Extras from notes

Question 1

How do you get lymphopaenia in SLE

Answer 1

IgM cold reactive, complement fixing Ab

Question 2

angioimmunoblastic lymphoma in SLE

Answer 2

lymphadenopathy

onion skin splenic arteries

Question 3

Increase what kind lymphoma in SLE

Answer 3

NHL

Question 4

preg and oestrogen on transferrin

Answer 4

increase

Question 5

differentiate acute illness and ACD

Answer 5

acute illness and transferrin will be normal
in ACD transferrin will be low

transferrin sats low in both
iron low in both

Question 6

How does trali happen and what time frame?

Answer 6

donor ab attack host leukocytes which sequester in lungs
CK from donor

6 hours post

Question 7

what red cell marker is most immunogenic

Answer 7

D

Question 8

why leukocyte reduce blood prodcts?

Answer 8

reduce HLA alloimmunisation
reduce febrile NON haemolytic transfusion reaction
reduce CMV transmission

Question 9

treat febrile non haemolytic reaction

Answer 9

stop transfusion
rule out haemolytic transfusion
antipyretics
consider abx

Question 10

reduce allergic reaction

Answer 10

wash cell producs

antihistamine

Question 11

reduce anaphylaxis risk

Answer 11

wash

use IgA def donor

Question 12

TRALI reduce by

Answer 12

reducing multips as donors

Question 13

Reduce GVHD

Answer 13

gamma irradiation

Question 14

CMV reduction reduce

Answer 14

leukoreduction

CMV negative donor

Question 15

alloimmunised to platelets

Answer 15

refractory to future transfusions

need HLA matched if alloimmunised, from a single donor

Question 16

do you give IVIG in myasthenia?

Answer 16

yes in LEMS

Question 17

what happens in delayed (not acute) haemolytic transfusion reaction?

Answer 17

re exposed to erythrocyte Ag OUTSIDE the ABO system when ABO alloAb are present
Develop after previous transfusion
see FIVE TO TEN DAYS post transplant with jaundice, anaemia, fevers, extravasc haemolysis

not same as acute haemolytic where ABO incompatable and QUICK

Question 18

mechanism of allergic reactions and anaphylaxis

Answer 18

urticaria common- from transfused proteins

WASH CELLS to prevent

Question 19

What is the normal role of factor V

Answer 19

factor Xa needs factor V and a calcium to do its thing on prothrombin

Question 20

Factor 5 leiden risk

Answer 20

venous only
increase risk initial but not recurrent- dont have on long term

homo increase 50 x
hetero or OCP increase 5 x

Question 21

mechanism of PT gene mutation

Answer 21

stabilise mRNA of prothrombin so increase translastion

hetero 2.5 x increase

Question 22

antithrombin def moa

Answer 22

normally antithrombin inactivates factor Xa
can rarely lead to HEPARIN RESISTANCE
highest risk
1.8% per year

Question 23

protein C usual role

Answer 23

inactiveate Va and VIIIa

Question 24

protein S usual role

Answer 24

cofactor for protein C

50% before age 50

Question 25

worst cancers to be prothrombotic

Answer 25

brain

pancrease

Question 26

what needs to be delayed testing post DVT?

Answer 26

FVL and PTgene mutation whenever

AT, protein C and S, dysfibrinogenaemia need to assess remote from event and off anticoag for 2-4 weeks

Question 27

drugs bad in G6PD

Answer 27

cipro
sulphur
primaquine
nitrofurantoin

Question 28

G6PD film findings

Answer 28

BITE cells

Heinz bodies

Question 29

When can you check G6PD def?

Answer 29

NOT in acute ep! Reticulocytosis means more and could be false neg.

Question 30

Coombs postiive in warm or cold?

Answer 30

Both
positive for IgG and weakly or not for C3 in warm
neg for IgG and pos for complement strongly in cold

steroids do not work in cold - cyclo or ritux

Question 31

most common cause drug induced HA

Answer 31

second third gen cephalosporins

penicillin- ab against drug-RBC immune comples
quinidine- complement mediated haemolysis
methyldopa- inhibit T supressor so autoAb from B cells

Question 32

RDW in iron def vs thal

Answer 32

Increase in iron def

normal in thal

Question 33

Hydroxyurea in sickle cell when

Answer 33

reduces mortality

give in painful episodes
acute chest syndrome
anaemia symptomatic

stimulates HbF production

Question 34

treat pain crisis in sickle cell

Answer 34

opioid analgesia
hydration
chest PT to avoid chest syndrome

Question 35

stroke in sickle cell what to do

Answer 35

exchange transfusion

Question 36

acute chest syndrome manage

Answer 36

in sickle cell

empiric broad spectrum abx
oxygen supplemental
pain meds
avoid overhydration as can give APO
eryth exchange transfusion  or just transfusion if still hypoxic- aim HB under 100

Question 37

DVT proph post MI

Answer 37

UFH

Question 38

DO MIXING STUDIES CORRECT NOAC coag abn?

Answer 38

NO!!!!

Question 39

serum transferrin an acute phase reactant?

Answer 39

negative acute phase reactant

Question 40

HFE mutation MOA

Answer 40

normal role HFE is to facilitate transferrin protein binding

HFE mutation means the intestines interpret strong transferrin signal as if body is deficient in iron

Question 41

causes of intestinal pseudo-obstruction

Answer 41

dilated small and large bowel loops

hypothyroid
DM
hypocalcaemia
hypokalaemia 
uraemia

Question 42

Best predictor of thrombotic risk in ET?

Answer 42

white cell count

Question 43

Treat ET

Answer 43

exclude acquired VWD with ristocetin before putting on low dose aspirin

age over 65 or prev thrombotic event put on hydroxyurea
pregnant and need treatment use interferon alpha
anagrelide is an alternative to hydroxyurea- inhibit mega K maturation

Question 44

Treat PCV

Answer 44

All aspirin
Over 60 or thromb event in past- hydroxyurea
phlebotomy to target haematocrit under 45
interferon alpha safe in pregnancy

Question 45

Life threatening bleeding in ITP

Answer 45

IVIG
steroids
platelets

Question 46

platelet life span

Answer 46

7-10 days