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Year 5 Pathology Revision > Haematology 1: Haemolytic Anaemias > Flashcards

Haematology 1: Haemolytic Anaemias Flashcards

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1
Q

What is the difference between intravascular and extravascular haemolysis ?

A

Intravascular- occurs inside the circulation
Extravascular- Occurs in the reticuloendothelial system

Inherited or acquired

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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 (glycosylphosphatidylinositol) anchor - one way cells attach proteins to their surface

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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 - increased sensitivity to lysis in hypotonic saline

also reduced binding to dye eosin-5-maleimide (flow cytometry)

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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
Fava beans 
Napthalene moth balls 
Infections
Antibiotics (Sulphonamides)

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

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18
Q

Name 2 characteristic cells seen in G6PD ?

A

Bite cells

Heinz bodies

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19
Q

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

A

Heinz bodies

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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

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21
Q

List 4 causes of basophilic stippling ?

A

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

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22
Q

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

A

HAM’s test

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23
Q

Which test is used to diagnose Malaria ?

A

Thick and thin blood films

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24
Q

Which test is used to diagnose Autoimmune haemolytic anaemia ?

A

DAT test

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25
Which type of immunoglobulin causes warm autoimmune haemolytic anaemia ?
IgG
26
Which type of immunoglobulin causes cold auto immune haemolytic anaemia ?
IgM
27
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 ?
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
Which test can be used to diagnose Paroxysmal nocturnal haemoglobinuria ?
Ham’s test | Or immunophenotyping
29
Which drug can be used to treat PNH?
Eculizumab
30
Which antibodies cause TTP ?
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
What is the Pentad for TTP ?
``` MAHA Thrombocytopenia Renal impairment fever Neurological symptoms ```
32
Which infection is commonly responsible for causing HUS ?
E.Coli
33
Which conditions are associated with cold AIHA ?
Think cold LID Lymphoproliferative - CLL, Lympomas Infections - Mycoplasma, EBV Do not know - Idiopathic
34
List 2 conditions associated with Spherocytes ?
Hereditary spherocytosis | Warm AIHA
35
List 2 conditions and 1 drug that are associated with warm AIHA ?
CLL SLE Penicillin
36
Name a congenital cause of aplastic anaemia ?
Fanconi anaemia
37
List 4 drugs can cause aplastic anaemia ?
Carbamazepine Chloramphenicol Cytotoxic drugs Phenytoin
38
Name 1 drug used in the treatment of Sickle cell anaemia ?
Hydroxycarbamide
39
Which drug acts to replenish vitamin b12 stores in pernicious anaemia ?
IV Hydroxocobalamine
40
List 2 drugs that can cause Folate deficiency ?
Methotrexate | Phenytoin
41
List causes of MAHA ?
``` TTP HUS DIC SLE Malignant hypertension Pre-eclampsia ```
42
what is haemolysis
shortened RBC survival (<120 days)
43
list causes of intravascular haemolysis
``` malaria G6PD deficiency mismatched blood transfusion (ABO) cold antibody haemolytic syndromes drugs MAHA eg HUS TTP paroxysmal nocturnal haemoglobinuria ```
44
what is the most common cause of intravsacular haemolysis
malaria | G6PD offers some protection - common
45
how can hereditary haemolytic anaemias be divided
disorders affecting: - membrane - cytoskeletal proteins, cation permeability - red cell metabolism - particularly glycolysis - haemoglobin - thalassaemia, sickle cell syndrome, unstable Hb variants
46
list consequences of haemolytic anaemia
ANAEMIA ERYTHROID HYPERPLASIA - increased RBC and reticulocyte production INCREASED FOLATE DEMAND - give supplementation SUSCEPTIBILITY TO PAROVIRUS B19 - infects erythroid cells in the BM - causes an aplastic crisis (dangerously low Hb) - ID a low reticulocyte count PROPENSITY TO GALLSTONES esp with gilberts syndrome INCREASED RISK OF IRON OVERLOAD INCREASED RISK OF OSTEOPOROSIS
47
what mutations cause gilberts syndrome
ugt1a1 ta7/ta7 genotype
48
clinical features of haemolytic anaemia
``` pallor jaundice splenomegaly pigmenturia family history ```
49
lab features of haemolytic anaemia
``` anaemia increased reticulocytes polychromasia hyperbilibrubinaemia increased LDH (esp in intravascular haemolysis) reduced/absent haptoglobins haemoglobinuria haemosiderinuria ```
50
features specific to intravascular haemolysis
LDH reduced or absent haptogobins (removes free haemoglobin) haemoglobinuria haemosiderinaemia
51
describe features of the RBC membrane
cytoskeletal scaffold make of spectrin proteins link bilayer to the cytoskeleton - band 3 ankyrin-1 - absence = haemolytic anaemia GPI - molecule lacking in paroxysmal nocturnal haemoglobinuria
52
what are the defects in hereditary spherocytosis
vertical interaction (between proteins that link the lipid bilayer to the cytoskeleton) band 3 protein 4.2 ankyrin B spectrin
53
what are the defects in hereditary elliptocytosis
horizontal interaction - mainly alpha and beta spectrin alpha spectrin beta spectrin protein 4.1
54
what is the most common defect of the red cell cytoplasm
hereditary spherocytosis | 75% have FH (AD)
55
appearance of hereditary spherocytosis on blood film
``` cells lack area of central pallor smaller than normal dense staining increased MCHC polychromatic cells (reticulocytes) ```
56
appearance of hereditary elliptocytosis on blood film
no polychromastia cells long shapes blood count usually normal not much haemolysis
57
heredtary pyropoikilocytosis on blood film
homozygous form of hereditary eliptocytosis fragment RBC poikilocytosis (variation in shape) can cause severe haemolytic anaemia
58
describe features of G6PD deficiency
prevalent where malaria is endemic x-linked G6PD enzyme catalyses the first step in the pentose phosphate pw generates NADPH which maintains intracellular glutathione (GSH) GSH needed to protect cells against oxidative stress
59
clinical effects of G6PD deficiency
neonatal jaundice acute haemolysis chronic haemolytic anaemia
60
describe the blood film of a G6PD deficient patient during acute haemolysis
lots of contracted cells, nucleated red cells, bite cells, hemighosts, heinz bodies in steady state, blood film usually normal
61
list some key metabolic pathways in RBC
``` embden- meyerhof - ATP, NADG hexose monophosphate shunt - NADPH rapoport-lueberig shuttle - 2,3-DPG nucleotide metabolism glutathione biosynthesis - GSH cytochrome b5 reductase - methemoglobin reduction ```
62
features of pyruvate kinase deficiency on blood smear
most common defect in glycolytic pathway lots of red cells have short projections - echinocytes number of these cells increase post-splenectomy
63
features of pyrimidine 5'- nucleotidase deficiency
defect of nucleotide metabolism | deficiency leads to basophilic stripping
64
first line investigations for haemolysis
DAT - AI Urinary haemosiderin /haemoglobin - IV haemolysis Osmotic fragility - hereditary spherocytosis G6PD +/- PK activity Heinz body stain - oxidative haemolysis Ham's test/ glow cytometry of GPI-linked proteins - PNH Thick and thin blood films - malaria
65
management of haemolytic anaemias
folic acid avoid precipitants red cell transfer/exchange immunisations against blood bourne viruses monitor for chronic complications cholecystectomy for symptomatic gallstones splenectomy if indicated
66
list indications for splenectomy
PK deficiency and some other enzymopathies HS thalassaemia syndromes immune haemolytic anaemia risk of overwhelming SEPSIS by encapsulated bacteria
67
what is haemoglobin hammersmith
sever unstable haemoglobin variant that produces a heinz body haemolytic anaemia

Year 5 Pathology Revision (106 decks)

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