Kaposiform hemangioendothelioma (KHE) is a rare, vascular malignancy that is often associated with coagulopathies and thrombocytopenia secondary to platelet trapping. Typically, a person diagnosed with KHE with Kasabach-Merritt phenomenon (KMP) presents with a reddish-purplish lesion, thrombocytopenia, and elevated D-dimer, which can lead to high morbidity and mortality. Sirolimus has been identified as a treatment option for KHE with or without KMP for reduction in lesion size and hematologic parameters. In this case report, a female born at 26.5 weeks was noted at birth to have a purpuric lesion on her right upper back and flank area. She was diagnosed with biopsy-confirmed KHE with KMP. She was started on sirolimus 0.01 mg (0.02 mg/kg; 0.14 mg/m2) once a day, and because of high trough concentrations treatment was held until concentrations decreased. Sirolimus was then microdiluted to a 0.01 mg/mL concentration in medium-chain triglyceride oil for administration. Prior to discharge from the hospital the commercially available product was dispensed for home use. After 6 months of treatment, she achieved a reduction in lesion size and improvement in hematologic parameters, and treatment was stopped at 9 months.
OBJECTIVE
Recently, a premature neonate, born at 26.5 weeks, was treated with sirolimus for kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon at the University of New Mexico Children's Hospital. Because of the lipophilic properties of the drug and an inability to draw the correct dose needed for the neonate with the standard 1 mg/mL concentration, sirolimus was diluted to 0.01 mg/mL (10 mg/L) in medium-chain triglyceride (MCT) oil. The objective of this study was to evaluate the stability of sirolimus diluted in MCT oil.
METHODS
Commercially available sirolimus oral solution was diluted with MCT oil from 1 mg/mL to 10 mg/L. The diluted samples were prepared by measuring 0.1 mL of the commercial product in an oral syringe and mixing with 9.9 mL of MCT oil. The 3 diluted samples were placed in amber glass vials, stored at 10°C, and analyzed over 14 days.
RESULTS
The initial concentration of the 3 samples ranged from 8.5 to 10.8 mg/L sirolimus, or 85% to 108% of the target value. No significant differences were seen between average concentrations on the days tested. However, the average drug concentration fell to approximately 90% of the theoretical 10 mg/L target concentration between days 7 and 10.
CONCLUSIONS
This study shows that sirolimus oral solution diluted with MCT oil to a final concentration of 10 mg/L was stable for at least 7 days when stored at 10°C, suggesting that the beyond-use date should be 7 days.
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