Haem

Question 1

Heparin & warfarin MoA

Answer 1

Heparin - Prevents activation factors 2,9,10,11

Warfarin - Affects synthesis of factors 2,7,9,10

Question 2

Acute intermittent porphyria tx

Answer 2

avoiding triggers

acute attacks
IV haematin/haem arginate
IV glucose

Question 3

Acute promyelocytic leukaemia associations

Answer 3

associated with t(15;17)
fusion of PML and RAR-alpha genes
Auer rods (seen with myeloperoxidase stain)
DIC or thrombocytopenia often at presentation

Question 4

Antiphospholipid syndrome mx in pregnancy

Answer 4

low-dose aspirin - at pregnancy start

low molecular weight heparin - once fetal heart is seen on ultrasound

Question 5

Warm vs Cold AIHA

Answer 5

Warm
- most common
- IgG
- haemolytic at body temp, at extravascular (spleen)
- Mx: treat underlying, steroids (+/- rituximab)

Cold
- IgM, at 4 deg C
- complement mediated, intravasc
- Raynaud’s and acrocynaosis
- steroids work less well

Question 6

Beta-thalassaemia major ix & mc

Answer 6

HbA2 & HbF raised
HbA absent
(B thalassemia trait: HbA2 raised & mild hypochromic, microcytic anaemia)

repeated transfusion
this leads to iron overload → organ failure
iron chelation therapy is therefore important (e.g. desferrioxamine)

Question 7

Target cells in

Answer 7

Sickle-cell/thalassaemia
Iron-deficiency anaemia
Hyposplenism
Liver disease

Question 8

Spherocytes

Answer 8

Hereditary spherocytosis
Autoimmune hemolytic anaemia

Question 9

Basophilic stippling

Answer 9

Lead poisoning
Thalassaemia
Sideroblastic anaemia
Myelodysplasia

Question 10

Heinz bodies

Answer 10

G6PD deficiency
Alpha-thalassaemia

Question 11

thresholds for transfusion:

Answer 11

Patients without ACS- 70 g/L

Patients with ACS- 80 g/L

in a non-urgent scenario, a unit of RBC is usually transfused over 90-120 minutes

Question 12

Burkitt’s lymphoma associations

Answer 12

c-myc gene translocation
t(8:14).
Epstein-Barr virus (EBV)
microscopic: ‘starry sky’ appearance

tumour lysis syndrome after chemo. (Rasburicase given to prevent)

Question 13

Complications of CLL

Answer 13

anaemia
hypogammaglobulinaemia leading to recurrent infections
warm autoimmune haemolytic anaemia in 10-15% of patients
transformation to high-grade lymphoma (Richter’s transformation)

Question 14

Chronic myeloid leukaemia mx

Answer 14

imatinib is now considered first-line treatment
- inhibitor of the tyrosine kinase associated with the BCR-ABL defect
- very high response rate in chronic phase CML

Question 15

DVT mx

Answer 15

DVT likely
- proximal leg vein USS w/in 4hrs
- if negative -> D dimer, if negative -> alternative dx
- If delay -> interim anticoagulation
- If USS -ve but D dimer +ve -> stop interim anticoagulant, repeat USS 6-8 days later

DVT unlikely
- D dimer w/in 4hrs
- If positive -> proximal leg vein USS w/in 4hrs

Question 16

types of thrombophilia

Answer 16

Factor V Leiden (activated protein C resistance) - most common

Prothrombin gene mutation

Protein C deficiency

Protein S deficiency

Antithrombin III deficiency

Question 17

G6PD deficiency ix

Answer 17

G6PD enzyme assay levels should be checked around 3 months after an acute episode of hemolysis

Question 18

G6PD deficiency associations

Answer 18

Mediterranean and Africa, most common
X-linked recessive, male

broad (fava) beans,
anti-malarials: primaquine
ciprofloxacin
sulph- group drugs

Heinz bodies, Bite and blister cell,

intravascular haemolysis

Question 19

Philadelphia chromosome

Answer 19

t(9;22) -> BCR-ABL gene - tyrosine kinase activity in excess of normal
CML

Question 20

Hereditary angioedema mx

Answer 20

Acute:
IV C1-inhibitor concentrate, fresh frozen plasma (FFP) if this is not available
(adrenaline, antihistamines, or glucocorticoids to not help)

prophylaxis: anabolic steroid Danazol may help

Question 21

Hodgkin’s lymphoma types

Answer 21

( Reed-Sternberg cell)

Nodular sclerosing - Most common (around 70%),

Lymphocyte predominant - Best prognosis

Question 22

ITP mx

Answer 22

1st. oral prednisolone
2nd or active bleeding/ urgent invasive procedure - pooled normal human immunoglobulin (IVIG)

Question 23

Platelet transfusion tx criteria

Answer 23

platelet count of <30 x 10^9 w significant bleeding, higher is severe

Platelet transfusion for thrombocytopenia before surgery/ an invasive procedure. Aim for plt levels of:
> 50×109/L for most patients
50-75×109/L if high risk of bleeding
>100×109/L if surgery at critical site

A threshold of 10 x 10^9 in no bleeding or. procedure

Question 24

Polycythaemia vera mx

Answer 24

aspirin
venesection
chemotherapy

Question 25

sickle cell disease ix & mx

Answer 25

haemoglobin electrophoresis

Longer-term management
- hydroxyurea
- pneumococcal polysaccharide vaccine every 5 years

Crisis:
- analgesia (opiates), rehydrate, oxygen, antibiotics ifinfection
- exchange transfusion

Question 26

Clinical tumor lysis syndrome

Answer 26

Laboratory tumour lysis syndrome plus one or more of the following:
increased serum creatinine (1.5 times upper limit of normal)
cardiac arrhythmia or sudden death
seizure

high potassium and high phosphate level in the presence of a low calcium.

(patients are higher risk should receive either allopurinol or rasburicase before chemo)