Iron overload in thalassemia

Iron accumulation in tissues (liver, heart, endocrine glands) in transfusion-dependent anemias (beta-thalassemia major, sickle cell disease, myelodysplastic syndromes). One unit of RBCs contains 200–250 mg of iron; normal body content is 3–4 g. Without chelation therapy, 10–15 years of regular transfusions lead to cardiac siderosis with heart failure and death. Per TIF 2023 and Spanish SEHH 2022 first-line is deferasirox or deferiprone, target ferritin <1000 ng/mL.

ICD-10 E83.11 drug

Also known as: Transfusional iron overload, Hemosiderosis in thalassemia

Drugs used for this condition

ADeferasiroxFirst line · Iron chelating agentsFirst-line iron-overload therapy in transfusion-dependent anemias (beta-thalassemia major, sickle cell disease, lower-risk MDS) per TIF 2023 and Spanish SEHH 2022. Starting dose 14 mg/kg once daily on empty stomach 30 minutes before food (Jadenu/film-coated tablets), or 20 mg/kg for the original Exjade form (dispersible tablets). Target – ferritin <1000 ng/mL, LIC (T2* MRI liver iron concentration) <7 mg/g dry weight.

How to read tiers A–F

Evidence tiers belong to the drug-indication pair, not to the drug as a whole: the same substance can be A for one condition and F for another.

AStrong evidence – first or second line in international guidelines
BRecommended in selected cases
CStudy data contradict each other
DBenefit not proven, no documented harm
FDocumented harm, or risk without benefit