Pneumocystis Jirovecii Pneumonia During Sirolimus Therapy for Kaposiform Hemangioendothelioma.

Abstract

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.

• Authors: Thomas B Russell, Emily K Rinker, Claire S Dillingham, Laurence B Givner, Thomas W McLean
• Journal: Pediatrics (Pediatrics) Vol. 141 Issue Suppl 5 Pg. S421-S424 (04 2018) ISSN: 1098-4275 [Electronic] United States
• PMID: 29610164 (Publication Type: Case Reports, Journal Article)
• Copyright: Copyright © 2018 by the American Academy of Pediatrics.

Chemical References

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

Topics

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