Abstract. Pulmonary lymphangioleiom

Abstract. Pulmonary lymphangioleiomyomatosis (PLAM) is a rare disease occurring frequently in women. The present study reported a case of PLAM in a 46-year-old female and reviewed the literature regarding PLAM. The female experienced dyspnea on exertion for 1 month. The chest computed tomography (CT) revealed diffuse thin-walled cystic lesions, consistent with radiological features of PLAM. CT-guided lung biopsy showed that characteristic smooth muscle cells were positive for smooth muscle actin, human melanoma black 45 and D2-40 by histology and immunohistochemistry techniques, resulting in a diagnosis of PLAM. The patient had a hysterectomy for treating uterine myoma 12 years previously. Additionally, lung diffusion function was impaired slightly, abdominal CT showed a right renal cyst and pelvic ultrasound exhibited a right oophoritic cyst. Sirolimus was administered, which improved the dyspnea and pulmonary function, and the patient required long-term follow-up. Therefore, this is a rare case of PLAM with renal cyst, oophoritic cyst and uterine myoma in a female. In conclusion, PLAM is a rare proliferative disease in the lung. Chest CT characteristically showed that the cysts were bilateral, diffuse and thin-walled. Lung biopsy may provide evidence for the diagnosis of PLAM, which was treated with sirolimus.

Hasil (Bahasa Indonesia) 2:

Abstrak. lymphangioleiomyomatosis paru (Plam) adalah penyakit langka yang terjadi sering pada wanita. Penelitian ini melaporkan kasus Plam dalam perempuan berusia 46 tahun dan meninjau literatur tentang Plam. betina mengalami dyspnea saat aktivitas selama 1 bulan. Dada computed tomography (CT) mengungkapkan lesi kistik berdinding tipis difus, konsisten dengan fitur radiologi dari Plam. CT-dipandu biopsi paru menunjukkan bahwa karakteristik sel otot polos yang positif untuk aktin otot polos, melanoma manusia 45 hitam dan D2-40 oleh histologi dan imunohistokimia teknik, sehingga diagnosis Plam. Pasien memiliki histerektomi untuk mengobati mioma uteri 12 tahun sebelumnya. Selain itu, fungsi difusi paru telah rusak sedikit, perut CT menunjukkan kista ginjal kanan dan USG panggul dipamerkan kista oophoritic tepat. Sirolimus diberikan, yang meningkatkan dyspnea dan fungsi paru, dan pasien diperlukan jangka panjang tindak lanjut. Oleh karena itu, ini adalah kasus langka Plam dengan kista ginjal, kista oophoritic dan miom uterus dalam wanita. Kesimpulannya, Plam adalah penyakit proliferatif langka di paru-paru. Dada CT khas menunjukkan bahwa kista yang bilateral, difus dan berdinding tipis. biopsi paru dapat memberikan bukti untuk diagnosis Plam, yang diobati dengan sirolimus.