Haemoglobinopathy: considerations for reproductive health TOG 2018

Question 1

Effect of iron overload on women’s reproductive health?

Answer 1

Delayed puberty
Oligo/amenorrhoea
Oligo/anovulation - secondary ovarian insufficiency

Question 2

Effect of iron overload on mens reproductive health?

Answer 2

Delayed puberty
Abnormal semen parameters
Secondary tesiticular insufficiency

Question 3

Risk of iron loading is dependant on what?

Answer 3

The transfusion requirements depending on the condition.

Higher dietary iron absorbntion

High risk Sickle cell anaemia, B Tham major

Question 4

How much iron does each unit of blood contain?

Answer 4

200-250mg iron

Question 5

What markers can be used to assess for iron overload?

Answer 5

Serum ferritin (non linear relationship)
T2 MRI
Ferriscan

Question 6

When should chelation start?

Answer 6

After 10-20 transfusion
Serum ferritin >1000

Question 7

What are the 3 iron chelators how do they work

Answer 7

desferrioxamine, deferiprone and deferasirox

Promote excretion of plasma non-transferrin-bound iron

Question 8

What is the prevalence of hypogonadotrophic hypogonadism in B thalmajor?

Answer 8

53-76%

Question 9

Successful conceptions occur with ferritin levels in the range of?

Answer 9

1000-11000 (upper normal 200-300)

Question 10

How common is erectile dysfunction in sickle disorders?

Answer 10

35%
Risk of priapism
independant of iron overload

Question 11

Potential treatment for hypogonadotrophic hypogonadism seconcondary to thalassaemia?

Answer 11

human menopausal gonadotrophin inducing ovulation & spermatogenesis induction

Question 12

Long term health consequence of hypogonadotrophic hypogonadism?

Answer 12

Low otegen can lead to cardiovascular disease, dementia and osteoporosis in reproductive age (similar to POF)

Consider oestrogen replacement