Haemolytic Anaemia Elliot Catchpole PCMD

Recap Mean Cell Volume = The size of each RBC B12 and Folate 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 B12 and Folate Chronic Disease

What is haemolysis?

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

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

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

Autoimmune Haemolysis 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

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

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

G6PD deficiency CAUSES: X-linked recessive – defective G6PD enzyme 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

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 Lymphoma 2) 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.

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?