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Intravascular Lymphoma Presenting as Telangectasias: Response to Rituximab and Combination Chemotherapy

  • Case Report
  • Published:
Journal of Cutaneous Medicine and Surgery: Incorporating Medical and Surgical Dermatology

Abstract

Background: Intravascular lymphoma is a rare and aggressive disease with abnormal lymphocytes confined within blood vessels. Cutaneous findings are common in the presentation of this disease. Objectives: The objectives of this article are (1) to describe a case of intravascular lymphoma treated with chemotherapy and rituximab and (2) to review the literature with respect to skin findings and outcomes of these patients after chemotherapy. Patient: Our patient had an unusual presentation of intravascular lymphoma characterized by tender, slightly indurated plaques composed of dense telangectatic vessels. Results: Our patient responded to combination chemotherapy with the addition of a partial course of rituximab (anti-CD20 monoclonal antibody). Conclusion: Intravascular lymphoma commonly presents with skin findings of tender indurated plaques and nodules. Although a number of clinical variations exist, telangectatic plaques are rarely described. Including our case, 50 patients have been reported after treatment with chemotherapy. Approximately 46% of treated patients exhibit either partial or complete response to chemotherapy. This is the first reported case of intravascular lymphoma treated successfully with the addition of rituximab. Overall, this malignancy remains an unusual and aggressive disease. Recognition of the cutaneous findings and early treatment with chemotherapy may alter the course of this disease. Rituximab may be an important addition to combination chemotherapy in treating intravascular lymphoma.

Antécédents: Le lymphome intravasculaire est une maladie rare et agressive qui se caractérise par la présence dans les vaisseaux sanguins de lymphocytes anormaux. Les affections cutanées sont fréquentes. Objectifs: L’objectif de cette étude est 1) de décrire un cas de lymphome intravasculaire traité par chimiothérapie et rituximab et 2) de passer en revue la littérature médicale afin de recenser les troubles dermatologiques associés à la chimiothérapie. Méthode: Le patient présentait un cas inhabituel de lymphome intravasculaire se caractérisant par des plaques de veines télangiectasiques densesm, légèrement endurées et sensibles. Résultats: Le patient a réagi à une chimiothérapie en combinaison et au rituximab (anticorps monoclonal anti-CD20). Conclusion: Le lymphome intravasculaire s’associe souvent à des manifestations dermiques de plaques et de nodules indurés et sensibles. Bien qu’un nombre de variations cliniques existe, les plaques télangiectasiques sont rarement décrites. En tout, 50 patients, y compris notre cas, ont été signalés à la suite de traitements de chimiothérapie. Environ 46% des patients traités guérissent complètement ou partiellement. Il s’agit du premier cas signalé de traitement réussi du lymphome intravasculaire par ajout de rituximab. Il s’agit d’une maladie inhabituelle et agressive. Le diagnostic d’une affection dermique et la chimiothérapie précoce pourraient altérer le cours de la maladie. Le rituximab serait un important ajout à la chimiothérapie en combinaison dans le traitement du lymphome intravasculaire.

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Authors and Affiliations

  1. Division of Dermatology, University of British Columbia, Vancouver, British Columbia, Canada

    Gabriele Weichert & Jason K. Rivers

  2. Department of Pathology, Laboratory of Medicine, University of British Columbia, Vancouver, British Columbia, Canada

    Magdalena Martinka

  3. British Columbia Cancer Agency, Vancouver, British Columbia, Canada

    Jason K. Rivers

Authors
  1. Gabriele Weichert
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  2. Magdalena Martinka
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  3. Jason K. Rivers
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Correspondence to Jason K. Rivers.

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Weichert, G., Martinka, M. & Rivers, J.K. Intravascular Lymphoma Presenting as Telangectasias: Response to Rituximab and Combination Chemotherapy . JCMS 7, 460–463 (2003). https://doi.org/10.1007/s10227-003-0153-6

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  • DOI: https://doi.org/10.1007/s10227-003-0153-6

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