haematology

Question 1

which infection is commonly known to precipitate haemolytic crisis

Answer 1

parvovirus (Erythema infectiosum)

slapped cheek syndrome

Question 2

Mx acute haemolytic crisis

Answer 2

supportive treatment +/- transfusion

Question 3

long term MX of hereditary spherocytosis

Answer 3

folate replacement

splenectomy

Question 4

classic presentations of the hereditary causes of haemolysis

Answer 4

neonatal jaundice
infection/ drugs precipitate haemolysis
gallstones
+/- splenomegaly depending on whether it is due to intra or extravascular haemolysis

Question 5

red blood cell transfusion thresholds

Answer 5

patients without acute coronary syndrome, transfusion threshold is 70g/L with post-transfusion target 70-90g/L

patients WITH acute coronary syndrome, transfusion threshold is 80g/L with post-transfusion target 80-100g/L

*does not apply to ongoing major haemorrhage or chronic anaemia requiring transfusion

Question 6

why do we irradiate blood products?

Answer 6

deplete T-lymphocytes

avoid transfusion related graft vs host disease

Question 7

most common genetic bleeding disorder and its inheritance pattern
presentation
Ix.

Answer 7

von willebrand disease
autosomal dominant

Ix. prolonged PT, APTT, low factor 8 and normal platelets. defective platelet aggregation with ristocetin

Question 8

presentation of VWF disease

Answer 8

mucosal bleeding (gums, nose) + menorrhagia

Question 9

Mx. of vwf disease

Answer 9

1. tranexamic acid for mild bleeding
2. desmopressin raises levels of vWF
3. factor 8 concentrate

Question 10

1 unit of RBCs

how quickly must be given after removal from fridge and over what timespan

Answer 10

non-urgent transfusions, transfuse 1 unit of RBCs over 90-120 minutes. Give within 4 hours of removal from fridge

Question 11

G6PD deficiency drugs causing haemolysis

Answer 11

aspirin, antimalarials, sulph-group drugs

Question 12

mx of patients at high risk of neutropenia.

which patients are at high risk of neutropenia and neutropenic sepsis

Answer 12

filgrastim (granulocyte-colony stimulating factor)

risk factors: 
elderly
specific ca: non-hodgkins, ALL 
previous neutropenic episodes
combo chemo + radiotherapy

** odly not given to patients with myeloid malignancies as can precipitate them further

Question 13

mx febrile neutropenia

Answer 13

piperacillin with tazopbactam

Question 14

prophylaxis chemotherapy induced neutropenia

Answer 14

fluorquinolone

Question 15

Histology of hodgkin’s lymphoma

Answer 15

4 types
***nodular sclerosing - women, good prognosis

mixed cellularity

lymphocyte predominant - best prognosis

lymphocyte depleted - rare, worst prognosis

Question 16

man with CLL comes in with fever, and swollen lymph nodes

Answer 16

richter transformation into high grade non-hodgkin’s lymphoma

Question 17

causes of iron deficiency

Answer 17

intake - poor diet
absorption - coeliac disease
use - children, pregnancy
loss - menstruation, GI bleeding

Question 18

Well’s score criteria

Answer 18

1 point for each

risk factors: active cancer (<6mo), paralysis, bedridden (surgery), previous documented DVT

signs: local leg tenderness, swollen, calf swelling >3cm compared to other, unilateral pitting oedema, non-varicose distended leg veins
- 2 if an alternative diagnosis is AS LIKELY

Question 19

DVT likely wells score and what do you do

Answer 19

DVT likely (2 or more)

USS within 4 hours → if it positive then treat, if it is negative do a D-dimer to confirm

if can’t do USS within 4 hours, do a D-dimer and treat with DOAC (rivaroxaban) and do the USS within 24hrs

if USS negative, D dimer positive, stop DOAC and repeat USS one week later

Question 20

DVT UNlikely wells score and what do you do

Answer 20

DVT unlikely (1 point or less)

do a D dimer within 4 hours, if positive then do USS
within 4 hours, if not possible within 4, give DOAC and do USS within 24

if can’t do a D dimer within 4 hours, give a DOAC until you can

Question 21

what cases would you NOT give a DOAC for VTE treatment?

what do you give?

Answer 21

severe renal impairment GFR<15/min
antiphospholipid syndrome

LMWH, unfractionated heparin or VKA

Question 22

duration of VTE anticoagulation

Answer 22

everyone for 3 months

+ 0 months if provoked eg. surgery

+ 3 months if unprovoked VTE – absence of bleeding risk factors

+ 1 - 3 months if cancer – a grey area and patient specific

Question 23

reversal agent for DOACs eg. rivaroxaban

Answer 23

andexanet alfa

Question 24

causes of neutropenia

Answer 24

Infection - viral (HIV, EBV< hepatitis), severe sepsis

Inflammation - rheumatological conditions, rheumatoid arthritis (Felty’s hypersplenism), SLE

Malignancy - aplastic anaemia, myelodysplastic malignancies

drugs - clozapine

Question 25

what is the philadelphia translocation and which disease is it associated with?

Answer 25

t(9;22) BCR-ABL gene – excessive tyrosine kinase activity

chronic myeloid leukaemia

Question 26

translocation in acute promyelocytic leukaemia

Answer 26

t(15;17)

Question 27

most common leukaemia in adults in western world and its presentation

Answer 27

B cell chronic lymphocytic leukaemia

asymptomatic (leukocytosis on incidental blood film) +/- generalised lymphadenopathy and hepatosplenomegaly

Question 28

blood film findings CLL

Answer 28

smudge/smear cells

Question 29

which DOAC is preferred for renal impairment patients

Answer 29

apixaban - minimal renal drug clearance

Question 30

reversal agent for dabigatran

Answer 30

idarucizumab

dabigatran is a direct thrombin inhibitor

Question 31

reversal of heparin

Answer 31

protamine

Question 32

beta thalassemia trait blood findings

Answer 32

no changes to blood film
mildly anaemic
quite low MCV
disproportionate microcytosis with mild anemia is the key

Question 33

inheritance pattern of sickle cell anemia

pathophysiology

Answer 33

autosomal recessive

codon 6 of the beta Hb chain substitution of glutamate for valine
this change means that in deoxygenated state the HbS molecule polymerises and the RBC sickles

heterozygous patients only sickle if severely hypoxic
homozygous patients sickle more easily

sickled cells haemolyse and sequestrate causing infarction

Question 34

Jehova’s witness needing surgery with high risk of blood loss

Answer 34

cell saver device – collects blood and reinfuses

Question 35

Warfarin reversal

Answer 35

1. stop warfarin
2. urgent — human prothrombin complex (1hour action but 6 hour half life) + vitamin K
3. vit K (4-24 hours oral vs. IV)
4. FFP

Question 36

reversal of anticoagulation (warfarin) or major haemorrhage

Answer 36

prothrombin complex concentrate

Question 37

cryoprecipitate vs FFP when to give

Answer 37

FFP - clinically significant but no major haemorhage. prolonged PT or APTT

cryoprecipitate - clinically significant but no major haemorhage. low fibrinogen. blood coagulopathies (vwf, haemophilias)