Myelofibrosis

Question 1

What is myelofibrosis?

Answer 1

Myelofibrosis is a condition in which there is scarring of the bone marrow, disrupting normal blood cell production

Question 2

Hoes does myelofibrosis occur?

Answer 2

This can be idiopathic, or caused by a previous myeloproliferative disorder such as PV or ET

Question 3

Describe the pathophysiology of myelofibrosis?

Answer 3

In myelofibrosis, abnormal proliferation of marrow fibroblasts causes bone marrow fibrosis (Scarring)

Question 4

What are some clinical features of myelofibrosis?

Answer 4

• Marrow failure
• Extra-medullary haematopoiesis (Liver and spleen)

Question 5

What symptoms are caused by marrow failure in myelofibrosis?

Answer 5

Anaemia
Bleeding
Infection

Question 6

What are some symptoms of extra-medullary haematopoiesis?

Answer 6

Splenomegaly
Hepatomegaly
LUQ abdominal pain
Possible portal hypertension

Question 7

What will blood film show in myelofibrosis?

Answer 7

• Leucoerythroblastic film appearance
• Teardrop poikilocytes (RBCs) in peripheral blood

Question 8

What is shown in this blood film?

Answer 8

Teardrop poikilocytes - Myelofibrosis

Question 9

What is meant by a leukoerythroblastic blood film?

Answer 9

Presence of erythroblasts (Nucleated RBCs) and myelocytes

Question 10

What is shown on this marrow biopsy?

Answer 10

Question 11

What will genetic testing show in myelofibrosis?

Answer 11

• JAK2 mutation
• CALR mutation
• MPL mutation

Approximately 10% of cases will be triple negative

Question 12

What are some supportive management options used in myelofibrosis?

Answer 12

Blood transfusion
Platelets
Antibiotics

Question 13

What are some disease control management options in myelofibrosis?

Answer 13

• Allogenic stem cell transplantation (Select cases)
• Splenectomy (Controversial)
• JAK inhibitors