Effective Initial Treatment of Diffuse Pulmonary Lymphangiomatosis with Sirolimus and Propranolol: A Case Report

Dimiene, Ieva; Biekšienė, Kristina; Zaveckienė, Jurgita; Andrulis, Mindaugas; Optazaite, Daiva-Elzbieta; Vagulienė, Neringa; Žemaitis, Marius; Miliauskas, Skaidrius

doi:10.3390/medicina57121308

Cited by 1 publication

(2 citation statements)

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“…There are no standardized treatment guidelines for DPL, owing to its rarity. However, various pharmacological therapies have been tried, including bevacizumab, sildenafil, thalidomide, propranolol, and sirolimus 10 . Reports utilizing sirolimus in DPL treatment are extremely limited.…”

Section: Discussionmentioning

confidence: 99%

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Pleural effusion due to lymphangiomatosis and the role of sirolimus: A case report

Riazuddin,

Farouk,

Ali

et al. 2023

Annals of Medicine &Amp; Surgery

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Introduction: Lymphangiomatosis is a rare abnormal proliferation of lymphatic vessels involving multiple organs like the brain, lung, heart, spleen, liver, and bones. Lymphangiomas constitute 5.6% of all benign tumors in infancy and adulthood. Case Presentation: We report a case of a young lady who presented with constitutional symptoms and progressive dyspnea. Her medical history is significant for muco-cutaneous albinism, diffuse hemangiomas of the bone and viscera, and consumptive coagulopathy status post-splenectomy. After initial investigations, she was found to have right-sided pleural effusion. Pleural fluid analysis indicated chylothorax. She had multiple drainages of the pleural fluid done, and afterward, ligation of the right thoracic duct was performed with a trial of sirolimus, which improved her chylothorax. Clinical Discussion: Several case reports have reported positive outcomes with sirolimus in the treatment of lymphangiomatosis. However, larger controlled studies are needed to confirm these findings. Conclusion: Sirolimus is promising as medical treatment for diffuse pulmonary lymphangiomatosis.

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Section: Discussionmentioning

confidence: 99%

“…Dimiene et al . 10 described the case of a 26-year-old man with progressive dyspnea, hemoptysis, and night sweats due to underlying DPL. The authors reported successful treatment with sirolimus and propranolol, which was used to control tachycardia and support treatment by reducing vascular endothelial growth factor levels.…”

Section: Discussionmentioning

confidence: 99%