Myeloproliferative neoplasms Flashcards
1
Q
What are myeloproliferative neoplasms?
A
Clonal haemopoietic stem cell disorders with an increased production of one or more types of haemopoietic cells, leading to increased proliferation of red cells, granulocytes and/or platelets
2
Q
What are the 2 main classes of MPN?
A
- BRC-ABL1 negative
- BRC-ABL1 positive
3
Q
What is BRC-ABL1also known as?
A
Philadelphia chromosome
4
Q
What is the main BRC-ABL1 positive MPN?
A
Chronic myeloid leukaemia (CML)
5
Q
What are the 3 main types of BRC-ABL1 MPN?
A
- Polycythaemia vera
- Myelofibrosis
- Essential thrombocythaemia
6
Q
When should an MPN be suspected?
A
If there is the presence of any of the following:
- High granulocyte count ±
- High red count or haemoglobin ±
- High platelet count ±
- Eosinophilia or basophilia ±
- Splenomegaly
- Thrombosis
- No reactive explanation
7
Q
What are some general symptoms of MPN?
A
- Gout
- Fatigue
- Weight loss
- Sweats
- Splenomegaly
- Marrow failure
- Risk of leukaemic transformation
- Thrombosis
8
Q
What occurs in Philadelphia chromosome mutation?
A
The gene product is a tyrosine kinase which causes abnormal phosphorylation (signalling) leading to the haematological changes in CML
9
Q
A
