Abstract
The inherited haemoglobin disorders and the clinical laboratory is essential in the diagnosis. Phlebotomy is the therapeutic procedure indicated to reduce blood viscosity and decreasing the risk of thrombotic events in the haemoglobin variants with high affinity for O2 or vaso-occlusive crises in Sickle Cell Disease (SCD) with double heterozygosity HbSC. We describe how therapeutic bleeding remains the treatment in two variant haemoglobinopathies, Haemoglobin Regina and the Haemoglobin Trollhattan, reported for the first time in the Italian population and a double heterozygosity HbSC. In patients with high haematocrit level when other secondary conditions of polyglobulia are excluded, a High-Performance Liquid Chromatography (HPLC) analysis for haemoglobin study should be suggested. Also in children with erythrocytosis frequently, such as the finding of Haemoglobin Okazaky.
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