Pneumocystis Jirovecii Pneumonia During Sirolimus Therapy for Kaposiform Hemangioendothelioma

Pediatrics. 2018 Apr;141(Suppl 5):S421-S424. doi: 10.1542/peds.2017-1044.


Sirolimus is an effective therapy for children with kaposiform hemangioendothelioma with or without the Kasabach-Merritt phenomenon. We report the case of a child with kaposiform hemangioendothelioma and the Kasabach-Merritt phenomenon who developed Pneumocystis carinii pneumonia (PCP) while on sirolimus and a prednisolone taper, after lack of adequate response to prednisolone, propranolol, and vincristine. He had a prompt positive clinical and laboratory response to sirolimus, but 4 weeks after starting it, at the age of 4 months, he developed PCP. This led to respiratory failure, which required extracorporeal membrane oxygenation. Sirolimus was temporarily discontinued, and he was successfully treated for PCP with sulfamethoxazole-trimethoprim and methylprednisolone. He was restarted on sirolimus 3 weeks after discharge and given sulfamethoxazole-trimethoprim prophylaxis. At the age of 22 months, while still on sirolimus, the lesion continued to improve with test results revealing stable hemoglobin and platelet counts. PCP is a rare but life-threatening side effect of sirolimus therapy, especially in the setting of concurrent steroid treatment. Pneumocystis prophylaxis should be considered for patients receiving sirolimus.

Publication types

  • Case Reports

MeSH terms

  • Anti-Bacterial Agents / therapeutic use
  • Antibiotics, Antineoplastic / adverse effects*
  • Antibiotics, Antineoplastic / therapeutic use
  • Antineoplastic Agents, Phytogenic / therapeutic use
  • Drug Therapy, Combination
  • Hemangioendothelioma / complications
  • Hemangioendothelioma / drug therapy*
  • Humans
  • Immunosuppressive Agents / adverse effects*
  • Immunosuppressive Agents / therapeutic use
  • Infant
  • Kasabach-Merritt Syndrome / complications
  • Kasabach-Merritt Syndrome / drug therapy*
  • Male
  • Pneumocystis carinii*
  • Pneumonia, Pneumocystis / complications
  • Pneumonia, Pneumocystis / diagnosis*
  • Pneumonia, Pneumocystis / drug therapy
  • Prednisolone / therapeutic use
  • Propranolol / therapeutic use
  • Respiratory Insufficiency / etiology
  • Sarcoma, Kaposi / complications
  • Sarcoma, Kaposi / drug therapy*
  • Sirolimus / adverse effects*
  • Sirolimus / therapeutic use
  • Vincristine / therapeutic use


  • Anti-Bacterial Agents
  • Antibiotics, Antineoplastic
  • Antineoplastic Agents, Phytogenic
  • Immunosuppressive Agents
  • Vincristine
  • Prednisolone
  • Propranolol
  • Sirolimus

Supplementary concepts

  • Kaposiform Hemangioendothelioma