CP40 - The Chronic Myeloproliferative Disorders And Chronic Myeloid Leukaemia Flashcards
what does CMPD stands for?
chronic myeloproliferative disorder
what is CML
chronic myeloid leukaemia
what is CMPD
malignant clonal stem cell disorder of the bone marrow
what are the 3 different disorder in CMPD?
Polycythaemia Vera
Essential thrombocytosis
Idiopathic Myelofibrosis
what happens in polycythaemia vera?
too many RBC
what are some symptoms for polycythaemia vera
splenomegaly, itching, headache, muzziness, general malaise, gout, peptic ulcer
what is the diagnosis of PV?
Hb/hct >0.5
what other condition can cause polycthaemia?
COPD - not enough O2
what is primary polycythaemia?
polycythaemia vera
what are some causes of secondary polycythaemia
central hypoxic process, chronic lung disease, CO poisoning, smoker, high altitude, erythropoietin producing tumours (renal tumours)
what are some specific genetic mutation which produce extra erythropoietin
JAK2 mutation
EXON12 mutation
how is polycythaemia vera
venesections, aspirin (COX receptor)
what does CALR mutation cause
calreticulin mutation cause thrombocytosis - cell signalling protein produce in ER
what are some examples of genetic mutation for ET?
JAK2 & CALR mutation
what are the treatment for ET?
anti platelet treatment (aspirin), cytoreduction
which out of the 3 conditions in the CMPD is the most serious?
Myelofibrosis
what are the common symptoms presented by myelofibrosis?
Pancytopenia - decreased no. RBC, WBC and platelet
B syndromes - fever, night sweat and weight loss
massive splenomegaly
What are B syndrome syndromes of?
Blood cancer
what are the causes of splenomegaly?
C - cancer H - haematological - myelofibrosis, CML, CLL, hariy cell leukaemia I - infection C - congestion - liver disease/portal A - autoimmune - haemolysis, SLE G - glycogen storage disorders O - other - amyloid, sarcoid
chicago
what are the type of cells which is specific for leukaemia
blast cells
what are the clinical features for chronic myeloid leukaemia?
high WBC conc (leucocytosis, leucoerythroblastic blood picture, anaemia, splenomegaly)
what are some symptoms for chronic myeloid leukaemia
Abdominal discomfort: Splenomegaly
Abdominal pain: Splenic infarction
Fatigue: Anaemia, catabolic state
Venous occlusion: Retinal vein, DVT, priapism
Gout Hyperuricaemia
what causes chronic myeloid leukaemia?
BCR-ABL fusion gene - codes for an active tyrosine kinase
