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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. Ashby differential agglutination Radioactive labels esp Cr 51 Cr 51 RBC destruction / isotope elution

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Haemolytic Anaemia

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  1. Haemolytic Anaemia • “Anaemia” due to decreased RBC survival (normal 120 days) • If compensation complete anaemia may be absent • Marrow can compenaste x 8

  2. 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

  3. Sites of RBC destruction • Intravascular gross RBC defect • Extravascular lesser RBC defect • RE system liver/spleen

  4. Spleen • Mechanical Filter • Metabolic Chalenge pH/hypoxia/glucose deprivation • Phagocytic cells recognise incomplete antibodies • Hypertrophy

  5. Mechanisms of RBC destruction • Membrane defects Shape/pliability Permiability to cations • Proteins to membrane – compliment/ agglutinins/incomplete AB • Chemical modification

  6. 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

  7. 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

  8. 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

  9. 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

  10. Congenital Spherocytosis • Autosomal Dominent • RBC membrane defect -- protein / lipid • Cation pumps Na out / K in • ATP from glucose required

  11. Spherocytosis -- haemolysis • Decreased deformability - surface/volume • Decreased ability to sustain glucose (ATP) deprivation

  12. Cong Spherocytosistreatment • Splencetomy

  13. Autoimmune Haem Anaemia • Autoimmunity – antibodies – attach to RBC membrane -- damage/reacts • RE bite • Resulting in membrane loss -- spherocyte

  14. Autoimmune Haem Anaemia • Older • Female/male • 25%+ of Irish Haemolysis

  15. AHA • 80% 2ndary / 20% 1ary • Anaemia +/- acute/insidious • Jaundice • Often precipitated – Infection / stress • Liver 33% • Spleen 50% • Features of underlying disease

  16. AHA • Anaemia • Spherocytosis -- smear / osmotic fragility • Bio of increased Haem catabolism • Coombs • Increased RBC regen -- retics/N RBC/ Erythroid hyperplasia

  17. AHA • Differential Acq Haem Anaemia Cong Spherocytosis • Course / Prognosis • Treatment – Primary disease Steroids Transfusion Splenectomy Folic acid

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

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