Myelofibrosis Flashcards
What is myelofibrosis?
It is defined as a myeloproliferative disorder in which there is increased haematopoietic stem cells
What is the pathophysiological cause of myelofibrosis?
There is hyperplasia of abnormal megakaryocytes, which results in the release of platelet derived growth factor – stimulating fibroblasts
This results in bone marrow fibrosis, in which bone marrow is replaced by scar tissue, leading to anaemia and leukopenia development
What is the pathophysiological consequence of myelofibrosis?
When bone marrow is replaced with scar tissue, haematopoiesis occurs within other regions of the body, such as the liver and spleen
This is known as extramedullary haematopoiesis and can lead to hepatomegaly and splenomegaly
What five risk factors are associated with myelofibrosis?
Older Age > 50 Years Old
Polycythaemia Vera
Essential Thrombocytopenia
Chemical Exposure
Radiation Exposure
What are the two chemicals associated with myelofibrosis?
Toluene
Benzene
What are the nine clinical features of myelofibrosis?
Fever > 38C
Fatigue
Night Sweats
Abdominal Pain
Petechiae Rash
Hepatosplenomegaly
Oesophageal Varices
Abdominal Ascites
Weight Loss
What three investigations are used to diagnose myelofibrosis?
Blood Tests
Blood Film
Bone Marrow Biopsy
What five blood test results indicate myelofibrosis?
Decreased Haemoglobin Levels
Increased/Decreased WBC Levels
Increased/Decreased Platelet Count
Increased Uric Acid Levels
Increased LDH Levels
What are the three features of myelofibrosis on blood film?
Teardrop Poikilocytes
Anisocytosis
Blast Cells
What is anisocytosis?
It is defined as the presence of varying sizes of blood cells
What are blast cells?
They are defined as immature red and white blood cells
What is the feature of myelofibrosis on bone marrow biopsy? Explain
There is an unobtainable sample, which is due to the fact that bone marrow tissue has turned into scar tissue and is therefore deemed as a dry tap
When is conservative management option of myelofibrosis?
It is used to manage ndividuals with mild disease or minimal clinical feature development
What is the conservative management option of myelofibrosis?
It involves regular blood test monitoring
When is chemotherapy management used to manage myelofibrosis?
It is used to manage myelofibrosis in individuals who are deemed unfit for surgical management
