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Pathology > Haemolytic Anaemias > Flashcards

Haemolytic Anaemias Flashcards

1
Q

congenital causes of haemolytic anaemia

A
  • hereditary spherocytosis
  • hereditary ellipocytosis
  • G6PD deficiency
  • PK deficiency
  • thalassemia
  • sickle anaemia
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2
Q

acquired causes of haemolytic anaemia

A
  • autoimmune haemolytic anaemia
  • microangiopathic haemolytic anaemia
  • malaria
  • wilson’s disease
  • zieve’s syndrome
  • mechanical haemolysis
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3
Q

clinical features of haemolytic anaemia

A
  • pallor
  • fluctuating jaundice
  • dark urine
  • splenomegaly
  • gallstones
  • folate deficiency
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4
Q

lab results of haemolytic anaemia

A
  • increased unconjugated bilirubin
  • increased urine bilinogen
  • low haptoblobins
  • reticulocytosis
  • marrow erythroid hyperplasia
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5
Q

what happens when there is intravascular haemolysis?

A
  • erythrocytes destroyed directly in circulation
  • Free Hb released rapidly and saturates plasma haptoglobins
  • Free excess Hb filtered by glomeruli
  • Appears as haemoglobinuria
  • Acute renal failure
  • Laboratory features: haemoglobinuria, haemosidinuria
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6
Q

what is hereditary spherocytosis?

A
  • autosomal dominant

- defect in spectrin

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7
Q

differential diagnosis for hereditary spherocytosis

A

autoimmune haemlysis (warm) - distinguish by Coombs test

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8
Q

investigations of hereditary spherocytosis

A
  • blood film
  • radiolabelled studies
  • spectrin assays
  • osmotic fragibility tests
  • autohaemolysis test
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9
Q

treatment of hereditary spherocytosis

A
  • splenectomy

- folate supplements

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10
Q

what does Coombs test do?

A

detects the antibody that causes autoimmune haemolysis

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11
Q

expected results from HS

A
  • high bilirubin
  • reticulocyte count
  • splenomegaly
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12
Q

what are the different grades of severity?

A
  • mild: compensates well

- severe: neonatal jaundice

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13
Q

what is G6PD deficiency?

A
  • glucose-6-phosphase dehydrogenase deficiency
  • X-linked
  • rapid intravascular haemolysis precipitated by oxidative stress
  • precipitants include infection, drugs, fava beans
  • Hb is normal between crisis
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14
Q

how do you diagnose G6PD?

A
  • blood film with bite cells and Heinz bodies

- G6PD assay

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15
Q

what is paraoxysmal noctural haemoglobinuria?

A
  • rare acquired clonal effect
  • red cell membrane unduely sensitive to complement mediated lysis
  • chronic intravascular haemolysis
  • recurrent thrombosis
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16
Q

how do you diagnose paraoxysmal noctural haemoglobinuria?

A
  • flow cytometry (CD55 and CD59)

- Ham’s test

17
Q

management of PNH?

A
  • leucodepleted blood products
  • long term anticoagulation
  • eculuzumab
  • BMT
18
Q

what are the types of autoimmune HA?

A
  • warm autoantibodies (IgG)

- cold autoantibodies (IgM)

19
Q

causes of autoimmune HA

A
  • idiopathic
  • viral infections
  • mycoplasma
  • lymphoma
  • systemic autoimmune diseaes
  • exposure to certain drugs
20
Q

lab features of autoimmune HA

A
  • spherocytic cells

- bits of the red cell removed

21
Q

investigations for autoimmune HA

A
  • blood film

- DCT positive

22
Q

management of autoimmune HA

A
  • avoid transfusion
  • immunesuppression
  • splenectomy
  • folate supplements
23
Q

what is thalassemia?

A

failure to produce a globin chain

- autosomal recessive

24
Q

which types of haemoglobin molecules are affected by alpha thalassemia?

A

HbA, HbF, HbA2

25
Q

what happens if there is a globin chain imbalance?

A

the protein gets denatured

26
Q

what type of anaemia does thalassemia give you?

A

microcytic hypochromic

27
Q

expected lab results for b-thalassemia

A
  • normal Hb
  • MCV, MCH reduced
  • RBC normal or raised
  • raised HbA2
28
Q

features that a thalassemia major patient has

A
  • severe anaemia (transfusion-dependent)
  • hyperplasia of bone marrow
  • hepatosplenomegaly
  • iron overload
  • endocrine deficiencies
  • osteoporosis
29
Q

management of thalassemia major

A
  • transfusion every month
  • iron chelation (to prevent iron overload - done orally)
  • splenectomy
  • hormone replacement
  • bisphosphonates
  • folate supplementation
  • bone marrow transplant
30
Q

what is thalassemia intermedia?

A

condition which is intermediate in severity between the major and the minor

31
Q

another name for thalassemia intermedia in alpha-globin deficiency

A

HbH disease

32
Q

which amino acid substitution is in sickle cell anaemia

A

valine for glutamic acid

33
Q

heretability of sickle cell anaemia

A

autosomal recessive

34
Q

trigger for a sickle crisis

A
  • infection
  • dehydration
  • cold
  • hypoxia
35
Q

presentation of sickle cell anaemia

A
  • jaundice
  • gallstones
  • anaemia
  • asplenic (multiple infarctions)
  • organ failure (multiple infarctions)
  • leg ulcers
  • recurrent infections
  • aseptic necrosis of bone
  • chronic renal disease
  • retinopathy
36
Q

when does sickle cell anaemia present?

A

6 months of age

37
Q

management of a sickle cell crisis

A
  • oxygen (for hypoxia)
  • IV fluids
  • antibiotics
  • analgesia
  • exchange transfusion
38
Q

management of a sickle cell patient

A
  • hydroxyurea to increase HbF
  • folate supplements
  • penicillin, vaccines
  • bone marrow transplant

Pathology (21 decks)

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