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.