what is Myelofibrosis?

Question

the disease and treatments and prognosis

Answer 1

Myelofibrosis, one of the myeloproliferative diseases, is the gradual replacement of the bone marrow by connective tissue.

The replacement of the bone marrow tissue reduces the patient's ability to generate new blood cells resulting in chronic anemia. A prime feature is "extramedullary hematopoeisis", i.e. the remaining blood-forming cells migrate to other sites in the body, e.g. the liver or spleen. Patients will typically have an enlarged spleen and liver, (hepatosplenomegaly), and examination of the blood cells will show "teardrop cells".

In order to diagnose myelofibrosis, the following criteria must be fulfilled: (1) no evidence of other myeloproliferative disorder (2) no evidence of secondary bone marrow fibrosis (3) leucoerythroblastic picture on blood film (4) presence of splenomegaly (5) increase fibrotic process in the bone marrow.

Typically affecting patients more than 50 years old, it is a chronic and debilitating condition. Currently the only known cure is a bone marrow transplant. However the disease moves very slowly and patients can live for more than five or ten and even as long as eighteen more years after diagnososis, (with treatment). Since the bone marrow replacement treatment is very rigorous, painful and high risk, involving the use of chemotherapy to destroy the patient's existing marrow tissue, and the typical patient is older, the bone marrow treatment is often not used.

There are other treatments available to reduce the effects of the disease, which are repeated at regular intervals to maintain quality of life for the patient

Answer 2

'''Myelofibrosis''' is the gradual replacement of the bone marrow by connective tissue. Its main feature is "extramedullary hematopoeisis", i.e. the blood-forming cells migrate to other sites in the body, e.g. the liver or spleen. Typically affects patients > 50 years. Patients will typically have hepatosplenomegaly, and the blood smear will show "teardrop cells".

OR

Myelofibrosis (also called agnogenic myeloid metaplasia) is a myeloproliferative disorder in which the bone marrow is initially over-active but then develops scar tissue (fibrosis). The term idiopathic means without known cause and differentiates this form of myelofibrosis from secondary myelofibrosis which may complicate other bone marrow diseases. Normal bone marrow has a very fine network of fibres supporting the blood forming tissues. In myelofibrosis this network is coarsened and thickened so that normal blood cell production is progressively reduced. As a result blood cell production begins to take place in the liver and spleen which become enlarged. These are both tissues which produce blood cells in the embryo but lose this function before birth. The production of blood cells in the liver and spleen is less efficient and so patients frequently develop anaemia.


OR

It is a disease of the bone marrow in which collagen builds up fibrous scar tissue inside the marrow cavity. The trigger for its development is a disturbance of the immune system. It may be called by several names including primary or ideopathic myelofibrosis (more common in the British health system) or agnogenic myeloid metaplasia (usually used in the USA). These names describe a situation where myelofibrosis is the first disease diagnosed. It is also possible to have myelofibrosis as a consequence of another myeloproliferative diseases such as polycythemia vera (PV) or essential thrombocythemia (ET).

The outcome is that the blood made by the marrow is of poor quality. The red blood cells are tear drop shaped, large and sluggish and the white blood cells are overabundant. This poor quality blood triggers a response from the spleen and liver as they try to compensate including creating blood themselves (usually only done by the foetus). If left untreated the person eventually will have marrow failure which leads to death. How is the diagnosis made?

Commonly the patient will attend a doctor about something else and may mention that they feel more tired than usual or that their abdomen in tender and enlarged or bloated (this comes from the enlarged spleen). An alert doctor will request a full blood screen which will identify that some elements of the blood counts are abnormal.

To be sure of the diagnosis a bone marrow biopsy is required to test the state of the marrow itself and provide conclusive evidence of the disorder. As well, an enlarged spleen and/or liver is also evidence of a myeloproliferative disorder.


symptoms:
Many people have no obvious symptoms when they are diagnosed and the process of accepting the diagnosis can be difficult. Others experience the tiredness, diarrhea and other symptoms and put it down to stress, menopause or old age. In the early stages the symptoms are not usually strongly obvious. Later they may include: painful abdomen (from enlarged spleen and liver), fluid retention in the abdomen; disturbances in vision (like spots or blurring of the eyes); joint pain; bone pain (from marrow expanding into the long bones; boils orother skin eruptions (like blood blisters just under the skin).


There is a huge amount of material available on the Internet about treatments and case reports and so on. As a rare disease there is not much research specifically on myelofibrosis.

Use the web page for suggestions of how and where to search; or just ask a question of the group. You might also want to search the mpd-net discussion group archives which are located at http://medinfo.org.

Answer 3

Myelofibrosis is a rare disease of the bone marrow in which collagen builds up fibrous scar tissue inside the marrow cavity. This is caused by the uncontrolled growth of a blood cell precursor, which results in the accumulation of scar tissue in bone marrow. Myelofibrosis goes by many names including idiopathic myelofibrosis, agnogenic myeloid metaplasia, chronic myelosclerosis, aleukemic megakaryocytic myelosis, and leukoerythroblastosis.

Answer 4

What Is Myelofibrosis

Answer 5

Primary myelofibrosis is a disorder of the bone marrow

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Answer 6

Proliferation of fibroblastic cells in bone marrow, causing anemia and sometimes enlargement of the spleen and liver.

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what is Myelofibrosis?
the disease and treatments and prognosis