Sickle beta+thalassemia is considered to be a mild form of sickle cell disease. However, some patients with mild disease can present with osteonecrosis. Here, we present a rare 3-year-old male who presented with acute pain, a baseline hemoglobin of 13 g/dL, who acutely developed multifocal osteonecrosis, and improved with partial exchange transfusion and hydroxyurea therapy.
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Article Synopsis
- - Multifocal osteonecrosis is a rare condition that can cause significant bone weakness and fractures, highlighted in a case of a 34-year-old woman whose symptoms worsened during pregnancy.
- - Investigations revealed the patient had an undefined autoimmune condition and low Protein S levels, along with a novel genetic variant related to Protein S deficiency.
- - The combination of inherited thrombophilia, autoimmune issues, and pregnancy-related changes contributed to the patient's severe symptoms, with her genetic variant potentially explaining the link between autoimmunity and thrombophilia.
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