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Paediatrics > Haematology > Flashcards

Haematology Flashcards

1
Q

What is the inheritance pattern of von Willebrand disease?

A

Autosomal dominant

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2
Q

What are the features of Fanconi anaemia ?

A 
Macrocytic anaemia 
Pancytopenia in 50%
Cafe au lait spots 50% 
Short stature 50% 
Thumb / radial anomalies
Microcephaly
Abnormal gonads 
Eye anomalies
Structural renal defects 
Dev delay

Cancer predisposition in 10-30%

Can have VACTERL associations

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3
Q

What organisms would a person with sickle cell anaemia be most vulnerable to?

A 
Strep pneumoniae
Neisseria meningidites
Klebsiella pneumoniae 
Haemophilus influenzae
Salmonella typhi 
Cryptococcus neoformans 
Pseudomonas aeruginosa

Ie encapsulated organisms due to functional asplenia

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4
Q

What condition is the finding of Birbeck granules on electron microscopy pathognemonic for?

A

Langerhans cell histiocytosis

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5
Q

What condition should you suspect with immunohistochemistry findings of large cells positive for S-100 and CD1a protein ?

A

Langerhans Cell Histiocytosis

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6
Q

How do you distinguish between haemophilia and lupus anticoagulant on mixing studies ?

A

Both have prolonged APTT
Haemophilia corrects with mixing studies
Lupus does not (or inhibitor)

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7
Q

What is the cause of these blood results?
Prolonged APTT
Prolonged thrombin time
Normal reptilase

A

Heparin effect

Reptilase assesses fibrinogen

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8
Q

Which type of haemophilia is most at risk of developing inhibitors ?

A

Severe haemophilia A 30%

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9
Q

Sickle cell disease blood film will show Howell jolly bodies - T/F

A

True

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10
Q

What is a distinguishing feature on blood film of G6PD?

A

Heinz bodies

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11
Q

Name 3 causes of shistocytes on blood film

A

Microangiopathic haemolytic anaemia HUS
DIC E.g. Kassabach Merritt
Malignancy

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12
Q

What stain is used to differentiate AML from ALL?

A

Myeloperoxidase

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13
Q

What are Auer rods suggestive of?

A

AML

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14
Q

What are Reed Sternberg cells suggestive of?

A

Hodgkin Lymphoma

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15
Q

What are Dohle bodies suggestive of ?

A

Located in neutrophils, marker of inflammation

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16
Q

What are hypersegmented neutrophils suggestive of?

A

B12/ folate deficiency

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17
Q

On an iron stain what are ringed sideroblasts suggestive of?

A

Mitochondrial disorders

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18
Q

What should normal RBC look like on film at birth?

A

Macrocytic normochromic

19
Q

When are Beta chains produced?

A

From 4-6 months

20
Q

What chromosome is Beta chain gene located on?

A

11

21
Q

What chromosome is alpha chain gene located on?

A

16

22
Q

What is raised HbA2 diagnostic for ? (Alpha 2, delta 2)

A

Beta thal

23
Q

What is foetal haemoglobin composed of?

A

Alpha 2

Gamma 2

24
Q

In alpha thalassaemia what is the consequence of 3 gene deletion?

A

HbH (4 beta chains)

25
Q

In alpha thalassaemia what is the consequence of 4 gene deletion ?

A

HbBarts

4 gamma chains

26
Q

What vitamin deficiency is suggested by raised MMA and homocysteine?

A

B12

NB folate def : normal MMA raised homocysteine

27
Q

Which immunoglobulin is associated with warm type autoimmune haemolytic anaemia ?

And cold?

A

Warm IgG

Cold IgM

28
Q

Name 4 causes of haemoglobinuria due to intravascular haemolysis

A 
Oxidant haemolysis G6PD 
ABO incompatibility 
Cold type AIHA 
Paroxysmal nocturnal haemoglobinuria 
Malaria
29
Q

What is the inheritance of G6PD?

A

X linked

30
Q

What organisms are patients susceptible to in sickle cell disease?

A

Encapsulated
Due to splenic sequestration
Strep pneumoniae
Salmonella - cause of osteomyelitis

31
Q

Name two types of congenital neutropenia syndromes

A

Kostmann

Schwachman diamond

32
Q

What do you see on blood film of ITP?

A

Few but large platelets

33
Q

What is the treatment for life threatening bleeding due to ITP ?

A 
Methylpred IV 
Platelet transfusion 
Recombinant factor VIIa 
IVIG 
Urgent splenectomy
34
Q

What is the commonest antibody found in neonatal alloimmune thrombocytopenia ?

A

Anti HPA 1 a

35
Q

What condition would you see giant platelets on film?

What is the defect ?

A

Bernard soulier

Glycoprotein 1 b deficiency - don’t aggregate

36
Q 
What syndrome should you suspect ? 
Bleeding due to plt dysfunction
Albinism 
Photophobia 
Pyogenic infections 
Peripheral neuropathy
A

Chediak higashi

37
Q

What is the inheritance of haemophilia ?

A

X linked recessive

38
Q

What is the severity of Haemophilia A based on factor levels ?

A

<1 % severe
1-5% moderate
5-50% mild

39
Q

What should you suspect in haemophilia A if APTT does not correct with mixing ?

A

Inhibitor

40
Q

What are the types of vWF deficiency ?

A

Type 1 - decreased amount normal multimer
Type 2 - decreased amount and abnormal
Type 3 - virtual absence

41
Q

What is the treatment for vWF def?

A

Desmopressin

Increases release of vWF from endothelial cells

42
Q

What should warfarin do to coags?

A

Prolonged PT

43
Q

What should heparin do to coags?

A

Prolonged APTT
Does not correct with mixing
Does not change with reptilase time

Paediatrics (17 decks)

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