Haemolytic anaemia Flashcards

1
Q

name 5 inherited haemolytic anaemia

A
  • Hereditary Spherocytosis
  • Hereditary Elliptocytosis
  • Thalassaemia
  • G6PD deficiency
  • Sickle cell anaemia
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2
Q

3 features of haemolytic anaemia

A
  • anaemia
  • splenomegaly (due to spleen filling with destroyed blood cells)
  • jaundice
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3
Q

blood film result in haemolytic anaemia

A

schistocytes

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4
Q

direct Coombs test in autoimmune haemolytic anaemia?

A

positive

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5
Q

Hereditary Spherocytosis inheritance?

A

autosomal dominant

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6
Q

shape of red blood cell in hereditary spherocytosis (from what to what)

A

biconcave disc to sphere shaped

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7
Q

what can precipitate aplastic crisis?

A

Parovirus infection

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8
Q

presentation of Hereditary Spherocytosis? (4)

A
  • jaundice
  • splenomegaly
  • gall stones
  • aplastic crisis
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9
Q

MCHC & reticulocytes in Hereditary Spherocytosis?

A
high MCHC
high reticulocytes (rapid turnover of cells)
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10
Q

management of Hereditary Spherocytosis

A
  • folate supplementation

- splenectomy

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11
Q

inheritance of G6PD Deficiency?

A

x linked recessive

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12
Q

triggers for G6PD Deficiency crises? (3)

A
  • infections
  • fava beans
  • medications
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13
Q

what is seen on blood film with G6PD Deficiency?

A

Heinz bodies

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14
Q

presentation of G6PD Deficiency?

A
  • neonatal jaundice
  • gall stones
  • anaemia
  • splenomegaly
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15
Q

medications that trigger G6PD Deficiency related haemolysis?

A
  • primaquine
  • ciprofloxacin
  • sulfonyureas
  • sulfasalazine
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16
Q

2 types of autoimmune haemolytic anaemia? and why

A

warm & cold

depending on the temperature at which the autoantibodies that attack the red blood cells function at

17
Q

causes of Warm Type Autoimmune Haemolytic Anaemia

A

idiopathic

18
Q

what is cold autoimmune haemolytic anaemia also called?

A

cold agglutinin disease

19
Q

what is cold AIHA often secondary to?

A
  • Lymphoma
  • Leukaemia
  • lupus
  • infections: CMV, EBV, HIV, mycoplasma
20
Q

management of AIHA

A
  • blood transfusions
  • steroids
  • rituximab
  • splenectomy
21
Q

Rituximab mechanism

A

Monoclonal antibody against B cells

22
Q

Inheritance of sick cell anaemia?

A

Autosomal recessive

23
Q

when is fetal haemoglobin (HbF) usually replaced by Haemoglobin A

A

6 weeks of life

24
Q

abnormal gene in sickle cell anaemia?

A

gene for beta globin, on chromosome 11

25
Q

what is the relation between sickle cell anaemia & malaria

A

having one copy of the gene/sickle cell trait, makes you more likely to survive malaria = selective advantage in malaria prevalent areas (e.g. Africa, India, Middle east, carribean)

26
Q

triggers of vaso-occlusive sickle crisis? (4)

A
  • cold
  • dehydration
  • infection
  • hypoxia
27
Q

general management of Sickle cell anaemia

A
  • maintain hydration & warmth
  • phenoxymethypenicillin
  • Hydroxycarbamide
  • blood transfusion
  • bone marrow transplant
28
Q

how to prevent infections in sickle cell patients?

A

Penicillin V/Phenoxymethylpenicillin OD

29
Q

Management of sickle cell crises?

A
  • admit
  • treat infection
  • keep warm & maintain hydration
  • analgesia
  • priapism
30
Q

role of priapism in sick cell crises?

A

in a crises, the sickle blood cells clump together and block/affect the blood flow in penis… to manage this; ‘priapism’ - blood aspiration

31
Q

what is a vaso-occlusive crisis?

A

sickle shaped blood cells clog up capillaries causing reduced blood perfusion distal to the blockage –> ischaemia

32
Q

what happens in a splenic sequestration crisis?

A

red blood cells clump together and block blood flow within spleen. The pooling of blood in the spleen can lead to a severe anaemia and circulatory collapse.

33
Q

what is aplastic crisis & what triggers it?

A
  • temporary loss of creation of new RBC

- triggered by Parovirus B19 infection

34
Q

what does Parovirus B19 infection trigger

A

aplastic crisis

35
Q

diagnostic critera for acute chest syndrome (2)

A
  • fever/ resp symptoms

- new infiltrates seen on x-ray

36
Q

management of acute chest syndrome (4)

A

1) infection management: abx/antivirals
2) blood transfusion
3) NIV
4) incentive spirometry