Myeloproliferative disorders Flashcards
that are these disorders?
clonal haemopoietic stem cell disorders with increased production of one or more type of cell
when should you consider a myeloproliferative disorder
high granulocyte count high red cell high platelet increased basophils and eosinophil splenomegaly thrmobosis in unusual place
NO REACTIVE CAUSE!!
which ones are BCR ABL 1 negative
polycythaemia rubra vera
essential thrombocythaemia
myelofibrosis
what is polycythaemia rubra vers
increase in red cells
high Hb/haemocrit and erythrocytosis
increased blood viscosity
what can cause secondary PRV
chronic hypoxia, smoking, EPO secreting tumour
what can cause pseudo PRV
dehydration, obesity, diuretic therpay
in this type you get plasma viscosity
what gene mutation should you check for in PRV?
JAK2
treatment for PRV
vensect to haematocrit <0.45
aspirin chemo
what is essential thrombocythaemia
uncontrolled production of abnormal platelets
what can cause myelofibrosis
marrow failure
marrow fibrosis
what is chronic myeloid leukaemia?
cancer of white blood cell where there is proliferation of myeloid cells in bone marrow
BCR ABL 1 positive!!!
important chromosome in chronic myeloid leukaemia
philadelphia chromosome ( a tyrosine kinase)
name some clinical features of chronic myeloid leukaemia?
upper abdo pain poor appetite low grade fever / night sweats gout increased infection SOB fatigue easy brusiing/ bleeding visual disturbance
how would you test for the philadelphia chromosome on CML?
FISH
how do you treat chronic myeloid leuakemia?
tyrosine kinase inhibitors
bone marrow transplant
