Normocytic anaemia Flashcards

1
Q

Causes of normocytic anaemia

A
  • Acute blood loss
  • Haemolytic anaemia
  • Anaemia of chronic disease
  • Chronic kidney disease
  • Aplastic anaemia
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2
Q

What are the two mechanisms of normocytic anaemia and how do you differentiate the two?

A

Increased destruction (haemolysis) = Increased reticulocytes

or

Reduced production (bone marrow failure) = Reduced reticulocytes

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3
Q

Causes of normocytic anaemia with increased reticulocytes

A
  • Hereditary
    • Hereditary spherocytosis
    • Sickle cell anaemia
    • Glucose-6-phosphate dehydrogenase deficiency
    • Pyruvate kinase deficiency

Acquired

  • Autoimmune haemolytic anaemia
  • Paroxysmal nocturnal haemoglobinuria
  • Micro/macroangiopathic haemolytic anaemia
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4
Q

Causes of normocytic anaemia with reduced reticulocytes

A
  • Chronic renal failure
  • Aplastic anaemia
  • Myelophthisic process
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5
Q

Symptoms of anaemia

A
  • Fatigue
  • SOB on exertion
  • Chest pain
  • Palpitations
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6
Q

Signs of anaemia

A
  • Tachycardia
  • Tachypnoea
  • Hypotension
  • Pallor
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7
Q

General investigations in anaemia

A

Bedside

  • Full set of observations

Bloods

  • FBC: reduced Hb. Assess MCV
  • Blood film
  • Iron studies
  • B12 and folate levels
  • Haemolysis screen: bilirubin, haptoglobin, Coombs test
  • U&Es: CKD
  • TFTs: hypothyroidism
  • LFTs: chronic liver disease

Imaging

  • Assess for site of blood loss

Special tests

  • Bone marrow biops
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8
Q

Definition of hereditary spherocytosis

A

Inherited defect in RBC membrane proteins leading to a haemolytic anaemia

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9
Q

Epidemiology of hereditary spherocytosis

A
  • Northern European and North American populations
  • 1 in 2000 people
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10
Q

Clinical features of herediatary spherocytosis

A
  • General features of anaemia
  • Neonatal jaundice
  • Splenomegaly
  • Gallstones
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11
Q

Investigations in hereditary spherocytosis

FBC

Blood film

LFTs

LDH

A
  • FBC: normocytic anaemia
    • Raised reticulocyte count
  • Blood film: spherocytes
  • LFTs: raised bilirubin
  • LDH: raised
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12
Q

Managment of hereditary spherocytosis

A
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13
Q

Define sickle cell anaemia.

What is meant by sickle cell disease, sickle cell trait and sickle cell crisis?

A
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14
Q

Epidemiology of sickle cell disease

A
  • Sickle cell trait: highest incidence in Sub-Saharan Africa
  • 8% of Afro-Caribbean people carry the sickle cell gene
    • Protective against falciparum malaria
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15
Q

Clinical features of sickle cell anaemia

A
  • General features of anaemia
  • Jaundice
  • Failure to thrive
  • Frontal bossing
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16
Q

Types of sickle cell crisis

A
  • Sequestration crisis
  • Aplastic crisis
  • Haemolytic crisis
  • Vaso-occlusive crisis
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17
Q

Features of sequestration crisis

A
18
Q

Features of aplastic anaemia

A
  • Infection with parovirus B19
  • Bone marrow suppression with reduced reticulocyte count
19
Q

Features of haemolytic crisis

A
  • Usually occurs in context of vaso-occlusive crisis
  • Increased haemolysis
  • Decreased Hb and increased reticulocytes
20
Q

Features of vaso-occlusive crisis

A
21
Q

Precipitants of vaso-occlusive crisis

A
  • Infection
  • Dehydration
  • Hypoxia
  • Acidosis
22
Q

Investigations for sickle cell anaemia

A
  • Newborn screening: Guthrie heel prick test
  • FBC:
    • Normocytic anaemia (MCV 80-95fL)
    • Increased reticulocytes
  • Blood film: sickle cells, Howell-Jolly bodies
  • Hb electrophoresis
23
Q

Genes affected, HbA, HbA2, HbF and HbS proportion in sickle cell trait and sickle cell disease

A

Trait: 1 gene; HbA 55%, HbA2 2%, HbF <2%, HbS 40%

Disease: 2 genes; HbA 0%, HbA2 2%, HbF 8%, HbS 90%

24
Q

Investigations in sickle cell crisis

A
25
Q

Management of sickle cell crisis

A
  • Supportive
    • Oxygen
    • Analgesia: often requires opiates
    • Rehydration: dehydration precipitates sickling
    • Antibiotics: particularly in chest crisis
    • Chest physiotherapy
  • Blood transfusion: in a severe crisis a blood transfusion reduces the proportion of HbS
  • Exchange transfusion: involves removal of HbS in exchange for normal Hb in a life threatening crisis eg organ damage
26
Q

Long term management of sickle cell disease

A
  • Avoid precipitants: eg infections, dehydration, hypoxia, cold
  • Pain managment: regularly prescribed medications to manage chronic pain
  • Hydroxycarbamide: increases HbF; indicated for patients with recurrent chest or pain crisis
  • Folic acid supplementation: 5mg once weekly
  • Lifelong phenoxymethylpenicillin: patients are at risk of infection from encapsulated bacteria due to hyposplenism from autosplenectomy
  • Regular vaccinations: pneumococcal vaccine every 5 years and annual influenza
27
Q

Definition of G6PD deficiency

A

Inherited X-linked recessive disorder resulting in a haemolytic anaemia.

(Results in reduced half life of RBCs)

28
Q

Epidemiology of G6PD deficiency

A
  • African, Mediterranean and Asian ethnicity
  • Prevalence of 50-70% in Kurdish Jews
  • Protetive role against malaria
29
Q

What is the life span of a red blood cell?

A

120 days

30
Q

What does G6PD do?

A

It protects the cells from oxidative damage.

Red cells are particularly prone to oxidative stress.

31
Q

Investigations in G6PD deficiency

A
32
Q

Managment of G6PD deficiency

A
  • Neonatal jaundice
    • Phototherapy or exchange transfusion
  • Blood transfusion
    • Hb <70g/L or
    • Hb <80g/L and cardiac co-morbidity
  • Folic acid
    • Daily until splenectomy
  • Splenectomy
    • Spleen removal reduces haemolysis
    • Delayed until patients are >6 years of age to reduce the risk of post splenectomy sepsis
33
Q

Define autoimmune haemolytic anaemia

A

Antibody mediated destruction of RBCs.

Divided into cold and warm depending on what temperature the anatibodies most avidly bind RBCs.

34
Q

Difference in pathophysiology between warm and cold autoimmune haemolytic anaemia.

A
  • Warm: IgG binds RBCs; Extravascular haemolysis
  • Cold: IgM binds RBCs; Extravascular and intravascular haemolysis
35
Q

Causes of warm autoimmune haemolytic anaemia

A
  • Idiopathic
  • SLE
  • CLL
  • Drugs
    • Penicillins
    • Cephalosporins
36
Q

Causes of cold autoimmune haemoglobin anaemia

A
  • Idiopathic
  • M. Pneumoniae
  • EBV
37
Q

Investigations in autoimmune haemolytic anaemia

A
  • Bloods
    • FBC:
      • Normocytic anaemia (MCV 80-95fL)
      • Increased reticulocytes
    • Blood film: spherocytes
    • LFTs: bilirubin raised
    • LDH: raised
    • Haptoglobin: reduced
  • Special test
    • Coombs test
      • Direct
      • Indirect
    • Cold aggltinin titre
38
Q

Managment of haemolytic anaemia

A
  • Treat underlying cause
  • Avoid cold in cold agglutinin disease
  • Blood transfusion
  • Immunosupressants
  • Plasmapheresis
  • Splenectomy
39
Q

Why does normocytic anaemia occur in chronic renal failure?

A

Decreased production of RBCs due to reduced EPO

40
Q

Pathophysiology of aplastic anaemia

A
41
Q

Myelophthisic process

A

Process which replaces bone marrow eg malignancy