Characteristics of Fatal Infections
Six infectious deaths were reported in patients with VAs receiving sirolimus (Table 3). One patient received PJP prophylaxis, one did not, and 4 studies did not report prophylaxis status. All of these patients were 2 years or younger, and 5/6 patients were infants (< 12 months of age). Four patients had KHE with KMP, one patient had microcystic lymphatic malformation, and one patient had generalized lymphatic anomaly (GLA). These fatal infections occurred within 2 months of starting sirolimus in 4/6 cases. Five of these deaths resulted from pneumonia and one case did not specify the type of infection. One patient with viral pneumonia also had ileitis. The responsible pathogens included viral (adenovirus, metapneumovirus), bacterial (Mycoplasma ), and unknown pathogens. Sirolimus was held at the time of infection in all cases. Co-administered steroids were not specified in 3/6 cases. Two patients were weaning off steroids at the time of the infection, and one patient did not receive any steroids. Deaths occurred 4 to 11 days after onset of infection. Further information on possible comorbidities was not reported.