Dicembre 2012 - Volume XXXI - numero 10
Pagina verde
1Scuola di Specializzazione in Pediatria, Ferrara; 2UOC di Pediatria e Neonatologia, Azienda Ospedaliera di Ravenna; 3Pediatria di Libera Scelta, AUSL di Ravenna; 4UO di Ortopedia e Traumatologia Pediatrica, Istituto Ortopedico Rizzoli, Bologna
Indirizzo per corrispondenza: lorenzo.mam@libero.it
Key words: Thalasso-drepanocytosis, Mild anaemia, Osteonecrosis, Case report
The paper describes the case of an 8-year-old Moroccan girl who presented with recurrent multi-focal bone pains associated with negative flogosis indexes and standard radiological exams. The magnetic resonance exam of her thighbones showed a multi-focal inflammation; bone biopsy showed that the inflammation was aseptic and secondary to an osteonecrosis caused by vaso-occlusive crisis. After the haemoglobin electrophoresis performed in consideration of the persistent evidence of a mild microcytic anaemia with anisocytosis, the diagnosis was of thalasso-drepanocytosis. Thalasso-drepanocytosis is one of the sickle cell syndromes characterised by heterozygosis associated with thalassemic haemoglobinopathy. It can be clinically similar to homozygote drepanocytosis and therefore it needs the same therapeutic approach and follow-up. The case highlights the importance of thinking about sickle cell crisis, also in absence of important anaemization, when foreign patients coming from world’s endemic areas for thalassemic haemoglobinopathy present with recurrent bone pains.
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