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Phase 3a - Paediatrics > Haematology > Flashcards

Haematology Flashcards

1
Q

What are causes of anaemia in infancy?

A
  • Physiologic anaemia of infancy MC
  • Anaemia of prematurity
  • Blood loss
  • Haemolysis (caused by things like haemolytic disease of newborn, hereditary spherocytosis, G6PD deficiency)
  • Twin-twin transfusion
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2
Q

What test can be used to check for immune hameolytic anaemia?

A

Direct Coombs test (DCT)

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3
Q

Causes of microcytic anaemia?

A
  • T – Thalassaemia
  • A – Anaemia of chronic disease
  • I – Iron deficiency anaemia
  • L – Lead poisoning
  • S – Sideroblastic anaemia
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4
Q

What is fanconi anaemia?

A

Rare, inherited genetic disorder that primarily affects bone marrow resulting in pancytopenia.

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5
Q

What inheritance pattern is fanconi anaemia?

A

Autosomal recessive

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6
Q

Complications of fanconi anaemia?

A
  • Acute myeloid leukemia (AML)
  • Squamous cell carcinomas
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7
Q

Sx of fanconi anaemia?

A

Congenital abnormalities:
* Short stature
* Skeletal abnormalities - in thumb/radius (forearms)
* Cafe-au-lait spots
* Microcephaly (small head size)
* Eye abnormalities - small eyes or strabismus (crossed eyes)
* GU abnormalities

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8
Q

Ix for fanconi anaemia?

A
  • Bloods - pancytopenia (due to bone marrow failure)
  • Chromosome breakage tests
  • Genetic testing
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9
Q

What is haemophilia A and B?

A

Inherited bleeding disorders caused by deficiencies in clotting factors 8 (A) and 9 (B) both which are imp. components of intrinsic pathway of coagulation cascade.

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10
Q

What kind of genetic inheritance are haemophilia A and B?

A

X-linked recessive

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11
Q

Sx of haemophilia A and B?

A
  • Spontaneous deep and severe bleeding into soft tissues, muscles and joints (resulting in a deforming arthropathy)
  • Excessive bleeding following trauma or surgical intervention
  • Cerebral haemorrhage
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12
Q

Ix for haemophilia?

A
  • Coagulation factors 8/9 assay
  • Bloods: clotting profile (APTT elevated), vWF antigen, defective platelet
  • Genetic testing
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13
Q

Mx of haemophilia?

A
  • For minor bleeds for haemophilia A pts: desmopressin (DDAVP)
  • For major bleeds: IV infusion - recombinant factor VIII or IX
  • Supportive mx for bleeding wounds - Antifibrinolytics (eg. tranexamic acid)
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14
Q

What is Von Willebrand disease (VWD)?

A

Inherited bleeding disorder characterised by reduced quantity or function of von Willebrand factor which is a protein that normally links platelets to the exposed endothelium and stabilises clotting factor 8. Therefore it’s deficiency leads to inc. risk of bleeding.

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15
Q

Describe the following subtypes of VWD:
* Type 1 VWD
* Type 2 VWD
* Type 3 VWD

A
  • Type 1 VWD: Partial quantitative deficiency in VWF
  • Type 2 VWD: Qualitative defects in VWF (e.g. decreased adhesion to platelets or factor VIII)
  • Type 3 VWD: Almost complete deficiency of VWF

Pts w/ type 2 or 3 VWD have more severe sx + at earlier stage than pts w/type 1.

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16
Q

Sx of VWD?

A
  • Excess/prolonged bleeding from minor wounds
  • Excess/prolonged bleeding post-operatively
  • Easy bruising
  • Menorrhagia
  • Epistaxis (nose bleed)
  • GI bleeding
17
Q

Ix for VWD?

A
  • Clotting tests - Prolonged APTT
  • Normal platelet count
  • GS - Von Willebrand factor level and activity assay
18
Q

Mx of VWD?

A
  • 1st line - Desmopressin (inc. factor 8 and VWF levels)
  • Can increase VWF through blood transfusions
  • Give tranexamic acid for minor bleeds/prior to surgery
19
Q

What is immune thrombocytopenia purpura?

A

Autoimmune cdtn characterised by reduction in number of circulating platelets - T2 hypersensitivity reaction.

20
Q

Primary causes of ITP?

A

viral infection or following immunisation

21
Q

Sx of ITP?

A
  • Easy or excessive bruising (purpura)
  • Superficial bleeding into skin that appears as rash of pinpoint-sized reddish-purple spots (petechiae) (usually on the lower legs)
  • Prolonged bleeding from cuts
  • Spontaneous bleeding from the gums or nose
  • Blood in urine or stools
  • heavy menstrual flow

ITP presents with mucocutaneous bleeding (rather than e.g. haemarthrosis which is often associated with defects in the coagulation cascade like in haemophilia)

22
Q

Ix for ITP?

A
  • FBC - platelet count
  • Blood film
  • Infl. markers
  • Bone marrow biopsy
23
Q

Tx of ITP

A
  1. Usually no tx require as pts return to normal spontaneously
  2. If severe (platelets<10):
    * Prednisolone
    * IVIG
    * Blood transfusions
    * Platelet transfusions - only works temporarily
24
Q

What medications should someone with ITP avoid?

A

NSAIDs, aspirin and blood thinning medications

25
Q

Complications of ITP?

A
  • Chronic ITP
  • Anaemia
  • Intracranial + subarachnoid haemorrhage
  • GI bleeding

Phase 3a - Paediatrics (14 decks)

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