­­Abbreviations: KHE, kaposiform hemangioendothelioma; CMV, cytomegalovirus; PJP,pneumocystis jirovecii pneumonia. a In KHE category, 11 patients with Kasabach Merrit Phenomenon (KMP), 12 patients in which KMP was not specified, and 1 patient with Tufted Angioma.b In Complex Lymphatic Anomaly category, 4 patients with Generalized Lymphangiomatosis, 2 patients with Gorham Stout Disease, and 1 patient with Central Conducting Lymphatic Anomaly.cMore than one infection was noted for some patients, so number of infections exceeds number of reported patients. Additionally, the type of infection was not specified for 60 patients.d URI/”Viral Infections” includes reported cases of Flu and COVID-19. e Pneumonia category includes 5 bacterial pathogens (including 1 pneumocystis jirovecii pneumonia), 8 viral pathogens, and 39 unspecified pathogens.f Grade of infection was not reported for 20 infections. g While some reported actual trough prior to infection, the vast majority of studies reported the goal range of troughs maintained during treatment. Of note, 16 participants counted in the “2 to 5” range were actually reported at “2 to 6”. Similarly, in the “5 to 10” range, 25 were actually reported as “4 to 12” and 8 were reported as “4 to 13”. Sirolimus dosing was not specified for 45 patients. h Antibiotic prophylaxis was not specified for 72 patients.
Table 3. Characteristics of Fatal Infections (n=5)