Haematology

Question 1

Coagulation disorder suspected but coagulation profile is normal - think of

Answer 1

Factor XIII deficiency

Question 2

What does factor XIII do?

Answer 2

Makes crosslinks between fibrin dimers, so stabilises the clot

Question 3

What kind of bleeding do you get with factor XIII deficiency?

Answer 3

Bleeding a few days after the injury

Question 4

How is factor XIII deficiency inherited?

Answer 4

Autosomal recessive

Question 5

How is factor XIII deficiency managed?

Answer 5

Either with factor XIII supplementation or cryoprecipitate infusions

Question 6

Which is the most severe vWF disease?

Answer 6

Type 3 (autosomal recessive)

Question 7

What does von willebrand factor do?

Answer 7

Acts as the transporter protein for factor VII and also helps platelet binding at endothelial sites

Question 8

How do you test for vWF?

Answer 8

Partial thromboplastin time often prolonged; ristocetin cofactor activity test measures plasma vWF function

Question 9

What are the coagulation screen abnormalities in factor XII deficiency?

Answer 9

Prolonged partial thromboplastin time

Question 10

How is factor XII deficiency inherited?

Answer 10

Autosomal recessive

Question 11

What causes allergic transfusion reactions?

Answer 11

Interaction between donor plasma proteins and recipient IgE antibodies

Question 12

What haematological abnormality does phenytoin cause?

Answer 12

Folate deficiency -> macrocytosis

Question 13

What haematological abnormality does hydroxyurea cause?

Answer 13

Macrocytosis with megaloblastic anaemia

Question 14

What haematological abnormality can penicillin cause?

Answer 14

Haemolytic anaemia

Question 15

What haematological abnormality can cotrimoxazole cause?

Answer 15

Aplastic anaemia

Question 16

Which disease: pancytopenia, short stature, cafe au lait spots, squint, limb anomalies, mental retardation, chromosome fragility, predisposition to leukaemia

Answer 16

Fanconi anaemia

Question 17

How is Fanconi anaemia inherited?

Answer 17

Autosomal recessive

Question 18

What haematological abnormalities are seen in Diamond-Blackfan syndrome?

Answer 18

Normo- or microcytic anaemia with low reticulocyte count

Question 19

Iron studies in iron deficiency anaemia

Answer 19

Low serum iron
High TIBC
Decrease in transferrin saturation
Increase in serum soluble transferrin receptors
Low ferritin
Increased zinc protoporphyrin/haem ratio
Decreased serum ferritin

Question 20

Iron studies in chronic disease

Answer 20

Low serum iron
low TIBC
Normal transferrin saturation
Normal/increased ferritin

Question 21

Iron studies in beta thalassaemia

Answer 21

Normal/increased serum iron
Normal TIBC
Normal transferrin saturation
Normal/increased ferritin

Question 22

Iron studies in sideroblastic anaemia

Answer 22

Normal/high serum iron
Normal/low TIBC
100% transferrin saturation
Increased ferritin

Question 23

X-ray features of osteosarcoma

Answer 23

Intramedullary sclerosis
‘Sunburst’ periosteal reaction
Colman’s triangle (raised periosteum away from bone)

Question 24

Which kind of anaemia does B12 deficiency present with

Answer 24

Microcytic anaemia, neutrophil hypersegmentation

Question 25

What do you see in blood during acute haemolysis?

Answer 25

Decreased plasma haptoglobin Increased plasma methane albumin Haemoglobinuria Heinz bodies

Question 26

Common causes of cold induced haemolytic anaemia?

Answer 26

Infectious mononucleosis, mycoplasma, CMV infection

Question 27

Which antibodies indue cold haemolytic anaemia?

Answer 27

IgM

Question 28

Which antibodies induce warm haemolytic anaemia?

Answer 28

IgG

Question 29

What causes fifth disease?

Answer 29

Parvovirus B19

Question 30

What is the incubation period of slapped cheek?

Answer 30

13-18 days; mainly infective during incubation period

Question 31

Factors in ALL associated with better outcome

Answer 31

Female sex cALL (early pre-B) Remission within 1 month

Question 32

Associated with gingival hypertrophy

Answer 32

Phenytoin Cyclosporin AML

Question 33

Prevention for TLS in high risk children?

Answer 33

``` Aggressive hydration (2-3l/day) Rasburicase (recombinant uric oxidase) ```

Question 34

Usual organisms for acute chest syndrome

Answer 34

Mycoplasma, chlamydia

Question 35

What are similarities and differences between vWD and haemophilia A?

Answer 35

Factor VIII levels are low in both APTT prolonged in both vWD has mucocutaneous bleeding and prolonged bleeding time Haemophilia A has joint bleeding and normal bleeding time + PT

Question 36

Avoid in G6PD:

Answer 36

``` Fava beans Naphthalene Chloroquine Aspirin Cotrimoxazole Nitrofurantoin Nalidixic acid Ascorbic acid ```

Question 37

X ray features of thalassaemia

Answer 37

``` Bone marrow expansion Frontal bossing Prominent maxillae Widening of bone cortex 'hair on end' or 'sun-ray' Phalanges are bilaterally convex or rectangular ```

Question 38

Chemotherapy drug causing acute dystonic reactions

Answer 38

Domperidone

Question 39

Chemotherapy drug causing haemorrhagic cystitis

Answer 39

Cyclophosphamide

Question 40

Chemotherapy drug causing coagulopathy, hepatotoxicity, pancreatitis, hyperglycaemia

Answer 40

L asparaginase

Question 41

Chemotherapy drug causing Redman syndrome (flushing)

Answer 41

Vancomycin

Question 42

Chemotherapy drug causing mucositis, hepatitis, dermatitis, osteoporosis

Answer 42

Methotrexate

Question 43

Chemotherapy drug causing cardiomyopathy, alopecia, mucositis

Answer 43

Doxorubicin

Question 44

Chemotherapy drug causing headache and chest pain

Answer 44

Ondansetron

Question 45

Chemotherapy drug causing headache, seizures, hypertension, rash, photophobia, nephrotoxicity

Answer 45

Ganciclovir

Question 46

Chemotherapy drug causing rash, eosinophilia, biliary sludging

Answer 46

Ceftriaxone

Question 47

Vaccines for those who have had splenectomy

Answer 47

Influenza Pneumococcal polysaccharide Haemophilus influenzae Meningococcal

Question 48

Features of ABO incompatibility vs rhesus

Answer 48

Occurs in first born Presence of spherocytes Exchange transfusion rarely needed

Question 49

Dysmorphic features of Diamond-Blackfan

Answer 49

``` Webbing of neck Short stature Cleft palate Anomalies of the thumb Microcephaly Hypertelorism Micrognathia ```