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Stanozolol treatment for successful prevention of attacks of severe primary cryofibrinogenemia
Sule Unal1, Fehime Kara, Seza Ozen
1Faculty of Medicine, Department of Pediatrics, Division of Pediatric Hematology, Hacettepe University, Ankara, Turkey. suleunal@hacettepe.edu.tr
Insights
Severe essential cryofibrinogenemia in children is rare and difficult to manage. Stanozolol prophylaxis effectively prevented new skin lesions in an 11-year-old boy unresponsive to other treatments.
Area of Science:
- Pediatric Hematology
- Rare Diseases
- Dermatology
Background:
- Severe essential cryofibrinogenemia is a rare pediatric condition.
- Diagnosis and management pose significant challenges for pediatricians.
- Previous treatments were ineffective for this patient.
Observation:
- An 11-year-old male presented with a history of digital loss due to cold exposure.
- The patient was diagnosed with primary cryofibrinogenemia after extensive medical evaluations.
- Conventional therapies including calcium channel blockers, acetyl salicylic acid, pentoxifylline, dextran, and steroids failed to improve his condition.
Findings:
- Stanozolol prophylaxis (2 mg/day orally) was initiated for the patient.
- No new skin lesions developed after starting stanozolol treatment.
- Existing lesions showed signs of healing during stanozolol therapy.
Implications:
- Stanozolol may be an effective treatment option for severe pediatric cryofibrinogenemia.
- This case highlights the potential of stanozolol in managing refractory cryofibrinogenemia.
- Further research is warranted to confirm stanozolol's efficacy and safety in pediatric patients.
Abstract:
Severe essential cryofibrinogenemia is rare in childhood, and both the diagnosis and the management are challenging for pediatricians. An 11-year-old male, who had already lost two digits following cold exposure, was referred after multiple visits to various hospitals and subsequently diagnosed as primary cryofibrinogenemia. His history revealed unresponsiveness to calcium channel blockers, acetyl salicylic acid, pentoxifylline, dextran, and steroids. Stanozolol (2 mg/day, orally) prophylaxis was initiated and no new skin lesions developed following starting this treatment. Some of the newly formed lesions at the onset of stanozolol healed.
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