CHYLOPERITONEUM, CHYLOTHORAX AND LOWER EXTREMITY LYMPHEDEMA IN WOMAN WITH SPORADIC LYMPHANGIOLEIOMYOMATOSIS SUCCESSFULLY TREATED WITH SIROLIMUS: A CASE REPORT

A Chachaj, K Drozdz, M Chabowski, P Dziegiel, I Grzegorek, A Wojnar, P Jazwiec, A Szuba

Abstract


Lymphangioleiomyomatosis (LAM) is arare disease characterized by diffuse thinwalledcysts throughout the lungs on computedtomography and diffuse proliferation ofabnormal smooth muscle-like cells (LAMcells) on lung biopsy. LAM affects womenalmost exclusively, predominantly in theirreproductive age. The most typical presentingsymptoms include dyspnea, spontaneouspneumothorax, cough and chylothorax.Abdominal findings represent less commoninitial manifestations of the disease and maypose diagnostic difficulties. The treatment ofLAM has not been fully established. Recentstudies report effectiveness of sirolimus inLAM patients.We report the case of a 45-year-old womanwith sporadic LAM, successfully treated withsirolimus, in whom the first manifestationof the disease was chyloperitoneum and afterthree and nine years, respectively, lymphedemaof the left lower extremity and rightsided chylothorax occurred.

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