myelofibrosis?== | ||
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==What to expect (Outlook/Prognosis)?== | ==What to expect (Outlook/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. | This disorder causes slowly worsening [[Bone marrow suppression|bone marrow failure]] with severe [[anemia]]. [[Thrombocytopenia|Low platelet count]] leads to easy [[bleeding]]. [[Splenomegaly|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. | The average survival of people with [[Myelofibrosis|primary myelofibrosis]] is about 5 years. However, some people may survive for decades. | ||
==Possible complications== | ==Possible complications== | ||
*Acute | *[[Acute myeloid leukemia]] | ||
*Liver failure | *[[Hepatic failure|Liver failure]] | ||
*[[Bleeding]] | |||
*[[Thrombosis|Thrombotic events]] | |||
*[[Congestive heart failure|Heart failure]] | |||
*[[Splenic rupture]] | |||
*[[Pulmonary hypertension]] | |||
*[[Peritonitis]] | |||
*[[Spinal cord compression]] | |||
*[[Bowel obstruction|Intestinal obstruction]] | |||
*[[Kidney]] problems | |||
==Source== | ==Source== | ||
Latest revision as of 03:25, 2 January 2019
For the WikiDoc page on this topic, click here
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Myelofibrosis |
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Myelofibrosis On the Web |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief:Sabawoon Mirwais, M.B.B.S, M.D.[2] Lakshmi Gopalakrishnan, M.B.B.S. [3]
Synonyms and keywords: Idiopathic myelofibrosis; Myeloid metaplasia; Agnogenic myeloid metaplasia; Primary myelofibrosis; Secondary myelofibrosis
Overview
Myelofibrosis is a disorder of the bone marrow, in which the marrow is replaced by scar (fibrous) tissue.
What are the symptoms of Myelofibrosis?
- Abdominal fullness related to an enlarged spleen
- Bone pain
- Bruising
- Easy bleeding
- Fatigue
- Increased likelihood of getting an infection
- Pallor
- Shortness of breath with exercise
- Weight loss
- Loss of appetite
- Night sweats
- Fever
- Early satiety
- Bleeding
- Peripheral swelling
- Frequent infections
- Dry cough
- Skin nodules
- Itchy skin
What causes Myelofibrosis?
- Bone marrow is the soft, fatty tissue inside your bones. Stem cells are immature cells in the bone marrow that give rise to all of your blood cells. Your blood is made up of:
- Red blood cells (which carry oxygen to your tissues)
- White blood cells (which fight infection)
- Platelets (which help your blood clot)
- Scarring of the bone marrow means the marrow is not able to make enough blood cells. Anemia, bleeding problems, and a higher risk of infections may develop as a result.
- The liver and spleen may try to compensate and make some of these blood cells, a process called 'extramedullary hematopoiesis'. This causes these organs to swell.
- The disorder usually develops slowly in people over age 50.
- Diseases such as leukemia and lymphoma may also cause bone marrow scarring. This is called secondary myelofibrosis.
Diagnosis
- Physical examination shows swelling of the spleen. Later in the disease, it may also show an enlarged liver.
- Tests that may be done, include CBC (complete blood count), bone marrow biopsy, and genetic testing.
- An examination of the blood shows teardrop-shaped red blood cells. Bone marrow biopsy may be done to rule out other causes of the symptoms.
When to seek urgent medical care?
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.
Treatment options
- There is no specific treatment for myelofibrosis. Treatment depends on the symptoms and degree of the low blood cell counts.
- The goal of treatment is to alleviate the symptoms. Treatment may involve:
- Blood transfusions to correct anemia
- Radiation and chemotherapy
- Medications such as 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 transplantation can improve the outlook, and may cure the disease.
Where to find medical care for Myelofibrosis?
Directions to Hospitals Treating Myelofibrosis
Prevention of Myelofibrosis
There is no known prevention.
What to expect (Outlook/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.
Possible complications
- Acute myeloid leukemia
- Liver failure
- Bleeding
- Thrombotic events
- Heart failure
- Splenic rupture
- Pulmonary hypertension
- Peritonitis
- Spinal cord compression
- Intestinal obstruction
- Kidney problems