Haemolytic anaemia

Question 1

state 5 signs of intravascular haemolysis?

Answer 1

haemoglobinaemia 
haemogloburia 
decreased serum haptoglobins 
increased urine haemosiderin 
methaemalbuminaemia

Question 2

what is a sign of extravascular haemolysis ?

Answer 2

splenomegaly

Question 3

state two enzyme causes of haemolysis ?

Answer 3

G6PD and pyruvate kinase deficiency

Question 4

what is the inheritance of G6PD deficiency?

Answer 4

X linked

Question 5

what process is G6PD used in?

Answer 5

pentose phosphate shunt

Question 6

what are some triggers of haemolysis in G6PD deficiency ?

Answer 6

broad beans
infection
drugs - antimalarials, henna, sulphonamides

Question 7

what 4 things are seen on a blood film for G6PD deficiency ?

Answer 7

blister cells
bite cells
ghost cells
Heinz bodies

Question 8

what is the treatment for G6PD deficiency ?

Answer 8

treat underlying cause

transfusion may be needed

Question 9

what is the inheritance of pyruvate kinase deficiency ?

Answer 9

autosomal recessive

Question 10

what are the three features of pyruvate kinase deficiency ?

Answer 10

jaundice, splenomegaly, anaemia

Question 11

what is seen on the blood film of B12/folate deficiency ?

Answer 11

oval macrocytes

hypersegmented PMN

Question 12

what is seen on the blood film of alcohol caused microcytic anaemia ?

Answer 12

target cells

Question 13

what is the role of B12 in the body?

Answer 13

DNA and myelin synthesis

Question 14

what is glossitis ?

Answer 14

beefy red tongue seen in macrocytic anaemia

Question 15

state four neuro complications of b12/folate deficiency ?

Answer 15

paraesthesia
peripheral neuropathy
optic atrophy
SACD

Question 16

what is SACD?

Answer 16

subacute combined degeneration of the spinal cord

Question 17

what is SACD usually caused by?

Answer 17

pernicious anaemia

Question 18

what are the signs of SACD?

Answer 18

combined symmetrical dorsal column and corticospinal tract loss

• mixed UMN and LMN signs
• spastic paraparesis
• brisk knee jerks
• absent ankle jerks
• up going plantars

temp and pain intact!!

Question 19

what are the two antibodies in pernicious anaemia?

Answer 19

intrinsic factor abs

parietal cells abs

Question 20

what cancer are pernicious anaemia patients more at risk of ?

Answer 20

gastric adenocarcinoma

Question 21

auto immune haemolytic anaemia warm is mediated by what?

Answer 21

IgG

Question 22

auto immune haemolytic anaemia cold is mediated by what?

Answer 22

IgM

Question 23

what are the causes of auto immune haemolytic anaemia warm ?

Answer 23

idiopathic
SLE
RA
Evans

Question 24

what is auto immune haemolytic anaemia cold coombs test positive for?

Answer 24

complement only

Question 25

what is PNH haemolytic anaemia ?

Answer 25

paroxysmal nocturnal haemoglobinuria

Question 26

what causes paroxysmal nocturnal haemoglobinuria ?

Answer 26

absence of RBC anchor molecule GPI which decreases complement and results in intravascular haemolysis

Question 27

what does paroxysmal nocturnal haemoglobinuria bloods result in ?

Answer 27

BMF anaemia, thrombocytopenia, neutropenia low complement CD55 and CD59

Question 28

What is haemolytic uraemic syndrome caused by?

Answer 28

E coli 0157

Question 29

what does haemolytic uraemic syndrome result in ?

Answer 29

``` bloody diarrhoea abdo pain microangiopathic hemolytic anemia thrombocytopenia renal failure ```

Question 30

what cells are seen on the blood film for haemolytic uraemic syndrome and TTP?

Answer 30

schistocytes

Question 31

what is the treatment for haemolytic uraemic syndrome?

Answer 31

usually resolves on its own

Question 32

what acquired deficiency can cause TTP?

Answer 32

ADAMTS13

Question 33

what does MAHA stand for?

Answer 33

microangiopathic hemolytic anemia

Question 34

what are the signs of TTP?

Answer 34

microangiopathic hemolytic anemia fever thrombocytopenia renal failure

Question 35

what is the treatment for TTP?

Answer 35

plasmapheresis immunosuppression splenectomy

Question 36

what is the commonest inherited haemolytic anaemia ?

Answer 36

hereditary spherocytosis

Question 37

what is the inheritance of hereditary spherocytosis?

Answer 37

autosomal dominant

Question 38

what are three features of hereditary spherocytosis?

Answer 38

splenomegaly pigment gallstones jaundice

Question 39

is hereditary spherocytosis immune mediated ?

Answer 39

no

Question 40

what are some triggers for sickle cell?

Answer 40

infection cold hypoxia dehydration

Question 41

what is the presentation of sickle cell? SICKLED

Answer 41

``` splenomegaly (which may cause sequestration crisis - fills with blood) infarction crises (pulmonary, mesenteric) kidney disease liver/lung disease erection dactylitis ``` - last due to thrombosis from sickled cells clumping together and blocking blood in the capillary system

Question 42

what is the treatment for sickle cell?

Answer 42

penicillin immunisations folate