Haematology Flashcards
CML
Philadelphia chromosome- translocation between Ch 9 & 22 -t(9:22) 得了这个病”就二二”
ABL gene + BCR gene = BCR-ABL gene - tyrosine kinase +++
60-70 years old with anaemia, weight loss and sweating
BIG spleen
Blood film: granulocytes at different stages (hence chronic, not just blasts)
But Can undergo blast transformation to become AML (most likely) or ALL
Tx: suppress tyrosine kinase - imatinib(TK inhibitor) “大姨妈就二二”
Von Willi Brand disease
Normally autosomal dominant, most common bleeding disorder
Problem with vWf(stabilises platelet) so that bleeding can’t stop
APTT increases slightly(as factor VIII also needs a bit help from vWf)
Bleeding time increases A LOT
PT is normal
DVT/PE anticoagulation Choice (NICE)
Provoked: 3 months
Unprovoked: 6 months or more
Cancer:3-6 months or more
Normally start with DOAC (apixaban/rivaroxaban)
Unless
1. Renal impairment (CrCl<15): LMWH+ warfarin then warfarin until INR in range
- Antiphospholipid syndrome: LMWH +warfarin then warfarin untilmINR in range
Different bleeding condition and their coagulation profiles (warfarin, heparin, Aspirin, vWF, DIC)
Warfarin (extrinsic): PT ++, normal APTT, Bleeding time and platelet
Heparin(intrinsic): APTT++, normal PT, BT and platelet
Aspirin (platelet): BT ++ but platelet is normal, and normal APTT and PT
vWF (affects platelet stabilisation and factor VIII(intrinsic pathway)): BT++, APTT++, other normal
DIC (everything affected): APTT++, PT++, BT++, platelet low (cause used up), fibrinogen low (used up), fibrinogen degradation product ++, there is schistocytes on blood films due to (microangiopathic haemolytic anaemia)
Polycythemia rubra vera
patient usually in their 60s present with pruritis (worse after shower), and plethoric face (red congested)
other symptoms: headache, dizziness, splenomegaly o/e
associated with JAK 2 mutation (first diagnostic test)
PCT (hematocrit) is high : >0.52 in male and >0.48 in female
Complications: all kinds of clots (stroke, MI, VTE…)
Tx: venesection
Hydroxyurea for high risk
