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Primary Myelofibrosis

August 08, 2012  by: Dr. Simran Kaur  Points: 8   Category: Health  Earning $0.20   Views: 307

Primary myelofibrosis is a clonal disorder of a multipotent haematopoietic progenitor cell in which there is increased fibrosis within the marrow, splenomegaly with extramedullary haemopoiesis.

         

Primary myelofibrosis is a clonal disorder of a multipotent haematopoietic progenitor cell in which there is increased fibrosis within the marrow, splenomegaly with extramedullary haemopoiesis.

Symptoms-

1. It affects the people above the age of sixty years.

2. Acute myeloid leukaemia is there.

3. Anemia, massive splenomegaly.

4. Fever, weight loss, sweating and heat intolerance.

5. Thrombocytopenia.

6. Portal hypertension with infection.

7. Hepatomegaly, lymphadenopathy, ascites, jaundice.

8. Pleural effusion, pericardial effusion, cardiac failure.

9. Extramedullary haemopoiesis can affect spleen, liver, lymph nodes, kidneys and adrenals.

10. Haemoglobin is decreased in later or advanced stages of disease.


Investigations-

1. Hb test.

2. TLC test.

3. Platelet count test.

4. Peripheral blood smear test.

5. Bone marrow test.

6. Leucocyte alkaline phosphatase test.

7. Serum vitamin B12 test.

8. X-ray of spine and extremities.


Treatment-

1. Treat anemia by treating the cause of anemia and also give folic acid supplementation.

2. Treat splenomegaly with appropriate medicines and local radiotherapy or splenectomy in selected cases.

3. Low dose irradiation therapy is given to treat extrameduallary haemopoiesis.

4. Bone marrow transplantation in selected cases.




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