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Semester 4: Haematology > haemolytic anaemias > Flashcards

haemolytic anaemias Flashcards

1
Q

haemolytic anaemia

A

anaemia due to reduced RBC survival

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2
Q

describe the normal RBC lifestyle

A
  1. RBC production (Iron, B12/Folate, Globin chains, Protoporphyrins)
  2. RBCs circulate for 120 days
  3. Removal of old RBCs (membrane changes are detected by macrophages)
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3
Q

Normal Red blood cell

A
  1. biconcave disc shape
  2. haemoglobin (carries o2)
  3. Metabolic pathways (ensuring RBC maintains structure and function)
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4
Q

what is haemolysis?

A

destruction of RBCs -> bone marrow compensates by increasing RBC production -> increased immature RBCs in the circulation (reticulecytosis, nucleated RBCs)

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5
Q

compensated haemolysis?

A

RBC production is able to compensate for the decrease in lifespan and so there is normal Hb levels

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6
Q

incompletely compensated haemolysis

A

RBC production is unable to keep up with the decreased Hb lifespan and so there is decreased Hb levels

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7
Q

clinical findings of haemolytic anaemia

A
  • jaundice (excess bilirubin which is a breakdown of haemoglobin)
  • pallor/fatigue
  • splenomegaly (increased workload lead yo an enlarged spleen)
  • dark urine: haemoglobinuria
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8
Q

chronic findings of haemolytic anaemia

A
  • gallstones due to the accumulation of pigment (more bilirubin)
  • leg ulcers (NO scavenging)
  • Folate deficiency (using more folate to make RBCs)
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9
Q

laboratory findings of haemolytic anaemia

A
  • Increased reticulocyte count
  • Increased unconjugated bilirubin
  • Increased LDH (lactate dehydrogenase)
  • Low serum haptoglobin
  • Increased urobilinogen
  • Increased urinary haemosiderin
  • Abnormal blood film
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10
Q

blood film of haemolytic anaemia

A
  • reticulocytes
  • polychromasia (high no of immature RBCs
  • nucleated RBC
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11
Q

Spherocytosis

A

RBCs are sphere-shaped rather than bi-concave disk shaped as normal

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12
Q

Elliptocytosis

A

red blood cells are elliptical rather than the typical biconcave disc shape.

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13
Q

what are the 3 components of red cell membrane structure

A

Lipid bilayer
Integral proteins
Membrane skeleton

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14
Q

defects in vertical interaction proteins leads to

A

hereditary spherocytosis

  • Spectrin
  • Band 3
  • Protein 4,2
  • Ankyrin
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15
Q

defects in the horizontal interaction proteins leads to

A

hereditary elliptocytosis

  • Protein 4.1
  • Glycophorin C
  • Spectrin
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16
Q

hereditary spherocytosis

A

inherited in autosomal dominant fashion
bone marrow makes the biconcave RBC as normal, but as it goes round the circulation the membrane is lost and the RBC becomes spherical

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17
Q

clinical features of hereditary spherocytosis

A
  • Asymptomatic to severe haemolysis
  • Neonatal jaundice
  • Jaundice, splenomegaly, pigment gallstones
  • Reduced eosin-5-maleimide (EMA) binding – binds to band 3
  • Positive family history
  • Negative direct antibody test
18
Q

management of hereditary spherocytosis

A

Monitor
Folic acid
Transfusion
Splenectomy

19
Q

Glucose-6-phosphate deficiency

A
  • Red blood cells break down (hemolysis) when the body is exposed to certain foods, drugs, infections or stress
  • Only has an effect when exposed to oxidant radicals: which denatures Hb forming aggregates & forms Heinz bodies
  • Oxidised membrane proteins which reduces RBC deformability
20
Q

G6PD deficiency

A

X-linked disorder

21
Q

prevalence of G6PD deficiency

A
  • Common in African, Asian, Mediterranean and Middle Eastern populations
  • Mild in African (type A), more severe in Mediterraneans (type B)
22
Q

Clinical features of G6PD deficiency

A

range from asymptomatic to acute episodes to chronic haemolysis

23
Q

What can trigger symptoms of G6PD

A 
Infections
Fava/ broad beans
Many drugs e.g.:
Dapsone
Nitrofurantoin
Ciprofloxacin
Primaquine
24
Q

Blood film of G6PD

A

Bite cells
Blister cells & ghost cells
Heinz bodies (methylene blue)

25
pyruvate kinase deficiency
PK required to generate ATP | Essential for membrane cation pumps (deformability)
26
genetics of PKD
autosomal deficiency
27
Haemoglobinopathies
a group of recessively inherited genetic conditions affecting the haemoglobin component of blood. They are caused by a genetic change (mutation) in the haemoglobin 
28
Structure of haemoglobin
Ferrous iron + Protoporphyrin = Haem 2a + 2B = Globin Haem + Globin = Haemoglobin
29
Thalassaemias
Production increased/ decreased amount of a globin chain (structurally normal) - excess unpaired globin chains are unstable - RBCS damaged - Ineffective erthropoeisis - Haemolytic anaemia
30
Variant haemoglobins
Production of a structurally abnormal globin chain
31
two types of beta thalassaemia
Beta thalassemia trait: one gene on chromosome 16 | Beta thalassemia major: two genes for beta thalassemia and no normal beta-chain gene (homozygous)
32
diagnosis of thalassaemia trait
``` Asymptomatic Microcytic hypochromic anaemia Low Hb, MCV, MCH Increased RBC Often confused with Fe deficiency HbA2 increased in b-thal trait –(diagnostic) a-thal trait often by exclusion globin chain synthesis (rarely done now) DNA studies (expensive) ```
33
beta thalassaemia trait
- need transfusion in the first day of life - If don't get a blood transfusion: * Failure to thrive * Progressive hepatosplenomegaly * Bone marrow expansion – skeletal abnormalities * Death in 1st 5 years of life from anaemia
34
side effect of blood transfusion
Iron overload Endocrinopathies Heart failure Liver cirrhosis
35
sickle cell disease
- Point mutation in the β globin gene: glutamic acid → valine - Insoluble haemoglobin tetramer when deoxygenated → polymerisation - sickle shaped cells
36
clinical features of SCD
``` Painful crises Aplastic crises Infections Acute sickling: Chest syndrome Splenic sequestration Stroke Chronic sickling effects: Renal failure Avascular necrosis bone ```
37
Laboratory features of SCD
``` Anaemia Hb often 65-85 Reticulocytosis Increased NRBC Raised bilirubin Low creatinine ```
38
how to confirm diagnosis of SCD
Solubility test Expose blood to reducing agent Hb S precipitated Positive in trait and disease
39
autoimmune haemolysis
``` Idiopathic Usually warm IgG, IgM Drug-mediated Cancer associated LPDs ```
40
Alloimmune haemolysis
``` Transplacental transfer: Haemolytic disease of the newborn: D, c, L ABO incompatability Transfusion related Acute haemolytic transfusion reaction ABO Delayed haemolytic transfusion reaction E.g Rh groups, Duffy ```

Semester 4: Haematology (5 decks)

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