Jornal de Hematologia e Doenças Tromboembólicas
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Acute Soft Head Syndrome in Sickle Cell Anemia: Creating a Firm Approach

Mark Drew1*, Derek Fladeland2, Roona Sinha3

Sickle Cell Disease (SCD) is the most common inherited disease worldwide and presents with a myriad of complications. A rare complication of SCD is Acute Soft Head Syndrome (ASHS). We present the case of a 14-year-old male with homozygous sickle cell anemia (HbSS) who presented with Vaso-Occlusive Crisis (VOC) complicated by the peculiar development of a scalp mass. Magnetic Resonance Imaging (MRI) showed calvarium changes consistent with ASHS. Current literature lacks standardized management for such a complication. As such, we outline recommendations for imaging modalities, therapeutic interventions, and ongoing management based on this patient’s course over two years.