Idiopathic myelofibrosis is a serious, but rare, bone marrow disease that disrupts your normal production of blood cells because of severe scarring, aka fibrosis, within your bone marrow. Classified as a chronic myeloproliferative disease, its root cause is a hematopoietic stem cell mutation, which then passes this mutation on to new cells. As mutated cells proliferate, they’ll cause an effect on the type of blood cells yielded.
Not believed to be congenital, myelofibrosis bone marrow disease typically results in the generation of too few red blood cells and an overabundance of white blood cells and blood platelets. Consequently causing severe anemia.
What causes this mutation is unknown, hence the health condition is referred to as ideopathic myelofibrosis. However, there may be an association to certain toxic chemicals and autoimmune diseases, such as:
- lupus
- toluene
- benzene
- scleroderma
- thorium dioxide
- ionizing radiation
Idiopathic myelofibrosis can come to life any age, yet this bone marrow disease is primarily seen in those over age 50.
Idiopathic myelofibrosis typically develops slowly, so you’ll likely experience only some vague anemic type symptoms for a while. Eventually, this bone marrow disease’s blood cell disruption may cause you some of these symptoms:
- fever
- pallor
- fatigue
- ascites
- pruritus
- hepatitis
- dyspnea
- weakness
- weight loss
- palpitations
- night sweats
- easy bruising
- severe anemia
- enlarged spleen
- frequent infections
- excessive bleeding
- unreasonable sweating
- stomach pain, discomfort
- lower extremity bone pain
Some of the complications caused by myelofibrosis include:
- liver failure
- thrombocytopenia
- portal hypertension
- gout caused by uric acid overproduction
- bone & connective tissue inflammation causing severe bone & joint pain
- upper left side, shoulder pain caused by inflammation or tissue death in your spleen
- decreased ability to fight infections caused by white blood cell malformation, mutation
- extramedullary blood formation causing tumor development resulting in hemoptysis, seizures
Idiopathic myelofibrosis typically becomes progressively worse and can cause your death. And some with this bone marrow disease will eventually develop acute myelogenous leukemia, a type of blood cancer that progresses very quickly. Yet, you could have myelofibrosis and live without anemia related or any of its other symptoms for years.
Myelofibrosis severe anemia can accompany other health conditions, like:
- lymphoma
- tuberculosis
- breast cancer
- prostate cancer
- Paget’s disease
- multiple myeloma
- Gaucher’s disease
- various forms of leukemia
- other myeloproliferative diseases
There’s presently no cure for myelofibrosis, as well as no standard treatment because it mainly focuses on relieving your symptoms. Therefore, without symptoms myelofibrosis treatment isn’t necessary.
For those that do experience idiopathic myelofibrosis symptoms, treatment varies extensively based on those symptoms, for instance:
- interferon
- splenectomy
- chemotherapy
- radiation therapy
- bisphosphonates
- blood transfusions
- thalidomide combined with prednisone
- iron,folic acid, vitamin B12 supplementation
- androgen male hormone therapy combined with corticosteroids
Provided you meet certain criteria, a bone marrow or stem cell transplant may be used to treat myelofibrosis. And spontaneous remission is possible, though it’s exceptional.
And because your blood cell count may very during the course of this bone marrow disease, coupled with the fact that other health conditions can cause severe anemia, diagnosis is frequently difficult.
