Chronic Myeloproliferative Disorders and CML Flashcards
What are myeloproliferative disorders?
Stem cell disorders of the bone marrow which are MALIGNANT
What is the transformation % into acute leukaemia?
~10%
What are stem cells?
Immature cells, initially pluripotent
What is PV?
Polycythaemia Vera - hypercellular, lots of RBC
What is ET?
Essential thrombosis - increased megakaryocytic > high platelet count (>400)
What is Idiopathic myelofibrosis?
Fibres bands laid down inside bone marrow = elongated cells, low blood count
What are the main points about PV
Affects all ages
Symptoms: thrombosis/stroke/MI as RBC = make blood thick, itching, plethoric face, headache, muzziness, malaise, tinnitus, peptic ulcer, gout
Signs: plethora, engorged retinal veins, splenomegaly
Diagnosis: persistent Hb/hct > 0.5
What is relative PV?
If dehydrated
What is primary PV?
Bone marrow condition = clone of red cells
What is secondary PV?
Hypoxia = increased production of RBC
Can increase RBC by testosterone/EPO injections
What are the first line tests for PV?
FBC, ferritin, EPO level, UE/LFT
What is erythropoietin?
A hormone produced by the kidneys
What is indicated by elevated EPO?
Secondary PV
What kind of cells can also produce EPO?
Cancer cells
What is a JAX2 mutation?
Janus kinase - signalling pathway for cytokine receptors
Controls cell proliferation
Mutation = cell cycle of EPO always on
An acquired genetic mutation
What % of px with PV have a JAX2 mutation?
~95%
What is the treatment for PV?
Venesections aiming for HCT <0.45
Aspirin 75mg orally
What is the prognosis for PV?
Good - 15 year median survival but risk of developing AML/myelofibrosis
What are the causes of Reactive Thromocytosis?
Surgery, infection, inflammation, malignancy, IRON DEFICIENCY, hypersplenism haemolysis, drug induced > steroids, adrenaline, TPO mimetic), post-chemo rebound
What investigations would be done for ET?
Platelet count - persistence platelets >450x10^9/L
What are the first line investigations for ET?
FBC, blood film, ferritin, CRP, CXR, ESR
What are the second line investigations for ET?
JAX2 (50%), CALR - affects reticulum in (45%), 90% of JAX2 -ve ET, bm biopsy
What is the treatment for ET?
Assess thrombotic risk (age, hypertension, DM, platelet >1500)
Antiplatelet treatment > aspirin 75mg daily
Cytoreduction with HYDROXYCARAMIDE, interferon, anagredlide, P32 in high risk px with 1 or more RF
What does hydrocarbamide do?
Anti platelet drug
Suppresses bone marrow
For px that can’t tolerate venesections
