Lymphangioleiomyomatosis with Tuberous Sclerosis Complex-A Case Study

Overview

Journal J Pers Med

Date 2023 Nov 25

PMID 38003913

Authors

Aleksandra Marciniak

Jolanta Nawrocka-Rutkowska

Agnieszka Brodowska

Andrzej Starczewski

Iwona Szydlowska

Affiliations

Soon will be listed here.

Abstract

Lymphangioleiomyomatosis (LAM) is characterized by lung cysts that cause lung deterioration, changes in the lymphatic system, and tumors in the kidneys. It mainly affects women of reproductive age and is a progressive disease. LAM can occur as an isolated disease or coexist with tuberous sclerosis (TSC). The source of LAM cells is unknown. Patients with confirmed LAM should be treated with an mTOR inhibitor, sirolimus, or everolimus. We present a case of LAM with TSC in a patient whose symptoms, including those in the lymph nodes and chyaloperitoneum, mainly concern the abdominal cavity.

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