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Lymphangioleiomyomatosis

  • Helmut H. Popper
  • Nader Morad
  • Issam A. Al-Bozom
  • Imaad Bin Mujeeb
  • Armando E. Fraire
Chapter

Abstract

A unique disorder, lymphangioleiomyomatosis (LAM) is characterized by proliferation of small myoid cells with formation of numerous cystic spaces that greatly distort the normal architecture of the lung. Currently, LAM is regarded as a member of the family of PEComas (tumors derived from perivascular epithelial cells) and is closely associated with tuberous sclerosis, multifocal micronodular pneumocyte hyperplasia, and angiomyolipomas of the kidney.

Keywords

Tuberous Sclerosis Myoid Cell Perivascular Epithelioid Cell Cystic Lung Disease Unique Disorder 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Copyright information

© Springer Science+Business Media, LLC 2010

Authors and Affiliations

  • Helmut H. Popper
    • 1
  • Nader Morad
    • 2
  • Issam A. Al-Bozom
    • 3
  • Imaad Bin Mujeeb
    • 3
  • Armando E. Fraire
    • 4
  1. 1.University of Graz School of MedicineGrazAustria
  2. 2.University of Massachusetts Medical SchoolWorcesterUSA
  3. 3.Hamad Medical CorporationDohaQatar
  4. 4.University of Massachusetts Medical SchoolWorcesterUSA

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