• Rev Med Interne · Oct 2013

    Case Reports

    [Macrophage activation syndrome as the presenting manifestation of intravascular lymphoma].

    • E Oehler, G Soubiran, B Fabiani, O Legrand, B Rio, and F Ghawche.
    • Service de médecine interne, centre hospitalier de Polynésie française, 98713 Tahiti, Polynésie française. Electronic address: erwan.oehler@cht.pf.
    • Rev Med Interne. 2013 Oct 1;34(10):636-40.

    IntroductionIntravascular large B cell lymphoma is a neoplastic cell proliferation leading to the occlusion of the lumen of small vessels. This is a rare haematological malignancy, which is difficult to diagnose because of a heterogeneous clinical presentation.Case ReportWe report a 62-year-old man who presented a macrophage activation syndrome as the presenting manifestation of an intravascular lymphoma. This association is frequently marked by a greater severity and clinical care requires an early and appropriate treatment.ConclusionDue to the polymorphism and the systemic presentation of intravascular large B cell lymphoma, the internist may be confronted with this disease, which is considered to be more severe if associated with a macrophage activation syndrome. Awareness of the intravascular large B cell lymphoma is important because the prognosis depends on the rapidity of the initiation of chemotherapy associated with rituximab.Copyright © 2012 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.

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