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Updated: Jun 13, 2025

A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
Unilateral Facial Swelling in a Sickle Cell Patient.
Rohan Akhouri1, Ali Fowler2, Colton T Schwarz2
1Pediatric Emergency Medicine, Children's Mercy Hospital, Kansas City, USA.
Acute soft head syndrome (ASHS) is a rare sickle cell disease complication. This case highlights ASHS in a young male, emphasizing early diagnosis and management for this unusual condition.
Area of Science:
- Hematology
- Pediatric Neurology
- Rare Diseases
Background:
- Sickle cell disease (SCD) is a genetic blood disorder.
- Acute soft head syndrome (ASHS) is a rare, often overlooked complication of SCD.
- ASHS diagnosis typically requires a high index of suspicion and exclusion of other conditions.
Observation:
- A case presentation of an 18-year-old male with SCD in the United States.
- The patient developed ASHS without a history of traumatic injury.
- This highlights the potential for non-traumatic onset of ASHS.
Findings:
- ASHS presents a diagnostic challenge in SCD patients.
- Early recognition is crucial for appropriate management.
- This case underscores the importance of considering ASHS in the differential diagnosis.
Implications:
- Increased awareness of ASHS among healthcare providers is needed.
- Education on recognizing and managing ASHS in SCD patients is vital.
- Further research may elucidate the pathophysiology and optimal treatment strategies for ASHS.
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