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  • Title: [Pulmonary lymphangioleiomyomatosis].
    Author: Dvorakovskaia IV, Baranova OP.
    Journal: Arkh Patol; 2005; 67(5):29-32. PubMed ID: 16323477.
    Abstract:
    A clinicomorphological analysis of 41 pulmonary lymphangioleiomyomatosis (PLAM) cases has been performed. Focal (7 cases) and diffuse (34 cases) forms were identified. Expression of NMB-45, desmin, actin, vimentin prove myogenic nature of the disease and confirm combination of two morphogenesis directions - proliferation of smooth muscle cells at the active phase of the growth and fibrosis at late stages. The presence of estrogen and progesteron receptors in smooth muscle cells shows hormonal dependance of cell proliferation. Thus, LAM is multisystem disease linked with abdominal tumors (angiomyolipomas, angioleiomyomas). Prognosis in the focal form is favourable, that in the diffuse form- unfavourable.
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