Haemolytic Anaemia

Question 1

Signs/Sympto haemolytic anaemia

Answer 1

all - jaundice with uncojugated billirubinaemia, normocytic anaemia, inc LDH, reticulocytosis, pigmented gallstones
intravasc (RBC rupture) - inc free plasma hb, inc serum LDH, dark red urine (dipstick +ve blood)
Extravasc (splenic consuption) - splenomeg, urobilinogen in urine (dipstick -ve blood) spherocytes on blood smear

Question 2

Causes of inherited HA

Answer 2

memb defect - hrtiditary sphero/eliptocytosis
enz defect - G6PD/pyruvate kinase def
Haemoglobinopathies - SCD. thallassaemia

Question 3

difference between heriditary sphero and eliptocytosis

Answer 3

S = vertical interaction
E = horizontal interaction

Question 4

causes acquired HA

Answer 4

immune - auto/allo
mechanical - metal valves/trauma
MAHA

Question 5

First symtpto G6PDD

Answer 5

neonatal jaundice, but otherwise asymptomatic

Question 6

What is show on slide in G6PDD

Answer 6

bite cells, nucelated RBCs, heinz bodies

Question 7

What is show on slide in pyruvate kinase def

Answer 7

echinocytes (hedgehog) and spherocytes

Question 8

dangers of G6PDD

Answer 8

acute haemolysis triggered by: oxidants, drugs, inf

Question 9

Inv for metabolic RBC disorders

Answer 9

DAT - direct antigen test - AI haemolysis
osmotic fragility - H sperocytosis
G6P + PK activity
Ham’s test - paroxysmal noctural haemoglobinuria

Question 10

managment of metabolic RBC disorders

Answer 10

Folic acid supp
avoid precipitating factors
transfusion
splenectomy

Question 11

Findings in WAIHA

Answer 11

Warm autoimmune hemolytic anemia 
\+ve DAT 
pos combs test
spherocytes
>37 degrees

Question 12

Tx for AIHA

Answer 12

steroids + immunosupp, rituximab

Question 13

Findings in cold agglutin disease

Answer 13

<37 degrees
+ve combs and DAT
often with raynauds

Question 14

Most common cause of HUS

Answer 14

E coli 0157:H7

look for sympto after diarrhoeal disease

Question 15

who does HUS effect

Answer 15

children and elderly

Question 16

What cause of HA would you suspect if gallstones at a young age

Answer 16

Memb defects

Question 17

What is show in periph blood in memb defect HA

Answer 17

reticulocytes

Question 18

what antibodies mediate warm vs cold AIHA

Answer 18

warm = IgG
cold = IgM

Question 19

warm vs cold AIHA - intra vs extravasc

Answer 19

Warm = extra
cold = intra

Question 20

asso of warm AIHA

Answer 20

CLL, SLA, methyldopa

Question 21

asso of cold AIHA

Answer 21

mycoplasma, EBV, HepC

Question 22

pathology of MAHA

Answer 22

microangiopatic haemolytic anaemia

damage to endo cells -> plt aggregation -> RBC fragmentation

Question 23

periph blood in MAHA

Answer 23

schistocytes, thrombocytopaenia

Question 24

what is HUS

Answer 24

Haemolytic uraemic syndro

MAHA + throbocytopaenia + acute renal F

Question 25

What is TTP

Answer 25

Thrombotic thrombocytopenic purpura

MAHA + thrombocytopaenia + acute renal F + fever + neruological sympto

Question 26

path of TTP

Answer 26

prot def -> cant break down VWF

Question 27

what drugs especially cause oxidative stress and who shoudk avoid them

Answer 27

anti-malarial + anti-fungal

avoid in G6PDD (-> haemolysis)