Extras from notes Flashcards

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
Q

mild mod severe haemophilia

A

SEVERE UNDER 1%
mod 1-4 %
mild 5-25%

26
Q

How does ristocetin cofactor activity work

A

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
Q

Why do joints bleed in haemophilia

A

make more factor ten a inhibitor synovium

28
Q

Clinically, acquired factor def how present?

A

Not with haemarthroses

Still get IM, GI bleeds and all the rest of it

29
Q

How do you get AL amylodosis?

A

From LIGHT chains
secondary to myeloma, waldenstroms etc

cardiac and neuro, macroglossia, periorbital ecchymoses
often low or undetectable SPEP
get SFLC

30
Q

How do you get AA amyloid?

A

Acute phase reactant- precursor of serum amyloid A

chronic infl like TB, bronchiectasis, RA

RENAL involvement ost common

31
Q

Amyloid on dialysis?

A

Beta 2 microglobulin

32
Q

first step in haemostasis

A

vascular spasm
then platelet plug
then coag

33
Q

NO and prostacyclin role in plt

A

released from normal adjacent endothelium and inhibits plt agg

34
Q

four things thrombin does

A

formation fibrin
increase thrombin in pos feedback
activate factor 13 which catalyses cross linkages of fibrin
increase platelet activation

35
Q

Factor 8 levels in liver disease

A

go up

36
Q

How does mesna work?

A

acrolein which is a metab of the cyclophos is toxic to urothelium–>mesna binds and inactivates acrolein–>prevent haemorrhagic cystitis

37
Q

what causes sideroblasts

A

this is iron accumulation

myelodysplasia
lead
TB meds
alcohol

38
Q

Tranexamic MOA

A

analogue of lysine- bind lysine sites on plasmin- prevent plasmin degrading fibrin

39
Q

CLL management

A

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
Q

Leuokcyte ALP in CML

A

down

41
Q

Periph cells in CML

A

both mature and immature granulocytes
plt up
basophilia–>flush, itch, diarrhoea

42
Q

Imatinib works how?

A

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
Q

What is major molecular remission in CML

A

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
Q

side effect nilotinib

dasatanib

A

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
Q

odds that any one sibling will be a match is

A

1- (0.75) to the n

46
Q

twin GVHD risk

sibling GVHD risk

A

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
Q

liver biopsy GVHD

A

small bile ducts segmentally disrupted

48
Q

In transplant, only give PJP proph once…

A

has engrafted

49
Q

classic meningitis cause post BMT

A

b cereus

50
Q

Why give valacic post transplant

A

prevent HSV pneumonia (unique to allo) and mucositis

also reduces HSV 1 oesoph and HSV 2 anogenital disease

51
Q

time period for CMV

A

30-90 days after engraftment

usually pneumonia - IVIG and gancic

52
Q

When EBV a problem post tx

A

1-3 months as early as
fevers, cervival LN, extranodal mass
EBV PCR monitor post

53
Q

Bridge trial: AF to interrupt or nor warfarin

A

no bridging is non inferior to LMWH and reduces risk major bleeding (but mostly chads 4 or below)

54
Q

microcytic

A
inron def
ACD
thalassaemia
sideroblastic
some MDS
hyperthyroid
55
Q

macro

A
B12/folate(mtx, pentamidine, trimeth, aza, zidovudine, hydroxyurea) 
alcohol
chronic liver disease
MDS
reticulocytosis from bleeding
phenytoin
Downs
copper def 
cold agglut-->factitious
56
Q

B12 uptake describe

A

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
Q

thrombolysis and bleeding, give

A

cryoprecipitate
platelets

protamine if given heparin

58
Q

More likely to have a haemorrhage post thrombolysis if

A

NIHSS score over 20

over 1/3 MCA territory hypodense on initial CT

59
Q

when use crypo

A

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
Q

IF fibrinogen over 1g/L in DIC do what

A

dont give prothrombinex, give FFP to address the multiple deficiencies typical of DIC