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Haematology > Red Cells 1 > Flashcards

Red Cells 1 Flashcards

1
Q

What is anaemia

A

Reduction in RBC or their Haemoglobin content

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2
Q

Aetiology of anaemia

A
  • Blood loss
  • Increased destruction
  • Reduced production
  • Defective production
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3
Q

4 important substances for RBC production

A
  • Iron
  • Vit B12
  • Folic acid
  • Erythropoietin
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4
Q

What happens to RBCs in hereditary sypherocytosis

A
  • They become spherical
  • Due to defects in 5 different structural proteins
  • Ankyrin
  • Alpha spectrin
  • Beta spectrin
  • Band 3
  • Protein 4.2
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5
Q

Presentation of hereditary spherocytosis

A
  • Anaemia
  • Jaundice
  • Splenomegaly
  • Pigment gallstones
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6
Q

Rx of hereditary spherocytosis

A
  • Folic acid
  • Transfusion
  • Splenectomy
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7
Q

Function of Glucose 6 Phosphate Dehydrogenase (G6PD)

A

Protects RBCs from oxidative damage

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8
Q

Commonesnt disease causing enzymopathy in the world

A

G6PD Deficiency

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9
Q

What happens to RBCs in G6PD deficiecy

A
  • Cells are vulnerable to oxidative damage
  • Protects against malaria (most common in malarial areas)
  • RBCs look like “Bite and Blister cells”
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10
Q

How is G6PD deficiency passed on

A
  • X-linked

- Affects males (female carriers)

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11
Q

Presentation of G6PD

A

-Neonatal jaundice
-Splenomegaly
-Pigment gallstones
-DRUG or broad bean or infection precipitated jaundice + anaemia
(Intravascular haemolysis + Haemoglobinuria)

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12
Q

Triggers of G6PD

A

-Infection
-Acute illness (DKA)
-Broad (Fava) Beans “favism”
-Drugs
Antimalrials (primaquine)
Antibacterials (nitrofurantoin)
Analgesics (aspirin)
-Sulphonamides and sulphones (Sulfasalazine)

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13
Q

Haemoglobin function

A

Gas exchange (O2 to tissues, CO2 to lungs)

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14
Q

Describe the Bohr effect

A

Haemoglobin’s oxygen binding affinity is inversely related to acidity + concentration of CO2

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15
Q

What has a great affinity for 2, HbF or HbA

A

HbF

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16
Q

What is normal adult haemoglobin composed of

A
  • Haem molecule
  • 2 alpha chains + 4 alpha genes
  • 2 beta chains + 2 beta genes
17
Q

What happens to haemoglobin in thalassaemia

A

Reduced or absent globin chain production

18
Q

What happens to haemoglonbin in Sickle cell disease

A

Structurally abnormal globin chain

19
Q

What is haemoglobin composed of in sickle cell disease

A
  • Haem molecule
  • 2 alpha chains
  • 2 beta (sickle) chains
20
Q

Pathogenesis of sickle cell disease

A
  • HbS polymerises when deoxygenated
  • Causing RBCs to deform, producing sickle cells
  • They’re fragile and haemolyse + occlude small vessels
21
Q

3 types of sickle cell crisis

A
  • Vaso-occlusive “painful” crisis
  • Aplastic crisis
  • Sequestration crisis
22
Q

Describe vaso-occlusive “painful” crisis

A
  • Common, due to microvascualr occlusion
  • Severe pain
  • Occlusion can cause mesenteric ischaemia (mimicing an acute abdome)
  • CNS infarction
  • Avascular necrosis of femoral head + leg ucers
  • Children <3y dactylitis (if hands + feet affected)
  • Priaprism
23
Q

Describe aplastic crisis

A
  • Due to parvovirus B19
  • With sudden reduction in bone marrow production (sep. RBCs)
  • Usually self limitting , <2wks
  • Transfusion may be needed
24
Q

Describe sequestration crisis

A
  • Mainly affects children as the spleen has not yet undergone atrophy
  • Pooling of blood in spleen +/- liver
  • Organomegaly
  • Severe anaemia + shock
  • Urgent transfusion required
25
Q

Life expectancy in sickle cell disease

A
  • Male early 40s
  • Female Late 40s

(childhood + perinatal mortality contribute to this reduction)

26
Q

Management of painful crisis

A
  • Analgesia (severe = opiates)
  • Hyrdation
  • O2
  • Consider antibiotics

Give analgesia within 30mins of presentation + avoid pethidine (demerol)

27
Q

Presentation of a chest crisis

A
  • Chest pain
  • Fever
  • Worsening hypoxia
  • Infiltrates of CXR
28
Q

Rx of a chest crisis

A
  • Resp. support
  • Antibiotics
  • IV fluids
  • Analgesia
  • Transfusion
29
Q

Management of sickle cell disease

A

Life long prophylaxis

  • Vaccination
  • Penicillin + malarial prophylaxis
  • Folic acid

Acute events

  • Hydration
  • O2
  • Prompt Rx of infection
  • Analgesia (opiates, NSAIDS)

(possible bone marrow transplant)

30
Q

Disease modifying drug for sickle cell disease

A

Hydroxycarbamide

Haematology (24 decks)

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