Lesioni ossee simmetriche e drepanocitosi

Abstract

An 8-year-old boy suffering from sickle cell disease (SCD) was admitted to emergency unit complaining of moderate-to-severe pain in his lower limbs. He received chronic transfusion therapy and hydroxyurea. Apparently, he had never experienced vaso-occlusive crises (VOC) before. At presentation he suffered pain in his left lower limb with mild functional limitation. Standard radiography was negative. Blood tests were consistent with chronic haemolytic anaemia, normal blood cell count and slight increase in inflammation markers. Blood cultures were negative. In the suspicion of a VOC, hydration and antalgic therapy with intravenous acetaminophen and ibuprofen were administered. Due to a poor clinical response, lower limbs MRI was performed and, interestingly, it revealed bilateral symmetrical tibial bone infarctions and analgesics were switched to tramadol in continuous infusion resulting in good pain control. He was discharged after nine days of hospitalization with complete remission of symptoms. SCD is a spectrum of hereditary haemoglobinopathies identified by abnormal haemoglobin S (HbS) molecule. Painful crisis are the most frequent complications and VOCs virtually affect every patient with SCD. MRI can be useful to identify skeletal abnormalities, even in the pain free period and to distinguish acute infarction from acute osteomyelitis. When available, all SCD patients suffering from significant acute bone pain should undergo an MRI study of the painful region as the initial imaging investigation because, even in patients who have never experienced VOCs, there may already be bone lesions at the first painful crisis.