Higher Serum Ferritin Levels Correlate with an Increased Risk of Cutaneous Morbidity in Adult Patients with β-Thalassemia: A Single-Center Retrospective Study

Abstract

Disturbed iron homeostasis characterizes β-thalassemia and increases its morbidity. Our aim was to retrospectively associate β-thalassemia disease characteristics with treatment-requiring skin conditions. The files of adult β-thalassemia (including sickle β-thalassemia) patients were screened over a 10-year period for treatment-requiring skin disease episodes and their correlation with hematologic diagnoses and epidemiological and serological characteristics. Seventy-eight patients were identified, and 7 (9%) developed at least one relevant episode including cutaneous small-vessel vasculitis (CSVV), urticaria, and leg ulcers. Average ferritin serum level correlated significantly with development of a dermatosis (2,034 ± 799 μg/l in cases vs. 920 ± 907 μg/l in the overall population; p = 0.001, ANOVA). This difference relied exclusively on the high ferritin levels observed in patients with ‘generalized' dermatoses (urticaria and CSVV: 3,860 ± 1,220 μg/l) as opposed to values within the normal range in the case of ‘localized' ones (leg ulcers: 662 ± 167 μg/l). The employed iron chelation treatment influenced ferritin levels (p = 0.002, Kruskal-Wallis test) since chelation with a single agent seems to increase the risk of a skin disease (p = 0.013, likelihood ratio method). Conclusively, serum ferritin can be evaluated as risk factor for generalized dermatoses, but not for leg ulcers, in patients with the β-thalassemia genotype. This risk can be efficiently controlled with adequate chelation.

Analysis of Skin, Hair and Nail Diseases in the Adults with Beta Thalassemia Major

“…In addition to disease-related effects in BTM, dermatologic side effects associated with chelation treatment agents are frequently encountered (2,4). Research on the frequency of dermatologic diseases in BTM is limited and few in the literature (2,4,5). In our study, skin diseases were determined in all, hair diseases were determined in 32, and nail diseases were determined in 26 patients.…”

“…In addition to the initiation of treatment of the disease in pediatric age, with the recent developments, the disease can be followed long-term without any mortality in adulthood. In addition to disease-related effects in BTM, dermatologic side effects associated with chelation treatment agents are frequently encountered (2,4). Research on the frequency of dermatologic diseases in BTM is limited and few in the literature (2,4,5).…”

“…In patients with beta thalassemia major (BTM); without transfusion and chelation treatment, life expectancy from the time of diagnosis is measured in months. In patients, iron accumulation develops in the tissues associated with continuous blood transfusions, low hepsin levels and ineffective erythropoiesis and consequently oxidative stress occurs (2). With this vicious circle, functional and physiological disorders occur in many organs such as skin, liver, heart, pancreas, gonad, pituitary and thyroid (3,4).…”

“…With this vicious circle, functional and physiological disorders occur in many organs such as skin, liver, heart, pancreas, gonad, pituitary and thyroid (3,4). In the literature, there are few studies about the frequency of dermatological diseases in patients with BTM (2,4,5). Beside this, progressively when the increase of iron accumulation in the body is considered, as far as we know there is no further research about dermatological disorders in only adult BTM patients.…”

Is Leg Ulceration a Defending Mechanism against Toxic Iron Accumulation?

“…In this issue of Acta Haematologica , the article of Skandalis et al [1] points out a neglected complication of high levels of serum ferritin, i.e. an increased risk of leg dermatitis and/or ulceration.…”