Lymphangioleiomyomatosis Diagnosed by Immunocytochemical and Genetic Analysis of Lymphangioleiomyomatosis Cell Clusters Found in Chylous Pleural Effusion

  • Hirama Michihiro
    Department of Respiratory Medicine, Juntendo University School of Medicine
  • Atsuta Ryo
    Department of Respiratory Medicine, Juntendo University School of Medicine
  • Mitani Keiko
    Department of Human Pathology, Juntendo University School of Medicine
  • Kumasaka Toshio
    Department of Human Pathology, Juntendo University School of Medicine
  • Gunji Yoko
    Department of Respiratory Medicine, Juntendo University School of Medicine
  • Sasaki Shin-ichi
    Department of Respiratory Medicine, Juntendo University Urayasu Hospital
  • Iwase Akihiko
    Department of Respiratory Medicine, Tokyo Metropolitan Koto Geriatric Medical Center
  • Takahashi Kazuhisa
    Department of Respiratory Medicine, Juntendo University School of Medicine
  • Seyama Kuniaki
    Department of Respiratory Medicine, Juntendo University School of Medicine

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Abstract

A 37-year-old woman presented with a cough and discomfort in the chest. Computed tomography revealed the right pleural effusion and a number of cysts in the lungs. Thoracentesis revealed LAM cell clusters (LCC) in chylous pleural effusion, confirmed by immunocytochemical examinations showing that the cells at the center of cluster were LAM cells positive for α-smooth muscle actin and HMB45 and the outer layer was lymphatic endothelium cells. When LCC were cultured in vitro, the loss of heterozygosity of TSC2 markers was detected. This case illustrates that LAM can be diagnosed by the identification of LCC without an invasive biopsy if complicated with chylous effusion.<br>

Journal

  • Internal Medicine

    Internal Medicine 46 (18), 1593-1596, 2007

    The Japanese Society of Internal Medicine

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