Haemolytic anaemia Flashcards
Hereditary spherocytosis is inherited in which fashion?
Autosomal dominant
What population is mainly affected by Hereditary spherocytosis
Northern Europeans
Causes of warm-type autoimmune haemolytic anaemia
SLE
Chronic lymphocytic leukaemia
Lymphoma
Causes of warm-type autoimmune haemolytic anaemia
Mycoplasma pneumonia
EBV infection and other viral infections
Lymphoma
Pathology of warm-antibody type haemolytic anaemia
Warm antibodies= IgG/IgA
- Ab bind to antigens on red cells and trigger immunological response
There is high disease activity at temperatures >37
Pathology of cold-antibody type haemolytic anaemia
Cold autoantibodies= IgM
- Finds to red cell antigens
- Disease activity highest at 0-4 degress
Cold type haemolytic anaemia presentation
Acrocyanosis
Raynaud phenomenon
Fatigue, malaise, dyspneoa
Pallor
Direct Coomb’s test findings for warm and cold type autoimmune haemolytic anaemia
Positive in both types
Warm type
- Antisera reacts with IgG
Cold type
- Antisera mainly reacts to complements
Management of autoimmune haemolytic anaemia
In warm-type
- First line= steroids
- Second-line= DMARDs
G6PD deficiency is mainly inherited via what pattern?
X-linked recessive
Function of G6PD enzyme
In pentose phosphate pathway
- Protects red cells from oxidative damage and subsequent haemolysis
G6PD deficiency mainly affects what population?
Mediterraneans and Africas
What are the common triggers of
haemolytic anaemia in GG6PD deficiency?
Broad beans
Drugs
- Primaquine
- Nitrofurantoin
- quinolones
- Sulfonamides
Severe infection
Signs of G6PD deficiency
Pallor (anaemia)
Jaundice
Gallstones
Splenomegaly
What is the diagnostic test for G6PD deficiency
G6PD enzyme activity
Blood film findings for G6PD deficiency
Macrocytic red cells
Acute haemolysis
- Heinz bodies
- Bite cells
Hereditary spherocytosis blood test findings
- FBC
- Blood film
- Coomb’s test
FBC
- Low Hb
- Raised Hct and MCHC
Blood filmd
- Spherocytes
- Reticulocytes
Coomb’s test= negative
Paroxysmal nocturnal haemoglobinuria
- Pathology
Sporadic mutation of haematopoietic stem cells
- Leads to the production of abnormal red cells, platelets and white cells
Loss of red cell surface proteins leads to activation of complement system= haemolytic anaemia
Paroxysmal nocturnal haemoglobinuria
- Presentation
Haemolytic anaemia
- pallor, fatigue
- Palpitations, reduced exercise tolerance
- jaundice
- Haemoglobinuria= Worse in the morning when urine is particularly concentrated
Thrombosis
Thrombocytopenia
- Bruising
- Menorrhagia
Diagnostic findings for
