Haemolytic Anaemia

Question 1

Definition of haemolytic anaemia

Answer 1

Anaemia due to increased destruction of RBCs

Question 2

Inherited causes of haemolytic anaemia (5)

Answer 2

Thalassaemia
SCD
G6PD
Hereditary spherocytosis
Hereditary elliptocytosis

Question 3

Acquired causes of haemolytic anaemia (5)

Answer 3

Autoimmune haemolytic anaemia 
Alloimmune haemolytic anaemia 
Paroxymal nocturnal haemoglobinuria
Microangiopathic haemolytic anaemia 
Prosthetic valve-related haemolytic anaemia

Question 4

Investigations in haemolytic anaemia

Answer 4

FBC: normocytic anaemia
Blood film: schistocytes
Direct Coombs test: + in autoimmune haemolytic anaemia

Question 5

General presentation of haemolytic anaemias

Answer 5

Jaundice, gallstones, splenomegaly.

Question 6

Features of haemolytic anaemia (3)A

Answer 6

Anaemia (reduced circulation of RBCs)
Splenomegaly (full of destroyed RBC)
Jaundice (increased bilirubin from high number of destroyed RBCs)

Question 7

Typical haemolytic anaemia presentation and aplastic crisis and parvovirus is typically caused by what?

Answer 7

Inherited spherocytosis / elliptocytosis

Question 8

Typical haemolytic anaemia presentation and crisis linked to infection / medication / Java beans is linked to what cause?

Answer 8

G6DP deficiency

Question 9

Epidemiology and genetics of inherited causes of haemolytic anaemia

Answer 9

Spherocytosis and elliptocytosis : AD, european

G6PD: X-linked, mediterranean / African

Question 10

Diagnosis of inherited haemolytic anaemia

Answer 10

Blood film findings
Heinz bodies: G6PD
Spherocytes / elliptocytes

Question 11

2 types of autoimmune haemolytic anaemia and their causes?

Answer 11

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.

Question 12

Treatment of autoimmune haemolytic anaemia

Answer 12

Blood transfusion
Prednisolone
Rituxamab
Splenectomy

Question 13

Treatment of autoimmune haemolytic anaemia

Answer 13

Blood transfusion
Prednisolone
Rituxamab
Splenectomy

Question 14

Causes of alloimmune haemolytic anaemia

Answer 14

Blood transfusion

HDNB

Question 15

Causes of microangiopathic haemolytic anaemia

Answer 15

DIC
HUS
TTP
SLE
Cancer

Question 16

Rx of prosthetic valve haemolytic anaemia

Answer 16

Monitoring
Oral iron
Blood transfusion (if severe)
Revision surgery (if severe)

Question 17

Types of thalassaemia

Answer 17

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

Question 18

Genetics of SCD

Answer 18

HBF is usually replaced by HbA at 6 weeks. In SCD it is instead replaced with HbS.
1 copy = trait (carrier), 2 copies = disease.

Question 19

Dx of SCD

Answer 19

Screening offered to at-risk pregnant women

5d heel prick test

Question 20

Complications of SCD

Answer 20

Anaemia
Increased risk of infection
Stroke
Avascular necrosis
Pulmonary HTN
Priapism
CKD
SC crises
Acute chest syndrome

Question 21

General Rx of SCD

Answer 21

Adequate hydration
Up-to-date vaccines
Prophylactic abx (penV)
hydroxcarbamide (HbF production)
Blood transfusion
BM transplant

Question 22

SC crises causes

Answer 22

Spontaneous or in response to triggers

dehydration, cold, infection, stress.

Question 23

Rx SC crisis

Answer 23

Supportive
Low threshold for admission
Treat any infection
Keep warm
Keep well hydrated
Simple analgesia.