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Updated: Nov 9, 2025

A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
[Carpal Extensor Tenosynovitis and Adult Sickle Cell Vaso-Occlusive Crisis]
N Vaserman1, N Boizan1, S Remadjidobian2
1Service de médecine interne-rhumatologie, CHC Cayenne, BP 6006, 97306 Cayenne Cedex, Guyane française, France.
Insights
Hand-foot syndrome, typically seen in young children, occurred in a 36-year-old adult with sickle cell disease (SS). This rare presentation during a vaso-occlusive crisis highlights a less recognized complication of sickle cell anemia.
Area of Science:
- Rheumatology
- Hematology
- Pediatrics
Background:
- Osteoarticular complications are common in sickle cell anemia.
- Hand-foot syndrome (dactylitis) is a characteristic manifestation typically observed in children under four years old.
- Its occurrence in adults is exceptionally rare.
Purpose of the Study:
- To report a unique case of hand-foot syndrome in an adult with sickle cell disease (SS).
- To describe the clinical presentation and diagnostic considerations for this rare manifestation.
- To emphasize the importance of considering vaso-occlusive complications in adult sickle cell disease.
Main Methods:
- Case report of a 36-year-old adult with homozygous sickle cell disease (SS).
- Clinical observation of hand-foot syndrome during a vaso-occlusive crisis, presenting as tenosynovitis of carpal extensors.
- Differential diagnosis to exclude infectious etiologies of palmar tenosynovitis.
Main Results:
- The adult patient presented with tenosynovitis mimicking infection but was diagnosed with hand-foot syndrome.
- The vaso-occlusive origin of the tenosynovitis was confirmed by the rapid clinical improvement.
- This case expands the known age range for hand-foot syndrome in sickle cell disease.
Conclusions:
- Hand-foot syndrome can occur in adult patients with sickle cell disease (SS), albeit rarely.
- Vaso-occlusive crises should be considered in the differential diagnosis of tenosynovitis in adults with sickle cell disease.
- This case underscores the diverse clinical spectrum of sickle cell anemia complications.
Abstract:
If the acute and chronic osteo-articular complications of sickle cell anemia are well-known to the rheumatologist, certain forms including hand-foot syndrome are less so since they almost exclusively concern small children less than 4 years of age. We report here the observation of a hand-foot syndrome in a 36-year-old adult, homozygous sickle cell disease (SS), during a vaso-occlusive crisis, manifested by tenosynovitis of carpal extensors. After eliminating the other etiologies, notably infectious of palmar tenosynovitis, the vaso-occlusive origin will be confirmed by the rapidly favorable evolution of the clinical picture.
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