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Haemolytic Anaemia. “Anaemia” due to decreased RBC survival (normal 120 days) If compensation complete anaemia may be absent Marrow can compenaste x 8. RBC Survival. Ashby differential agglutination Radioactive labels esp Cr 51 Cr 51 RBC destruction / isotope elution

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haemolytic anaemia
Haemolytic Anaemia
  • “Anaemia” due to decreased RBC survival (normal 120 days)
  • If compensation complete anaemia may be absent
  • Marrow can compenaste x 8
rbc survival
RBC Survival
  • Ashby differential agglutination
  • Radioactive labels esp Cr 51
  • Cr 51 RBC destruction / isotope elution
  • RBC survival 120 days
  • Cr 51 ½ life 25-30 days
sites of rbc destruction
Sites of RBC destruction
  • Intravascular gross RBC defect
  • Extravascular lesser RBC defect
  • RE system liver/spleen
spleen
Spleen
  • Mechanical Filter
  • Metabolic Chalenge pH/hypoxia/glucose deprivation
  • Phagocytic cells recognise incomplete antibodies
  • Hypertrophy
mechanisms of rbc destruction
Mechanisms of RBC destruction
  • Membrane defects

Shape/pliability

Permiability to cations

  • Proteins to membrane – compliment/ agglutinins/incomplete AB
  • Chemical modification
mechanisms of rbc destruction 2
Mechanisms of RBC destruction 2
  • Abn of Hb influencing flow

Hb with inccreased visc

Pppt Hb

  • Physical factors extrinsic to RBC

External trauma

Heart valve

Arteriolar disease

Fibrin

haemolytic anaemia general
Haemolytic Anaemiageneral
  • Anaemia may be absent

may be acute / chronic

  • Haem catabolism

indirect bilirubin / jaundice

Gall stones – pigment

Urinary / faecal urobilinogen

  • Erythroid hyperplasia

Marrow activity / bone changes

Reticulocytosis

Folate requirements

Iron absorption (no iron def)

  • Aplastic crises
haemolytic anaemia classification intrinsic
Haemolytic Anaemia ClassificationIntrinsic
  • Abn. Cell shape

Cong Spherocytosis/Ovalocytosis

Acanthocytosis

  • Red cell Enzyme defects

G6PD def

PK def

  • Hb-opathies

Quantitative -- Thalasaemias

Qualitative -- Sickle cell, Unstable etc

  • Porphyria
  • Paroxysmal Nocturnal Haemoglobinuria
haemolytic anaemia classification extrinsic
Haemolytic AnaemiaClassification Extrinsic
  • Mechanical Disruption

March Hburia

Vasc prosthesis

Microangiopathic

Burns

  • Disruption via chemicals/micro organisms
  • Antibody mediated (Acquired HA)

Warm

Cold

Drugs

Erythroblastosis foetalis

  • RE (spleen) sequestration

Hypersplenism

Acute infection

congenital spherocytosis
Congenital Spherocytosis
  • Autosomal Dominent
  • RBC membrane defect -- protein / lipid
  • Cation pumps Na out / K in
  • ATP from glucose required
spherocytosis haemolysis
Spherocytosis -- haemolysis
  • Decreased deformability - surface/volume
  • Decreased ability to sustain glucose (ATP) deprivation
autoimmune haem anaemia
Autoimmune Haem Anaemia
  • Autoimmunity – antibodies – attach to RBC membrane -- damage/reacts
  • RE bite
  • Resulting in membrane loss -- spherocyte
autoimmune haem anaemia1
Autoimmune Haem Anaemia
  • Older
  • Female/male
  • 25%+ of Irish Haemolysis
slide23
AHA
  • 80% 2ndary / 20% 1ary
  • Anaemia +/- acute/insidious
  • Jaundice
  • Often precipitated – Infection / stress
  • Liver 33%
  • Spleen 50%
  • Features of underlying disease
slide24
AHA
  • Anaemia
  • Spherocytosis -- smear / osmotic fragility
  • Bio of increased Haem catabolism
  • Coombs
  • Increased RBC regen -- retics/N RBC/ Erythroid hyperplasia
slide25
AHA
  • Differential

Acq Haem Anaemia

Cong Spherocytosis

  • Course / Prognosis
  • Treatment – Primary disease

Steroids

Transfusion

Splenectomy

Folic acid

coombs test
Coombs Test
  • Detects IgG / Compliment on RBC membrane by using a polyvalent antibody to agglutinate cells if positive