Infection Characteristics
The type of infection was reported for 81% of infections (n=255/315).
Of these, most infections were reported as upper respiratory infections
or “viral infections” (n=137, 54%), followed by pneumonia (n=52,
20%), and cutaneous infections (n=20, 8%). Within the pneumonia
category, one patient was reported to havePneumocystis jiroveciipneumonia (PJP).25 This patient was 4 months old with
a diagnosis of KHE with Kasabach-Merritt Phenomenon (KMP), receiving
treatment with sirolimus at 0.05 mg/kg per dose twice a day with a
trough goal of 8 to 15 ng/mL. At 2 months of age, this patient was
treated with vincristine, prednisolone, and propranolol. At 3 months of
age, the patient discontinued vincristine and propranolol, started
sirolimus, and began a steroid wean. Four weeks after initiation of
sirolimus therapy, the patient developed PJP. Sirolimus was temporarily
held, and the pneumonia was treated successfully with
sulfamethoxazole-trimethoprim and methylprednisolone. Notably, this
patient had not received PJP prophylaxis. Four cases of bacteremia (1%)
were reported. Common Terminology Criteria for Adverse Events (CTCAE)
grading was reported for 91% of infections. Most infections were grade
1 to 2 (n=194, 66%), and 32% (n=95) were grade 3 to 4. Six patients
(n=2%) had grade 5 infections (i.e., they died as a result of
infection). Most patients received antibiotic prophylaxis against PJP
(n=175, 80%), while 42 (19%) did not receive prophylaxis. Of note,
prophylaxis was not specified for 73 patients.