Iron overload in non-transfusion-dependent thalassemia: a clinical perspective.
Iron overload due to increased intestinal iron absorption represents an important clinical problem in patients with non-transfusion-dependent thalassemia (NTDT), particularly as they advance in age. Current models for iron metabolism in patients with beta (β)-thalassemia intermedia (TI) suggest that suppression of serum hepcidin results in increased iron absorption and release of iron from the reticuloendothelial system, leading to depletion of macrophage iron, relatively low levels of serum ferritin, and liver iron loading. The clinical consequences of iron overload in patients with NTDT are multifactorial and include endocrinopathy, bone disease, thromboembolism, pulmonary hypertension, cerebrovascular and neuronal damage, liver fibrosis or cirrhosis, and increased risk of hepatocellular carcinoma. Although serum ferritin levels correlate with liver iron concentration (LIC), they underestimate iron load in these patients compared with transfusion-dependent patients with equivalent LIC. Therefore, direct measurement of LIC is recommended with chelation therapy as indicated.
IRCCS Ca' Granda Foundation Maggiore Policlinico Hospital, University of Milan, Milan, Italy.
Khaled M
KM
Musallam
Maria D
MD
Cappellini
John C
JC
Wood
Ali T
AT
Taher
D001803:Blood Transfusion; D006801:Humans; D007408:Intestinal Absorption; D007501:Iron / Q000378:metabolism*; D007502:Iron Chelating Agents / Q000627:therapeutic use; D019190:Iron Overload / Q000188:drug therapy / Q000378:metabolism*; D013789:Thalassemia / Q000378:metabolism*
D007502:Iron Chelating Agents; D007501:Iron