Myeloproliferative disorders Flashcards
Myeloproliferative - definition
Proliferation of myeloid cells within the bone marrow
Myeloid cells within the bone marrow - categories
Granulocytes
Red cells
Platelets
Histology
Mixture of different cell types
Hypercellular
What are the 2 subtypes
BCR-ABL1 negative
BCR-ABL1 positive
Which 3 conditions are BCR-ABL1 negative?
Polycythaemia Rubra Vera
Essential thyombocythaemia
Idiopathic myelofibrosis
What condition is BCR-ABL1 positive?
Chronic myeloid leukaemia
When should you consider MPD as a diagnosis?
When there is NO reactive (secondary) explanation
High granulocyte count +/- High RBC count / Hb +/- High platelet count +/- Eosinophilia/basophilia +/- Splenomegaly
MPD is always a primary problem in the bone marrow itself. True or false?
True
Eosinophilia/basophilia is more likely to indicate primary or secondary problem
Primary (MPD)
If patient has bloods taken and the results are significantly abnormal, what is the course of action?
Re-check the blood count to make sure there haven’t been any errors
Clinical features
Asymptomatic
Increased cell turnover
- gout, fatigue, weight loss, sweats
Symptoms/signs due to splenomegaly
erythromelalgia
Polycythaemia rubra vera - blood count features
High Hb / HCT
Erythrocytosis (too many red cells)
Low EPO levels
May have excessive production of other lineages
Secondary polycythaemia - features
Chronic hypoxia
Smoking
EPO-secreting tumour
Pseudopolycythaemia
Normal amount of RBC but on blood count it appears to be high as it is relative to a reduction in the plasma volume
Polycythaemia rubra vera - clinical features
Common to MPD Headache Fatigue Itch (aquagenic puritis) - worse on exposure to warm water