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Coagulation Disorders Associated with Vascular Anomalies

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Hemangiomas and Vascular Malformations

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Abstract

Coagulation disorders have been associated with vascular anomalies. Kasabach–Merritt phenomenon (KMP) describes thrombocytopenia and consumptive coagulopathy in the presence of a kaposiform hemangioendothelioma (KHE) or a tufted angioma (TA). A variant of KMP is associated with multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT). Localized intravascular coagulopathy (LIC) is encountered in 45 % of venous malformations, and it is characterized by high D-dimer level, low fibrinogen, and normal platelet count. All these patients are at risk of potential aggravation to disseminated intravascular coagulopathy (DIC). Clinicians and biologists must work in concert to diagnose the vascular anomaly and understand the pathophysiology of the coagulation disorder. Although the coagulation activation always generates thrombocytopenia and elevation of D-dimer levels, the targets of the treatment are different: tumor for KMP and consumption of the coagulation factors for LIC.

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Authors and Affiliations

  1. Department of Dermatology, Hospital center of Caen, University of Caen Basse-Normandie, Caen Cedex 9, France

    Anne Dompmartin MD, PhD & Morgane Barreau MD

  2. Department of Hematology, Hospital center of Caen, University of Caen Basse-Normandie, Caen Cedex 9, France

    Yohann Repessé MD & Agnès Le Querrec MPhSc

Authors
  1. Anne Dompmartin MD, PhD
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  2. Morgane Barreau MD
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  3. Yohann Repessé MD
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  4. Agnès Le Querrec MPhSc
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Corresponding author

Correspondence to Anne Dompmartin MD, PhD .

Editor information

Editors and Affiliations

  1. Department of Vascular Surgery, Center for Vascular Malformations "Stefan Belov", Clinical Institute Humanitas "Mater Domini", Castellanza (Varese), Italy

    Raul Mattassi

  2. Vascular Malformations, Facharztklinik Hamburg, Hamburg, Germany

    Dirk A. Loose

  3. Department of Vascular Surgery, "G. Salvini" Hospital, Garbagnate Milanese (Milan), Italy

    Massimo Vaghi

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Dompmartin, A., Barreau, M., Repessé, Y., Le Querrec, A. (2015). Coagulation Disorders Associated with Vascular Anomalies. In: Mattassi, R., Loose, D., Vaghi, M. (eds) Hemangiomas and Vascular Malformations. Springer, Milano. https://doi.org/10.1007/978-88-470-5673-2_4

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  • DOI: https://doi.org/10.1007/978-88-470-5673-2_4

  • Publisher Name: Springer, Milano

  • Print ISBN: 978-88-470-5672-5

  • Online ISBN: 978-88-470-5673-2

  • eBook Packages: MedicineMedicine (R0)

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