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Related Experiment Videos

Electron microscopy of the plexiform lesion.

P Smith, D Heath

    Thorax
    |April 1, 1979
    PubMed
    Summary
    This summary is machine-generated.

    Electron microscopy revealed myofibroblasts and novel "fibrillary cells" in plexogenic pulmonary arteriopathy. These fibrillary cells may represent primitive cardiovascular stem cells involved in lesion organization.

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    Area of Science:

    • Cardiovascular Pathology
    • Cell Biology
    • Pulmonary Hypertension

    Background:

    • Plexogenic pulmonary arteriopathy is a severe vascular disease characterized by complex vascular remodeling.
    • The cellular components and their origins within these lesions remain incompletely understood.

    Observation:

    • Electron microscopy was used to examine pulmonary arteries from a patient with plexogenic pulmonary arteriopathy.
    • Intimal fibrosis, described as "onion-skin" type, was observed in muscular pulmonary arteries.
    • Cells within the fibrosis, termed myofibroblasts, exhibited characteristics of both smooth muscle cells and fibroblasts.

    Findings:

    • Myofibroblasts and distinct "fibrillary cells" were identified within plexiform lesions.
    • Fibrillary cells contained abundant, randomly oriented filaments and were found lining vascular channels and within the lesion stroma.

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  • These cells appeared to phagocytose fibrin and contribute to the organization of plexiform lesions into fibrous scars.

    • Implications:

    • Fibrillary cells share similarities with vasoformative reserve cells and cells found in cardiac myxomas and valve tumors.
    • This suggests fibrillary cells may be primitive, multipotential cardiovascular stem cells.
    • Understanding these cells could offer new insights into the pathogenesis and potential therapeutic targets for pulmonary arteriopathy.