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High Yield: Haematology > Haemolytic Anaemias > Flashcards

Haemolytic Anaemias Flashcards

1
Q

Clinical features of extravascular haemolysis?

A

Splenomegaly

Jaundice due to unconjugated bilirubin

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2
Q

Clinical and lab findings of intravascular haemolysis?

A

Increased free plasma haemoglobin (haemoglobinaemia)
Haemoglobinuria (dark red urine)
Decreased haptoglobin (binds free haemoglobin)

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3
Q

Lab findings of all haemolytic anaemias?

A 
Increased unconjugated bilirubin
Increased urobilinogen
Increased LDH
Reticulocytosis (increased MCV and polychromasia)
May have pigmented bile gallstones
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4
Q

Inherited causes of haemolytic anaemias?

A

Membrane defect: hereditary spherocytosis, hereditary eliptocytosis
Enzyme defect: G6PD deficiency, pyruvate kinase deficiency
Haemoglobinopathies: sickle cell disease, thalassaemias

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5
Q

Acquired causes of haemolytic anaemias?

A

Immune: autoimmune (warm or cold), alloimmune (haemolytic transfusion reactions)
Non immune: mechanical (metal valves, trauma), PNH, MAHA. Infections (malaria), drugs

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6
Q

Characteristic blood cell in hereditary spherocytosis?

A

Spherocytes with loss of central pallor

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7
Q

Type of haemolysis seen in hereditary spherocytosis and the clinical finding?

A

Extravascular

Splenomegaly- spherocytes consumed by splenic macrophages

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8
Q

How is hereditary spherocytosis diagnosed?

A

Osmotic fragility test

DAT is negative

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9
Q

Treatment for hereditary spherocytosis?

A

Splenectomy

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10
Q

What are people with hereditary spherocytosis susceptible to?

A

Increased risk for aplastic crisis with parvovirus B19 infection of erythroid precursors

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11
Q

Inheritance of hereditary spherocytosis?

A

Autosomal dominant

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12
Q

Inheritance of G6PD deficiency?

A

X linked

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13
Q

Characteristic blood film of G6PD deficiency?

A

Heinz bodies

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14
Q

Role of G6PD?

A

Helps RBCs make glutathione which protects them from OXIDANT damage.

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15
Q

Features seen in G6PD attack?

A

Rapid anaemia and jaundice

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16
Q

Type of haemolysis in G6PD deficiency?

A

Intravascular

Get dark urine

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17
Q

How to diagnose G6PD deficiency?

A

Enzyme assay 2-3 months after a crisis.

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18
Q

Features of pyruvate kinase deficiency?

A

Autosomal recessive

SEVERE NEONATAL JAUNDICE, splenomegaly, haemolytic anaemia

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19
Q

Inheritance of sickle cell anaemia and the mutation that occurs?

A

Autosomal recessive

Mutation in beta chain of haemoglobin, single amino acid change of glutamic acid to valine (hydrophobic)

20
Q

Difference between sickle cell trait and disease?

A

Trait: HbAS- usually asymptomatic
Disease: HbSS- severe

21
Q

What increases the risk of sickling?

A

Hypoxaemia, dehydration and acidosis

22
Q

What does hydroxurea treatment do?

A

Increase levels of HbF

HbF protects against sickling; high HbF at birth is protective for the first few months of life.

23
Q

Type of haemolysis present in sickle cell disease?

A

Extravascular

24
Q

What is seen on the blood film for sickle cell disease?

A

Sickle cells and target cells

25
Q

How can sickle cell be diagnosed?

A

Sickle cells and target cells on blood film
Sickle solubility test
Hb electrophoresis

26
Q

Smear of beta thalassaemia?

A

Microcytic, hypochromic RBCs and target cells

27
Q

Diagnosis of beta thalassaemia?

A

Hb electrophoresis

28
Q

What test is used for autoimmune haemolytic anaemia?

A

Coombs test/direct antiglobulin test

Will be positive

29
Q

Blood film of warm auto immune haemolytic anaemia?

A

Spherocytes
IgG binds RBCs in the relatively warm temperature of the central body (warm agglutinin); membrane of antibody coated RBC is consumed by splenic macrophages resulting in spherocytes.

30
Q

What is warm auto immune haemolytic anaemia associated with?

A

CLL, SLE, METYHLDOPA

31
Q

Antibody in warm auto immune haemolytic anaemia?

A

IgG

32
Q

Antibody in cold agglutinin disease?

A

IgM

33
Q

What infections are associated with cold agglutinin disease?

A

Mypoplasma pneumoniae and infectious mononucleosis (EBV), hepatitis C

34
Q

What is the cause of paroxysmal nocturnal haemoglobinuria?

A

ACQUIRED loss of protective GPI surface markers of blood cells which usually protects against complement.
Get COMPLEMENT MEDIATED LYSIS
Chronic intravascular haemolysis especially at night

35
Q

Haemolysis seen in warm AIHA?

A

Extravascular

36
Q

Haemolysis seen in cold AIHA?

A

Intravascular

37
Q

Causes of microangiopathic haemolytic anaemia?

A

Haemolytic uraemic syndrome
Thrombotic thrombocytopenic purpura
Disseminated intravascular coagulation

38
Q

Blood film features of microangiopathic haemolytic anaemia?

A

SCHISTOCYTES

THROMBOCYTOPENIA

39
Q

What is MAHA?

A

Pathological formation of platelet microthrombi in small vessels, causing mechanical destruction of red blood cells.

40
Q

How do you distinguish HUS and TTP from DIC?

A

Normal APTT, PT and fibrinogen.

Abnormal in DIC

41
Q

What is TTP caused by?

A

Deficiency of ADAMTS13 which is needed to chop up vWF multimers, so get abnormal platelet adhesion and microthrombi which chop up the RBCs.

42
Q

What is the pentad of symptoms in in TTP?

A 
MAHA
FEVER
RENAL IMPAIRMENT
NEUROLOGICAL SYMPTOMS (PREDOMINANT PROBLEM)
THROMBOCYTOPENIA
43
Q

What is haemolytic uraemic syndrome caused by?

A

Commonly by Escherichia coli 0157:H7

Classically in children

44
Q

Features of haemolytic uraemic syndrome?

A 
Symptoms occur after a diarrhoeal illness
MAHA
Thromboctyopaenia
Acute renal failure
Self limiting in children
45
Q

Antibody involved in haemolytic disease of the newborn?

A

IgG

ONLY IgG CAN CROSS THE PLACENTA!

46
Q

Features of HELLP syndrome?

A

HAEMOLYSIS, ELEVATED LIVER ENZYMES, LOW PLATELETS

Life threatening complication associated with pregnancy.

MAHA, INCREASED AST, INCREASED ALT, DECREASED PLATELETS, NORMAL APTT, NORMAL PT

Differentials include DIC: in DIC the APTT is INCREASED, the PT is INCREASED, fibrinogen is DECREASED, AFLP (marked transaminitis)

Management- supportive delivery of the foetus

High Yield: Haematology (11 decks)

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