Skin colouration in thalassemia

Skin colouration in thalassemia

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Now when i was studying about thalassemia, I read that anaemia is its major characterstic. I can understand that fact. But it was also written that the skin gets pale.

Wikipedia says that the skin turns yellowish.

I want to ask firstly is it that the skin gets darker when the affected person is exposed to sun and is otherwise pale?

And also why does the skin turn yellow/pale in the first place? Does that have to do something with Iron?

Thalassemia is a genetic disease, in which either the alpha- or the beta-globins are missing or mutated. The hemoglobin of the blood is a protein complex which in adults consists of 2 alpha- and 2 beta-globin subunits. The ratio of these proteins needs to be exactly right, if one of them isn't produced enough or even missing, this leads to non-functional hemoglobin and subsequently abnormal erythrocytes.

These are then removed and broken down in the spleen (which is often abnormally enlarged), which also leads to the breakdown of the non-functional hemoglobin. The main product of the hemoglobin breakdown is Bilirubin, which is yellow. The yellow skin is a sign of jaundice, which is caused by high levels of Bilirubin in the blood. The cause for the high Bilirubin levels in the blood of Thalassemia patients is the abnormal high rate of Hemoglobin breakdown.