Aessopos Athanasios, Kati Maria, Tsironi Maria, Polonifi Ekaterini, Farmakis Dimitrios
Cardiac Outpatient Clinic for Hemoglobinopathies, First Department of Internal Medicine, University of Athens Medical School, Laiko Hospital, Athens, Greece.
Haematologica. 2006 May;91(5 Suppl):ECR11.
Thalassemia intermedia is a heterogeneous, transfusion-independent form of b-thalassemia, with a clinical course dominated by multi-organ effects of chronic tissue hypoxia, in which hemoglobin F percentage seems to play an important role. We describe the case of a transfusion-independent thalassemia intermedia patient (total hemoglobin 10.7 g/dl) with high hemoglobin F percentage (70%), who presented with persistent leg ulcerations. The patient was successfully treated with one-year exchange blood transfusions, which reduced hemoglobin F percentage to 35%.
中间型地中海贫血是一种异质性、无需输血的β地中海贫血形式,其临床病程以慢性组织缺氧的多器官影响为主,其中血红蛋白F百分比似乎起重要作用。我们描述了一例无需输血的中间型地中海贫血患者(总血红蛋白10.7 g/dl),其血红蛋白F百分比高(70%),出现持续性腿部溃疡。该患者通过为期一年的换血输血治疗成功治愈,血红蛋白F百分比降至35%。