Haemolytic Anaemia Flashcards
Definition of haemolytic anaemia
Anaemia due to increased destruction of RBCs
Inherited causes of haemolytic anaemia (5)
Thalassaemia SCD G6PD Hereditary spherocytosis Hereditary elliptocytosis
Acquired causes of haemolytic anaemia (5)
Autoimmune haemolytic anaemia Alloimmune haemolytic anaemia Paroxymal nocturnal haemoglobinuria Microangiopathic haemolytic anaemia Prosthetic valve-related haemolytic anaemia
Investigations in haemolytic anaemia
FBC: normocytic anaemia
Blood film: schistocytes
Direct Coombs test: + in autoimmune haemolytic anaemia
General presentation of haemolytic anaemias
Jaundice, gallstones, splenomegaly.
Features of haemolytic anaemia (3)A
Anaemia (reduced circulation of RBCs)
Splenomegaly (full of destroyed RBC)
Jaundice (increased bilirubin from high number of destroyed RBCs)
Typical haemolytic anaemia presentation and aplastic crisis and parvovirus is typically caused by what?
Inherited spherocytosis / elliptocytosis
Typical haemolytic anaemia presentation and crisis linked to infection / medication / Java beans is linked to what cause?
G6DP deficiency
Epidemiology and genetics of inherited causes of haemolytic anaemia
Spherocytosis and elliptocytosis : AD, european
G6PD: X-linked, mediterranean / African
Diagnosis of inherited haemolytic anaemia
Blood film findings
Heinz bodies: G6PD
Spherocytes / elliptocytes
2 types of autoimmune haemolytic anaemia and their causes?
Warm: haemolysis of RBCs at normal or above temperatures. Idiopathic
Cold: AKA cold agglutinin disease: RBCs agglutinate at low temperatures. typically 2o to lymphoma, leukaemia, SLE, CMV, HIV, EBV.
Treatment of autoimmune haemolytic anaemia
Blood transfusion
Prednisolone
Rituxamab
Splenectomy
Treatment of autoimmune haemolytic anaemia
Blood transfusion
Prednisolone
Rituxamab
Splenectomy
Causes of alloimmune haemolytic anaemia
Blood transfusion
HDNB
Causes of microangiopathic haemolytic anaemia
DIC HUS TTP SLE Cancer
Rx of prosthetic valve haemolytic anaemia
Monitoring
Oral iron
Blood transfusion (if severe)
Revision surgery (if severe)
Types of thalassaemia
a - defect on alpha-globin chains on Ch16
B - defect on beta-globin chains on Ch11
minor: 1 normal and 1 abnormal gene. Monitoring, microcytic anaemia
intermedia: 2 defective or 1 defective 1 deletion. Worse microcytic aneamia, blood transfusions and iron chelation to prevent overload
Major: no functioning beta-globin. Most severe. splenectomy +/- BM transplant
Genetics of SCD
HBF is usually replaced by HbA at 6 weeks. In SCD it is instead replaced with HbS.
1 copy = trait (carrier), 2 copies = disease.
Dx of SCD
Screening offered to at-risk pregnant women
5d heel prick test
Complications of SCD
Anaemia Increased risk of infection Stroke Avascular necrosis Pulmonary HTN Priapism CKD SC crises Acute chest syndrome
General Rx of SCD
Adequate hydration Up-to-date vaccines Prophylactic abx (penV) hydroxcarbamide (HbF production) Blood transfusion BM transplant
SC crises causes
Spontaneous or in response to triggers
dehydration, cold, infection, stress.
Rx SC crisis
Supportive Low threshold for admission Treat any infection Keep warm Keep well hydrated Simple analgesia.
