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Haemolytic Anaemia

Haemolytic Anaemia. Elliot Catchpole PCMD. Recap. Mean Cell Volume = The size of each RBC. MACROCYTIC >96. Normocytic 76-96. Microcytic <76. Haemolysis. Non- Megaloblastic. -IRON deficiency Thalassaemias Sideroblastic. -Alcohol -Liver Disease - MYLODYSPLASIA. -G6PD Deficiency

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Haemolytic Anaemia

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  1. Haemolytic Anaemia Elliot Catchpole PCMD

  2. Recap • Mean Cell Volume = The size of each RBC MACROCYTIC >96 Normocytic 76-96 Microcytic <76 Haemolysis Non-Megaloblastic • -IRON deficiency • Thalassaemias • Sideroblastic -Alcohol -Liver Disease -MYLODYSPLASIA -G6PD Deficiency -Hereditary spherocytosis -Paroxysmal Nocturnal Haemoglobinurea -Autoimmune Haemolysis -Sickle Cell Megaloblastic B12andFolate Chronic Disease

  3. What is haemolysis?

  4. Haemoglobin Cycle Iron Bilirubin Haem Blood ‘Unconjugated/indirect’ Haem Bilirubin ALBUMIN Bilirubin LIVER Bilirubin SPLEEN CONJUGATION!!! Water solubility ‘Conjugated/direct’ K (destruction) GI Urobilinogen

  5. Haemolysis (Intravascular) LDH Haptoglobin Haemolysis Indirect Bilirubin MCV = Normal/raised Generally ACUTE Reticulocyte • ?Dark urine? • ?Jaundice? • Gallstone risk

  6. Intravascular Sickle Cell • CAUSES: SCD = homozygote, Trait = heterozygote • *PRESENTATION* - Trait = only haematuria in normal circumstancesSCD = PAIN Parvovirus B19 = HCT drops precipitously • INVESTIGATIONS – Blood smear – best initialShows Sickled cells for SCD, nothing for trait - Hb electrophoresis • TREATMENT– Pain = Supportive (O2, fluids, pain meds) Fever/infection = above + antibiotics Parvovirus = Transfusion/Ig for virusCNS = give exchange transfusionProphylaxis = Folate + Hydroxyurea • COMPLICATIONS - GallstonesOcclusion problems = leg ulcers, osteomyelitis, CNS complications (stroke, visual disturbance)

  7. Either Autoimmune Haemolysis • CAUSES: Autoimmune – PHx of autoimmune conditions, medications (penicillin, quinidine) • *PRESENTATION*Warm = chronic haemolysis (extravascular)Cold = in the cold (<4*) (intravascular) • INVESTIGATIONS – COOMBS TEST based on the 2 risks (autoimmune Hx and medications) • TREATMENT – Steroids  stop  Recurs? More steroids  stop  Constant recurrence?  SPLENECTOMY

  8. Extravascular Hereditary Spherocytosis • CAUSES: Autosomal dominant RBC membrane defect. • *PRESENTATION* - Young – extravascular – splenomegaly, jaundice • INVESTIGATIONS – Osmotic fragility test – cells will burst • TREATMENT – Splenectomy

  9. PNH (Paroxysmal Nocturnal Haemoglobinurea) Intra. • CAUSE: Genetic defect in creating DAF/CD55 • *PRESENTATION* - Dark urine IN MORNING (haemolysis at night) • INVESTIGATIONS – Test for DAF/CD55 • TREATMENT + Steroids + Anticoagulation (prevent thrombosis)+ SCT (stem cell transplant, curative) • COMPLICATIONS – Large vessel thrombosis (so give anticoagulants) - Associated with Leukaemia and Aplastic Anaemia

  10. G6PD deficiency Intravascular • CAUSES: X-linked recessive – defective G6PD enzyme • *PRESENTATION* - Oxidising crisis (jaundice + anaemia) on oxidising stressors (e.g. Henna, infection) • INVESTIGATIONS – G6PD levels >8weeks ‘Heinz Body’ smear Exclude other causes • TREATMENT – Avoid stressors

  11. Questions • A patient comes to the ED with sudden onset of fatigue and SOB. The HCT is 20 and the MCV is slightly elevated. The reticulocyte count, LDH, and indirect bilirubin are all elevated, with a low haptoglobin.1) History SLE and Lymphoma2) Recurrent episodes with a large spleen, with a European family history 3) Sudden onset of haemolysis in a male with acute infection. A similar reaction occurs when certain medications are used.

  12. Questions • An African American man comes into the ED with back, chest and thigh pain. He has a history of sickle-cell disease. He has a temperature of 38.5C. CXR and urinalysis are normal. Oxygen, fluids and analgesics are started. His HCT is 28.1) What is the most urgent step at this time?2) How would you confirm a Parvovirus infection?3) How would you treat this?

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