1. Haemolytic Anaemia
Elliot Catchpole
PCMD

2. 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

3. What is haemolysis?

4. 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

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

6. 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)

7. 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

8. 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

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 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

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 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.

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 ) What is the most urgent step at this time? 2) How would you confirm a Parvovirus infection? 3) How would you treat this?