Lymphangiomatosis

Changed by Henry Knipe, 21 Sep 2021
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Lymphangiomatosis refers tois a rare mesenchymal disorder, which that is characterised by developmental "malformation" of multiple lymphatic channels (usually with dilatation). If

Terminology

If lymphatic channels are purely dilated and not malformed is it usuallythe term lymphangiectasia is usually used.

If it If lymphangiomatosis occurs in a singular localised form it is then termed athe term lymphangioma. If its can be used, and if it is widespread throughout the body it forms the spectrum of athen generalised lymphatic anomaly can be used.

Pathology

The condition can have various forms depending on organ involvement and can include :

Specific forms

  • multisystem
    • Associations conditions

    See also
    • -<p><strong>Lymphangiomatosis </strong>refers to rare mesenchymal disorder, which is characterised by developmental "malformation" of multiple lymphatic channels (usually with dilatation). If purely dilated and not malformed is it usually <a href="/articles/lymphangiectasia">lymphangiectasia</a>.</p><p>If it occurs in a singular localised form it is then termed a<a href="/articles/lymphatic-malformations-1"> lymphangioma</a>. If its widespread throughout the body it forms the spectrum of a <a href="/articles/generalised-lymphatic-anomaly">generalised lymphatic anomaly</a>.</p><p>The condition can have various forms depending on organ involvement and can include </p><ul>
    • -<li>renal<ul><li>
    • -<a href="/articles/renal-lymphangiectasia">perirenal lymphangiomatosis</a> / <a href="/articles/renal-lymphangiectasia">renal lymphangiomatosis</a><ul><li><a href="/articles/familial-renal-lymphangiomatosis">familial renal lymphangiomatosis</a></li></ul>
    • -</li></ul>
    • +<p><strong>Lymphangiomatosis </strong>is a rare mesenchymal disorder that is characterised by developmental "malformation" of multiple lymphatic channels (usually with dilatation).</p><h4>Terminology</h4><p>If lymphatic channels are purely dilated and not malformed the term <a href="/articles/lymphangiectasia">lymphangiectasia</a> is usually used. If lymphangiomatosis occurs in a singular localised form it is then the term <a href="/articles/lymphatic-malformations-1">lymphangioma</a> can be used, and if it is widespread throughout the body then <a href="/articles/generalised-lymphatic-anomaly">generalised lymphatic anomaly</a> can be used.</p><h4>Pathology</h4><p>The condition can have various forms depending on organ involvement:</p><ul>
    • +<li>
    • +<a href="/articles/renal-lymphangiomatosis">perirenal</a>/<a href="/articles/renal-lymphangiectasia">renal lymphangiomatosis</a>
    • -<li>thoracic <ul><li>
    • +<li>
    • -<a href="/articles/pulmonary-lymphangiectasia">pulmonary lymphangiomatosis</a> /<a href="/articles/pulmonary-lymphangiectasia"> pulmonary lymphangiectasia</a>
    • -</li></ul>
    • +<a href="/articles/pulmonary-lymphangiectasia">pulmonary lymphangiomatosis</a> / <a href="/articles/pulmonary-lymphangiectasia">pulmonary lymphangiectasia</a>
    • -<li>abdominal <ul><li>hepatic - <a href="/articles/hepatic-lymphangiomatosis">hepatic lymphangiomatosis</a>
    • -</li></ul>
    • +<li>abdominal <ul><li><a href="/articles/hepatic-lymphangiomatosis">hepatic lymphangiomatosis</a></li></ul>
    • +</li>
    • +<li>multisystem<a href="/articles/hepatic-lymphangiomatosis"> </a><ul><li><a href="/articles/kaposiform-lymphangiomatosis-1">Kaposiform lymphangiomatosis</a></li></ul>
    • -</ul><p>Specific forms </p><ul><li><a href="/articles/kaposiform-lymphangiomatosis-1">Kaposiform lymphangiomatosis</a></li></ul><h5>Associations conditions</h5><ul><li>
    • +</ul><h5>Associations</h5><ul><li>
    • -</li></ul><h4>See also</h4><ul><li><a href="/articles/lymphangioleiomyomatosis-1">lymphangioleiomyomatosis (LAM)</a></li></ul>
    • +</li></ul>

    References changed:

    • 1. Blei F. Lymphangiomatosis: Clinical Overview. Lymphatic Research and Biology. 2011;9(4):185-90. <a href="https://doi.org/10.1089/lrb.2011.0020">doi:10.1089/lrb.2011.0020</a>
    • 2. Gordon K & Mortimer P. Progressive Lymphangiomatosis and Gorham's Disease: Case Report and Clinical Implications. Lymphatic Research and Biology. 2011;9(4):201-4. <a href="https://doi.org/10.1089/lrb.2011.0021">doi:10.1089/lrb.2011.0021</a>
    • 3. Jin D, Sun X, Shen W, Zhao Q, Wang R. Diagnosis of Lymphangiomatosis: A Study Based on CT Lymphangiography. Academic Radiology. 2020;27(2):219-26. <a href="https://doi.org/10.1016/j.acra.2019.03.024">doi:10.1016/j.acra.2019.03.024</a>
    • 1. Blei F. Lymphangiomatosis: Clinical Overview. Lymphat Res Biol. 2011;9(4):185-90. <a href="https://doi.org/10.1089/lrb.2011.0020">doi:10.1089/lrb.2011.0020</a>
    • 2. Gordon K & Mortimer P. Progressive Lymphangiomatosis and Gorham's Disease: Case Report and Clinical Implications. Lymphat Res Biol. 2011;9(4):201-4. <a href="https://doi.org/10.1089/lrb.2011.0021">doi:10.1089/lrb.2011.0021</a>
    • 3. Jin D, Sun X, Shen W, Zhao Q, Wang R. Diagnosis of Lymphangiomatosis: A Study Based on CT Lymphangiography. Acad Radiol. 2020;27(2):219-26. <a href="https://doi.org/10.1016/j.acra.2019.03.024">doi:10.1016/j.acra.2019.03.024</a>

    Tags changed:

    • cases

    Systems changed:

    • Urogenital

    Updates to Synonym Attributes

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