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What is the prognosis of primary myelofibrosis?
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Dr. Mariola Ficinski, MD
1245 Wilshire Blvd Ste 817Los Angeles, CA 90017 Internal Medicine
Expert Medical Information
Primary myelofibrosis is a myeloproliferative neoplasm with a negative Philadelphia (PH) chromosome.
Clinically, it is characterized by anemia, splenomegaly, the presence of immature granulocytes, erythroblasts, teardrop-shaped red blood cells, and an increase in CD34+ cells in the peripheral blood, as well as myelofibrosis and osteosclerosis.
Patients with no clinical symptoms and a stable condition are placed under watchful waiting.
For patients with anemia, thrombocytopenia, and splenomegaly, treatments such as hydroxyurea, androgens, EPO, interferon, prednisone, and thalidomide are selected. Other treatments include supportive therapies such as blood transfusion.
Currently, primary myelofibrosis remains an incurable disease. However, studies have found that patients with JAK2V617F, MPLW515 mutations have a good prognosis, with a median survival of 128 - 176 months. Patients in whom JAK2, MPL, and CALR mutations are not detected have a poor prognosis.
Common symptoms include fever, fatigue, and discomfort in the affected area. Always consult with a healthcare professional for an accurate diagnosis.
Diagnosis typically involves a physical examination, medical history review, and possibly laboratory tests or imaging studies depending on the suspected cause.
Treatment options vary based on the specific condition, severity, and individual health factors. They may include medication, lifestyle changes, physical therapy, or in some cases, surgical intervention.
Seek immediate medical attention if you experience severe symptoms, rapid worsening of condition, or if self-care measures aren't providing relief within a reasonable timeframe.