Haemolytic Anaemia

Elliot CatchpolePCMD

Recap• Mean Cell Volume = The size of each RBC

Microcytic<76

Normocytic76-96

MACROCYTIC>96

-IRON deficiency

-Thalassaemias

-Sideroblastic

Chronic Disease

Haemolysis

B12 and

Folate

-Alcohol-Liver Disease-MYLODYSPLASIA

Megaloblastic

Non-Megaloblastic

-G6PD Deficiency-Hereditary spherocytosis-Paroxysmal Nocturnal Haemoglobinurea-Autoimmune Haemolysis-Sickle Cell

• What is haemolysis?

Haemoglobin Cycle

Haem

Iron

Bilirubin

Blood

ALBUMIN

BilirubinHaem

SPLEEN

LIVER

‘Unconjugated/indirect’

(destruction)

Bilirubin

‘Conjugated/direct’

CONJUGATION!!!Water solubility

K

Bilirubin

GIUrobilinogen

Haemolysis

HaemolysisMCV =

Normal/raised

Generally ACUTE

Indirect Bilirubin

Haptoglobin

(Intravascular)

LDH

Reticulocyte

•?Dark urine?•?Jaundice?•Gallstone risk

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 virus CNS = give exchange transfusion Prophylaxis = Folate + Hydroxyurea

• COMPLICATIONS - GallstonesOcclusion problems = leg ulcers, osteomyelitis, CNS complications (stroke, visual disturbance)

Intravascular

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

Either

Hereditary Spherocytosis

• CAUSES: Autosomal dominant RBC membrane defect.

• *PRESENTATION* - Young – extravascular – splenomegaly, jaundice

• INVESTIGATIONS – Osmotic fragility test – cells will burst

• TREATMENT – Splenectomy

Extravascular

PNH (Paroxysmal Nocturnal Haemoglobinurea)

• 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

Intra.

G6PD deficiency

• 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

Intravascular

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.

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?