anaemia pt 3

Question 1

how to RBCs produce ATP

Answer 1

cytoplasmic enzyme pathway (as no mitochondria or nucleus)

Question 2

Glucose-6-phosphate dehydrogenase (GP6D) is used in

Answer 2

reducing NADP to NADPH using ATP activating glutathione protecting cells from oxidative stress

Question 3

GP6D deficiency inheritance pattern

Answer 3

X-linked recessive (M>F)

Question 4

most common RBC disorder=

Answer 4

GP6D deficiency

Question 5

commonest people affected by GP6D

Answer 5

mediterranean and african

Question 6

clinical features of GP6D def (4)

Answer 6

• neonatal jaundice (haemolytic anaemia)
• anaemic crisis precipitated by drugs or fava beans
• FBC normal between attacks
• gall stones and splenomegaly

Question 7

drugs precipitating GP6D def anaemic crisis

Answer 7

anti-malarial

ciprofloxacin

Question 8

2nd most common metabolic disorder of RBC

Answer 8

pyruvate kinase def

Question 9

effect of pyruvate kinase def

Answer 9

reduced production of ATP in 1st pathway

Question 10

inheritance of pyruvate kinase def

Answer 10

autosomal recessive

Question 11

clinical features of pyruvate kinase def (3)

Answer 11

rigid cells
haemolytic anaemia
splenomegaly

Question 12

blood film of pyruvate kinase def shows

Answer 12

prickle cells and reticulocytosis

Question 13

auto-immune anaemia =

Answer 13

RBC destruction due to auto-Ig

Question 14

what test is positive in auto-immune anaemia

Answer 14

coombs test

Question 15

2 types of auto-immune anaemia

Answer 15

cold vs warm -based on temp Ig best attaches to RBC

Question 16

key features of warm auto-immune anaemia

Answer 16

• IgG involved
• strongly +ve coombs test
• phagocytosed in spleen

Question 17

presentation of warm autoimmune anaemia (3)

Answer 17

middle-aged females
relapsing and remitting anaemia and jaundice
spleen often palpable

Question 18

investigation results of warm auto-immune anaemia

Answer 18

haemolytic anaemia

spherocytosis

Question 19

what can also be present in warm auto-immune anaemia called Even’s syndrome

Answer 19

thrombocytopenia or neutropenia

Question 20

causes of warm auto-immune anaemia

Answer 20

idiopathic 
secondary to: 
-CLL
-rheumatic disorders (SLE) 
-lymphoma 
-carcinoma's
-drugs

Question 21

drugs causing auto-immune anaemia

Answer 21

methyldopa 
penicillins 
cephalosporins 
NSAIDs 
quinine

Question 22

treatment of warm auto-immune anaemia

Answer 22

corticosteroids

2nd line splenectomy

Question 23

cold auto-immune anaemia leads to

Answer 23

mild haemolytic anaemia

Question 24

causes of cold auto-immune anaemia

Answer 24

• mono
• mycoplasma pneumoniae
• lymphomas
• paroxysmal cold haemoglobinuria

Question 25

causes of microcytic anaemia (4)

Answer 25

iron deficiency thalassaemia anaemia of chronic disease sideroblastic anaemia

Question 26

causes of macrocytic anaemia megaloblastic

Answer 26

vit B12 def | folate def

Question 27

causes of macrocytic normoblastic anaemia (5)

Answer 27

``` alcohol increased reticulocytes (haemolysis, haemorrhage) liver disease hypothyroidism drug therapy -azathioprine ```

Question 28

normocytic anaemia causes (7)

Answer 28

- acute blood loss - anaemia of chronic disease - CKD - autoimmune rheumatic disease - marrow infiltration/ fibrosis - endocrine disease - haemolytic anaemias