Myelofibrosis Flashcards
What is myelofibrosis.
It is clonal proliferation of haemopoietic stem cells to bone marrow fibrosis.
It is a premalginant condition.
What is the mean age of diagnosis of myelofibrosis.
Over 50.
What are the clinical signs of myelofibrosis. (7)
Hypermetabolic symptoms (ie ‘B’ symptoms): night sweats, fever, weight loss.
Pallor.
Bruising.
Oral thrush.
Massive hepatosplenomegaly.
Abdominal discomfort due to hepatosplenomagely.
Bone marrow failure.
What is seen on a blood film in a patient with myelofibrosis. (2)
Leucoerythroblastic and tear drop RBC.
What are the complications of myelofibrosis. (3)
DIC.
Transformation to AML.
Liver failure.
What are the poor prognositic factors for patients with myelofibrosis. (4)
Increased age.
Anaemia.
Leucopenia.
Abnormal marrow karyotype.
What is the mechanism of action of myelofibrosis.
There is hyperplasia of megakaryocytes which produce platelet-derived growth factor, leading to intense marrow fibrosis and myeloid metaplasia.
What is the cause of the hepatosplenomegaly seen in myelofibrosis.
Due to haemopoiesis in the spleen and liver.
What is the cause of bone marrow failure in myelofibrosis. (3)
Decreased Hb levels.
Infections.
Bleeding.
What is the prognosis for patients with myelofibrosis.
3-5years.
What are the characteristics of the bone marrow in myelofibrosis. (2)
Initially, the marrow is hypercellular with an excess of abnormal megakaryocytes which released growth factors to the marrow microenvironment, resulting in a reactive proliferation of fibroblasts.
As the disease progresses, the marrow becomes fibrosed.
What can be high in the blood of a patient with myelofibrosis.
Urate levels may be high due to increased cell breakdown.
What is seen on a bone marrow biopsy of a patient with myelofibrosis. (3)
Excess of megakaryocytes.
Increased reticulin.
Fibrous tissue replacement.
What mutation is often found in myelofibrosis.
JAK2.
It is present in approximately half of patients.
What is a common deficiency in patients with myelofibrosis.
Folate.
