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1
Q

What can you give with doxorubicin to reduce cardiac toxicity?

A

Dexrazoxazine- EDTA chelator that is cardioprotective

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2
Q

Budd Chiari, do

A

JAK 2 mutation - 50% have

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3
Q

hydroxyurea MOA

A

in ET; reduce production of deoxyribonucleotide by inhibit ribonucleotide reductase

in sickle cell promote fetal Hb formation which reduces sickling

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4
Q

ESR in PRV

A

low

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5
Q

ALP in PRV

A

high

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6
Q

What other cell things do you see with PRV

A

high plt

leukocytosis with basophilia
ncrease serum B12

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7
Q

treat PRV

A

low dose aspirin all
phlebotomy survival benefit except not risk myelofib- aim hct under 45

hydroxyurea if over 50 or history thrombosis
can give interferon alpha but poorly tolerated, safe in preg

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8
Q

how to ET patients bleed?

A

soak up all the VWF- relative type 2 VWD

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9
Q

ddx for ET

A

having CML with high plt- think if phil positive

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10
Q

are thrombopoetin levels useful in ET

A

No- low or high

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11
Q

treatment ET

A

hyroxyurea if over 65 or thrombotic event
normalising plt count does not change risk arterial or venous thrombosis
anagrelide as only reduces plt line- could use if WCC cannot take hydroxyurea

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12
Q

Myelofibrosis periph smear

A

leukoerythroblastic blood picture with nucleated red cells and eearly white cells and tear drop cells

on BM - HYPERCELLULAR, increase Mega K, collagen reticulin replace bone marrow cavity

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13
Q

New agent for myelofibrosis

A

Ruxolitinib
selective inhibitor janus kinase 1 and 2
reduces spleen size
improve night sweats, itch, abdo discomfort, QOL

side effect bleeding anaemia neutropaenia

not survival advantage

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14
Q

How is the globulin gap useful

A

in hyperviscosity, could see globulins minus albumin over 4

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15
Q

treatment for hyperviscosity from monoclonal

A

plasmaphoresis

often follow up with rituximab and cyclophosphamide or fludarabine chemo regimen

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16
Q

What is the sensory neuropathy assoc with waldenstroms

A

Sensorimoton neuropathy associated with anti myelin associated glycoprotein Ab

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17
Q

MGUS is

A 
M protein under 30g/L 
and
under 10% plasma cells on BM exam
and
no anaemia, kidney failure, bone disease, other myeloma end organ disease
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18
Q

What do you do if suspicious but serum protein EP normal, free light chains normal?

A

24 hour urine PE

immunofixation assay urine

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19
Q

What imaging do you get in IgM MGUS?

A

Ct - lymphad chest and abdo

more likely assoc with waldenstroms of NHL than myeloma

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20
Q

Normal kappa to lambda

A

0.25- 1.26

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21
Q

IgG or IgA MGUS risk…

A

MM

AL amyloidosis

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22
Q

IgM MGUS risk…

A

waldenstroms

AL amyloidosis

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23
Q

bad ways to get AML

A

treatment related
old transformation MDS or MPD
high risk cytogenetics

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24
Q

AML induction

A

7+3
cytarabine+ anthracycline

but in older patients cytarabine causes cerebellar tox and increases death

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25
mild mod severe haemophilia
SEVERE UNDER 1% mod 1-4 % mild 5-25%
26
How does ristocetin cofactor activity work
ability of serum to aggregate platelets in the presence of ristocetin normal or increase in haemophilia, along with VWFag. Both low in VWD this is the case all except the rare type of VWD wehre cannot bind the factor 8 molecule- so need to check VWF-factor 8 binding to establish diagnosis
27
Why do joints bleed in haemophilia
make more factor ten a inhibitor synovium
28
Clinically, acquired factor def how present?
Not with haemarthroses | Still get IM, GI bleeds and all the rest of it
29
How do you get AL amylodosis?
From LIGHT chains secondary to myeloma, waldenstroms etc cardiac and neuro, macroglossia, periorbital ecchymoses often low or undetectable SPEP get SFLC
30
How do you get AA amyloid?
Acute phase reactant- precursor of serum amyloid A chronic infl like TB, bronchiectasis, RA RENAL involvement ost common
31
Amyloid on dialysis?
Beta 2 microglobulin
32
first step in haemostasis
vascular spasm then platelet plug then coag
33
NO and prostacyclin role in plt
released from normal adjacent endothelium and inhibits plt agg
34
four things thrombin does
formation fibrin increase thrombin in pos feedback activate factor 13 which catalyses cross linkages of fibrin increase platelet activation
35
Factor 8 levels in liver disease
go up
36
How does mesna work?
acrolein which is a metab of the cyclophos is toxic to urothelium-->mesna binds and inactivates acrolein-->prevent haemorrhagic cystitis
37
what causes sideroblasts
this is iron accumulation myelodysplasia lead TB meds alcohol
38
Tranexamic MOA
analogue of lysine- bind lysine sites on plasmin- prevent plasmin degrading fibrin
39
CLL management
robust- fludarabine based chemo old and crusty- cyclophos/chlorambucil based relapsed- ibrutinib (brutons TKI), alemtuzumab Ritux improves if in comb dont treat unless dropping cell counts or RAi 3-4
40
Leuokcyte ALP in CML
down
41
Periph cells in CML
both mature and immature granulocytes plt up basophilia-->flush, itch, diarrhoea
42
Imatinib works how?
compeditive inhibition of ATP binding site of ABL kinase in inactive conformation-->inhibit tyrosine phosphorylation of proteins involved in BCR-ABL signal transduction apoptosis is induced
43
What is major molecular remission in CML
over 3 log reduction in BCR ABL transcript levels at 18 montsh not same as complete haem remission with normal cell counts, WCC under 10
44
side effect nilotinib | dasatanib
nilot- sudden death dasat- pleural effusion increase likely major molec response and cytogen remission at one year can give first up or once resistant to imat
45
odds that any one sibling will be a match is
1- (0.75) to the n
46
twin GVHD risk | sibling GVHD risk
0% 15% methotrex reduces GVHD but delays engraftment reduce risk if take T cells out of graft, but increase risk relapse and graft failure
47
liver biopsy GVHD
small bile ducts segmentally disrupted
48
In transplant, only give PJP proph once...
has engrafted
49
classic meningitis cause post BMT
b cereus
50
Why give valacic post transplant
prevent HSV pneumonia (unique to allo) and mucositis | also reduces HSV 1 oesoph and HSV 2 anogenital disease
51
time period for CMV
30-90 days after engraftment usually pneumonia - IVIG and gancic
52
When EBV a problem post tx
1-3 months as early as fevers, cervival LN, extranodal mass EBV PCR monitor post
53
Bridge trial: AF to interrupt or nor warfarin
no bridging is non inferior to LMWH and reduces risk major bleeding (but mostly chads 4 or below)
54
microcytic
``` inron def ACD thalassaemia sideroblastic some MDS hyperthyroid ```
55
macro
``` B12/folate(mtx, pentamidine, trimeth, aza, zidovudine, hydroxyurea) alcohol chronic liver disease MDS reticulocytosis from bleeding phenytoin Downs copper def cold agglut-->factitious ```
56
B12 uptake describe
eat from eggs, meat, dairy bind R protein/transcobalmin 1 in saliva broken down by panc enzymes bind intrinsic factor from parietal cells terminal ileum bind cubilin receptor-->uptake transport on holocobalmin (transcobalmin 2) to liver BM kidneys
57
thrombolysis and bleeding, give
cryoprecipitate platelets protamine if given heparin
58
More likely to have a haemorrhage post thrombolysis if
NIHSS score over 20 | over 1/3 MCA territory hypodense on initial CT
59
when use crypo
liver disease and low fibrinogen and bleeding thrombolysis DIC sometimes for factor 13 def if renal failure and plt dysfunction does not respond to dialysis or DDAVP
60
IF fibrinogen over 1g/L in DIC do what
dont give prothrombinex, give FFP to address the multiple deficiencies typical of DIC

Haematology (19 decks)

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