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Romesh Path Fellah > Haematology 1: Haemolytic Anaemias > Flashcards

Haematology 1: Haemolytic Anaemias Flashcards

1
Q

What is the difference between intravascular and extravascular haemolysis ?

A

Intravascular- occurs inside the circulation

Extravascular- Occurs in the reticuloendothelial system

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2
Q

Which one of these is an example of a disease in which you get extravascular haemolysis ?

A) Malaria
B) Hereditary spherocytosis
C) G6PD
D) Microangiopathic haemolytic anaemia

A

B- hereditary spherocytosis

Occurs in the spleen.
The others are all intravascular

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3
Q

Name one drug that causes intravascular haemolysis ?

A

Dapsone

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4
Q

What is the inheritance pattern of Hereditary spherocytosis ?

A

Autosomal dominant

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5
Q

What happens in haemolytic anaemia patients if they become infected with Parvovirus b19 ?

A

Transient aplastic crisis

Massive reduction in Hb and circulating RBCs. Low reticulocytes shows RBC production has stopped

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6
Q

What is the Name of the disease caused by Parvovirus b19 infection in children ?

A

Fifth’s disease

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7
Q

“Slapped cheeks” rash is characteristic of which infection in children ?

A

Parvovirus B19 (fifth’s disease)

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8
Q

What disease can immunocompromised patients get if they become infected with Parvovirus B19 ?

A

Pure red cell aplasia

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9
Q

Which stain can detect hepatic siderosis (iron overload)?

A

Perl’s stain (Prussian blue)

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10
Q

What are haptoglobins ?

A

A Protein in the blood that binds to and removes free haemoglobin

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11
Q

What causes Paroxysmal nocturnal haemoglobinuria ?

A

Acquired genetic defect in GPI anchor

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12
Q

What does a low level or absent haptoglobin suggest ?

A

Intravascular haemolysis

All the haptoglobins have been used up and there is lots of free haemoglobin

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13
Q

Which of these proteins is not part of the red cell membrane ?

A)Spectrin
B)Ankyrin-1
C)Band 3
D) GPI
E) Myosin
A

E) Myosin

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14
Q

What is the diagnostic test for Hereditary spherocytosis ?

A

Osmotic Fragility test

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15
Q

Which autosomal recessive disease similar to hereditary eliptocytosis has abnormal sensitivity to heat?

A

Hereditary Pyropoikilocytosis

More severe

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16
Q

How is G6PD inherited ?

A

X-linked

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17
Q

List 6 triggers of acute haemolysis in patients with G6PD

A 
Antimalarials 
Dapsone
Favs beans 
Napthalene moth balls 
Infections
Antibiotics (Sulphonamides)

Normally G6PD patients are asymptomatic but these agents are oxidative and cause acute haemolysis

18
Q

Name 2 characteristic cells seen in G6PD ?

A

Bite cells

Hemighosts

19
Q

Oxidative haemolysis causes characteristic Blue inclusions of denatured haemoglobin in RBCs. These are also known as ………………………

A

Heinz bodies

20
Q

Name one type of cell that is characteristically seen in PKD (pyruvate kinase deficiency) ?

A

Burr cells (echinocytes)

They are spikey cells that look like sea urchins

21
Q

List 4 causes of basophilic stippling ?

A

Pyrimidine 5 nucleotidase deficiency
Lead poisoning
Megaloblastic anaemia (alcoholics)
Thalassaemia

22
Q

Which test is used to diagnose Paroxysmal nocturnal haemoglobinuria (PNH) ?

A

HAM’s test

23
Q

Which test is used to diagnose Malaria ?

A

Thick and thin blood films

24
Q

Which test is used to diagnose Autoimmune haemolytic anaemia ?

A

DAT test

25
Q

Which type of immunoglobulin causes warm autoimmune haemolytic anaemia ?

A

IgG

26
Q

Which type of immunoglobulin causes cold auto immune haemolytic anaemia ?

A

IgM

27
Q

Child recovering from measles suddenly presents with haemoglobinuria after going into an ice bath. Complement levels are low. Donath-Landsteiner test is positive.

Most likely diagnosis ?

A

PCH (paroxysmal cold haemoglobinuria)

Preceding viral infection
Cold exposure
IgG antibodies bind to P-antigen on red cells
Causes complement mediated haemolysis on warming up again

28
Q

Which test can be used to diagnose Paroxysmal nocturnal haemoglobinuria ?

A

Ham’s test

Or immunophenotyping

29
Q

Which drug can be used to treat PNH?

A

Eculizumab

30
Q

Which antibodies cause TTP ?

A

Antibodies against ADAMTS13

(ADAMTS13 is an enzyme which normally cuts vWF into smaller strands of protein. In TTP, very long strands of vWF cause shearing of RBCs in vessels leading to haemolysis and schistocytes)

31
Q

What is the Pentad for TTP ?

A 
MAHA
Thrombocytopenia 
Renal impairment 
fever
Neurological symptoms
32
Q

Which infection is commonly responsible for causing HUS ?

A

E.Coli

33
Q

Which conditions are associated with cold AIHA ?

A

Think cold LID

Lymphoproliferative - CLL, Lympomas
Infections - Mycoplasma, EBV
Do not know - Idiopathic

34
Q

List 2 conditions associated with Spherocytes ?

A

Hereditary spherocytosis

Warm AIHA

35
Q

List 2 conditions and 1 drug that are associated with warm AIHA ?

A

CLL
SLE

Penicillin

36
Q

Name a congenital cause of aplastic anaemia ?

A

Fanconi anaemia

37
Q

List 4 drugs can cause aplastic anaemia ?

A

Carbamazepine
Chloramphenicol
Cytotoxic drugs
Phenytoin

38
Q

Name 1 drug used in the treatment of Sickle cell anaemia ?

A

Hydroxycarbamide

39
Q

Which drug acts to replenish vitamin b12 stores in pernicious anaemia ?

A

IV Hydroxocobalamine

40
Q

List 2 drugs that can cause Folate deficiency ?

A

Methotrexate

Phenytoin

41
Q

List causes of MAHA ?

A 
TTP
HUS
DIC
SLE
Malignant hypertension
Pre-eclampsia

Romesh Path Fellah (96 decks)

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