Haematology 10: CML And Myeloproliferative Disorders Flashcards
What is the difference between relative (pseudo) and true Polycythaemia ?
Relative: low plasma volume, normal Hb, high haematocrit
True: normal plasma volume, high Hb, high haematocrit
List 4 myeloproliferative disorders ?
Polycythaemia Vera
Essential thrombocytopenia
Primary myelofibrosis
CML
If a person has 18% myeloblasts do they have
A) MDS
B) CML
C) ALL
D) AML
A)-MDS
MDS is 5-19% blasts
AML is 20% + blasts
CML has fully differentiated but overproduced Myelocytes
ALL is lymphocytic not myeloid
What is the physiological role of Tyrosine kinases ?
Phosphorylate genes that cause cell growth (increased myeloid cell production)
They do not affect differentiation just proliferation
Which gene is most commonly mutated in polycythaemia rubra Vera ?
JAK2
What affect does JAK2 mutation have on erythropoeisis ?
JAK2 normally phosphorylates EPO receptors in response to EPO.
When mutated this process is constitutively activated so erythropoiesis occurs without regulation by EPO
What is the most pathognomonic sign/symptom of polycythaemia rubra Vera ?
Aquatic pruritus- hot baths causing itching
Give 2 treatments of polycythaemia rubra Vera ?
Hydroxycarbamide
Venesection
Which leukaemia is associated with massive Splenomegaly and hepatomegaly ?
CML
What are the 3 phases of CML ?
Chronic phase
Accelerated phase
Blast crisis
What is the most common chromosomal abnormality in CML ?
t(9;22)- BCR-ABL
Philadelphia chromosome
Which modality is useful for monitoring treatment response in CML ?
RT-PCR (reverse transcriptase)
What is the 1st line treatment for CML ?
Imatinib (tyrosine kinase inhibitor)
Which test allows you to tell the difference between reactive neutrophilia and leukaemia neutrophilia (CML) ?
Leukocyte alkaline phosphatase
Low in CML
Normal/ High in reactive neutrophilia
