Haematology Flashcards

1
Q

Coagulation disorder suspected but coagulation profile is normal - think of

A

Factor XIII deficiency

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2
Q

What does factor XIII do?

A

Makes crosslinks between fibrin dimers, so stabilises the clot

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3
Q

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

A

Bleeding a few days after the injury

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4
Q

How is factor XIII deficiency inherited?

A

Autosomal recessive

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5
Q

How is factor XIII deficiency managed?

A

Either with factor XIII supplementation or cryoprecipitate infusions

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6
Q

Which is the most severe vWF disease?

A

Type 3 (autosomal recessive)

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7
Q

What does von willebrand factor do?

A

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

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8
Q

How do you test for vWF?

A

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

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9
Q

What are the coagulation screen abnormalities in factor XII deficiency?

A

Prolonged partial thromboplastin time

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10
Q

How is factor XII deficiency inherited?

A

Autosomal recessive

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11
Q

What causes allergic transfusion reactions?

A

Interaction between donor plasma proteins and recipient IgE antibodies

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12
Q

What haematological abnormality does phenytoin cause?

A

Folate deficiency -> macrocytosis

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13
Q

What haematological abnormality does hydroxyurea cause?

A

Macrocytosis with megaloblastic anaemia

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14
Q

What haematological abnormality can penicillin cause?

A

Haemolytic anaemia

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15
Q

What haematological abnormality can cotrimoxazole cause?

A

Aplastic anaemia

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16
Q

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

A

Fanconi anaemia

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17
Q

How is Fanconi anaemia inherited?

A

Autosomal recessive

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18
Q

What haematological abnormalities are seen in Diamond-Blackfan syndrome?

A

Normo- or microcytic anaemia with low reticulocyte count

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19
Q

Iron studies in iron deficiency anaemia

A
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
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20
Q

Iron studies in chronic disease

A

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

21
Q

Iron studies in beta thalassaemia

A

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

22
Q

Iron studies in sideroblastic anaemia

A

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

23
Q

X-ray features of osteosarcoma

A

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

24
Q

Which kind of anaemia does B12 deficiency present with

A

Microcytic anaemia, neutrophil hypersegmentation

25
Q

What do you see in blood during acute haemolysis?

A

Decreased plasma haptoglobin
Increased plasma methane albumin
Haemoglobinuria
Heinz bodies

26
Q

Common causes of cold induced haemolytic anaemia?

A

Infectious mononucleosis, mycoplasma, CMV infection

27
Q

Which antibodies indue cold haemolytic anaemia?

A

IgM

28
Q

Which antibodies induce warm haemolytic anaemia?

A

IgG

29
Q

What causes fifth disease?

A

Parvovirus B19

30
Q

What is the incubation period of slapped cheek?

A

13-18 days; mainly infective during incubation period

31
Q

Factors in ALL associated with better outcome

A

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

32
Q

Associated with gingival hypertrophy

A

Phenytoin
Cyclosporin
AML

33
Q

Prevention for TLS in high risk children?

A
Aggressive hydration (2-3l/day)
Rasburicase (recombinant uric oxidase)
34
Q

Usual organisms for acute chest syndrome

A

Mycoplasma, chlamydia

35
Q

What are similarities and differences between vWD and haemophilia A?

A

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

36
Q

Avoid in G6PD:

A
Fava beans
Naphthalene
Chloroquine
Aspirin
Cotrimoxazole
Nitrofurantoin
Nalidixic acid
Ascorbic acid
37
Q

X ray features of thalassaemia

A
Bone marrow expansion
Frontal bossing
Prominent maxillae
Widening of bone cortex
'hair on end' or 'sun-ray'
Phalanges are bilaterally convex or rectangular
38
Q

Chemotherapy drug causing acute dystonic reactions

A

Domperidone

39
Q

Chemotherapy drug causing haemorrhagic cystitis

A

Cyclophosphamide

40
Q

Chemotherapy drug causing coagulopathy, hepatotoxicity, pancreatitis, hyperglycaemia

A

L asparaginase

41
Q

Chemotherapy drug causing Redman syndrome (flushing)

A

Vancomycin

42
Q

Chemotherapy drug causing mucositis, hepatitis, dermatitis, osteoporosis

A

Methotrexate

43
Q

Chemotherapy drug causing cardiomyopathy, alopecia, mucositis

A

Doxorubicin

44
Q

Chemotherapy drug causing headache and chest pain

A

Ondansetron

45
Q

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

A

Ganciclovir

46
Q

Chemotherapy drug causing rash, eosinophilia, biliary sludging

A

Ceftriaxone

47
Q

Vaccines for those who have had splenectomy

A

Influenza
Pneumococcal polysaccharide
Haemophilus influenzae
Meningococcal

48
Q

Features of ABO incompatibility vs rhesus

A

Occurs in first born
Presence of spherocytes
Exchange transfusion rarely needed

49
Q

Dysmorphic features of Diamond-Blackfan

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