3 Red cell disorders Flashcards

1
Q

Define anaemia

A

It is a reduced level of haemoglobin

- common clinical conditions

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2
Q

List the symptoms of anaemia (depend on severity)

A
  • Shortness of breath
  • Weakness/Lethargy
  • Tachycardia
    [Skin colour is not a reliable sign - as this is determined by blood flow]
  • Nail bed and conjunctiva may be pale

Severe anaemia in elderly subjects may cause angina

  • Glossitis (pain red tongue) + angular cheilitis (fissures at the corner of mouth)
  • DPG may be elevated in RBC’s so O2 is more readily given up to tissues
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3
Q

Describe Iron deficiency anaemia

A

(Fe is needed for haemoglobin)
Fe deficient anaemia is a Form of Microcytic anaemia:
- decreased mean corpuscular volume

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4
Q

List some causes of Iron-deficiency anaemia

A

Causes:
(normally diet = need AND input = output)
BUT;
- If input reduced - poor diet, surgical removal of the stomach
- OR output increased:
> menstruation, GI bleeding (ulcers from use of NSAIDs), colon cancer (50% seen with anaemia)
- OR demand increased (pregnancy):
> There may be iron deficiency, when bone marrow + macrophage Fe stores depleted, then anaemia occurs (or folate deficiency - macrocytic anaemia)

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5
Q

Explain how chronic inflammation may lead to chronic disease anaemia

A

If there is an inflammatory condition of the bowel -

  • The inflammation causes the release of cytokine IL-1 and IL-6
  • This affects the production of the enzyme hepcidin, which regulates the uptake of iron

This is another form of microcytic anaemia

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6
Q

How can Fe-deficient anaemia be differentiated from chronic disease anaemia?

A

Doing a blood test:

  • The iron storage is measured in the bloodstream by ferritin (levels)
  • Microcytic anaemia and low ferritin levels mean = Fe deficient anaemia
  • Microcytic anaemia and normal ferritin levels mean = Chronic disease anaemia
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7
Q

Describe the differences between acute and chronic bleeds

A

Acute - rapid blood loss e.g. haemorrhage

  • Severe loss of blood
  • BP falls
  • Over time there is haemodilution

Chronic - long term bleeding e.g. gastric bleeding/excessive menstruation

  • Regular basis - does not drop BP
  • V rarely causes haemodilution

As chronic bleed gives the body time to adapt (due to longer time)

  • In acute blood loss - lose a bit of everything
  • So chronic - compensate with the loss of volume
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8
Q

Give some treatments of iron deficiency

A
  • Find and treat the underlying cause (e.g. find the cause of GI bleed + stop etc.)
  • Replace lost Fe - lost oral administration in form of FeSO4 (ferrous sulfate)
    > GI tract is the normal entry for FE entry in diet
    > Guarantee longer time for efficacy
    > If px is intolerant to Iron tablets, administer IV Fe
  • Prophylaxis in pregnancy - oral Fe with Folic Acid widely used
  • Very rarely - transfusion needed
    > If Fe deficiency is not diagnosed for a long time, and anaemia is long term
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9
Q

Describe Renal Anaemia

A

The kidney responds to the level of RBC, so if RBC is low, the kidney releases more EPO (erythropoietin)

  • BUT if the kidney is affected by a condition where its function is impaired
  • The EPO level will drop
  • Leading to the development of renal anaemia - ‘Normocytic anaemia’

Treatment
- with Fe and EPO (leads to the stimulation of the bone marrow to produce RBCs)

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10
Q

Discuss pregnancy and anaemia

A

There could be a potential Fe and Folate deficiency
- Maybe normocytic anaemia as:
> Pregnancy increases MCV
> BUT iron deficiency decrease MCV

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11
Q

Describe megaloblastic anaemia

A

It is an abnormal RBC maturation due to defective DNA synthesis, that is out of step with cytoplasmic development: bone marrow megaloblasts (v large cells)

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12
Q

Describe macrocytic anaemia

A

It is due to Vit. B12 or Folate deficiency
- Increased incidence of alcohol abuse

It gives rise to anaemia, jaundice (excess breakdown of Hb due to ineffective erythropoiesis)

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13
Q

Explain the significance of Vit. B12

A

Vitamin B12 is an essential cofactor for purine and pyrimidine synthesis (cell division).

  • Comes from animal products
  • Requires (Castle’s) intrinsic factor for absorption (absent after gastrectomy)
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14
Q

Explain the significance of folate, and its uses

A

Folate (folic acid) - essential for thymidylate synthesis

  • (a rate-limiting step in DNA synthesis, as thymidine, is a pyrimidine base)
  • It is found in most food (especially the liver, greens, yeast, marmite)

Drugs that can affect this process:
- Methotrexate - inhibits dihydrofolate reductase (therefore folate regeneration is impaired - treat with folinic acid)

Vit B12 and Folate come from diet

  • if there is a poor diet, replace B12 or Fe orally (intake problem)
  • BUT if malabsorption) - IV, intramuscular
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15
Q

Describe haemolytic anaemia

A

It is an increased rate of RBC destruction
Potential causes:
- Spherocytosis - genetic; abnormal reduction in RBC membrane protein (spectrin) - cells become fragile
- Acquired - hemolytic transfusion reaction, malaria, drug-induced
- Jaundice and enlarged spleen

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16
Q

Describe jaundice and its relation to haemolytic anaemia

A

There is an increase in LDH (an enzyme in RBC) that is released in response to RBC breakdown
- SO bone marrow responds to breakdown, by making more RBCs
> BUT they release immature status of RBC
- This results in an increase in reticulocyte levels
- Product of catabolism of haemolysis - increase in Bilirubin

So, the production of bilirubin increases when large quantities of RBC’s are broken down

17
Q

Describe autoimmune anaemia

A

How to identify it:

  • Ab attached to RBC membrane - causes a breakdown
  • To detect these Ab’s: COOMBS test (direct + indirect)
  • depending on if you want to detect Ab on RBC surface or in serum
18
Q

Describe sickle cell anaemia

A

It is genetic:
- Due to a single nucleotide pleomorphism
- Which generates an amino acid substitution
> Valine for glutamic acid

  • This results in the production of abnormal Hb (Hb S)
    > It forms crystals at low O2

RBC’s form sickle shapes and may block microcirculation
- e.g. in the spleen

It can cause haemolytic anaemia

19
Q

Describe thaalassaemia’s

A

It is a form of microcytic anaemia

Genetic condition:
- There is a reduced rate of a or B globin unit production
(results in many variations)
- Deletion of both a-genes leads to death in the uterus as Hb (y4) produced
- One a-deletion results in:
> Reduced RBC volume and Haematocrit

20
Q

Describe aplastic anaemias

A

Panctocytopenia: is the insufficient production of RBC’s, WBC’s, and platelets

(Pure red cell aplasia is the insufficient production of RBC’s)

This can result in:

  • Decreased resistance to infection
  • Increased bleeding
  • Increased tiredness
21
Q

Describe how one may get aplastic anaemias

A

It is a mostly acquired disease:

  • Viral, radiation or drugs
  • Cytotoxic. (anti-cancer drugs)
  • Chloramphenicol
  • Sulphonamides
  • Insecticides
22
Q

List some treatments of aplastic anaemias

A
  • Bone marrow transplant (with tissue match)
  • Immunosuppressant - to prevent immune destruction of stem cells
  • Colony-stimulating factor - increases WBC count
23
Q

Describe Polycythaemia

A
  • Increased Hb count and haematocrit (>55 in males and > 47 in females
  • Increased blood viscosity - poor tissue perfusion
24
Q

List some signs and symptoms of polycythaemia

A
  • Ruddy appearance
  • Cyanosis
  • Headaches
  • Blurred vision
  • Hypertension
25
Q

Give causes of polycythaemia

A

Primary:

  • changes in bone marrow
  • stem cell defect

Secondary:
- increased Erythropoietin
(altitude, smoking, renal carcinoma)

26
Q

Give treatments to polycythemia

A

Primary:

  • Venesection (bleeding)
  • Cytotoxic agents (myelosuppression)