title: [Macrophage activation syndrome as the presenting manifestation of intravascular lymphoma].
abstract: BACKGROUND
  Intravascular 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.
  
  
  METHODS
  We 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.
  
  
  CONCLUSIONS
  Due 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.
authors: Service de médecine interne, centre hospitalier de Polynésie française, 98713 Tahiti, Polynésie française. Electronic address: erwan.oehler@cht.pf.
authors: E
authors: E
authors: Oehler
authors: G
authors: G
authors: Soubiran
authors: B
authors: B
authors: Fabiani
authors: O
authors: O
authors: Legrand
authors: B
authors: B
authors: Rio
authors: F
authors: F
authors: Ghawche
mesh_terms: D006801:Humans; D016403:Lymphoma, Large B-Cell, Diffuse / Q000150:complications* / Q000175:diagnosis*; D055501:Macrophage Activation Syndrome / Q000175:diagnosis* / Q000209:etiology*; D008297:Male; D008875:Middle Aged; D019043:Vascular Neoplasms / Q000150:complications* / Q000175:diagnosis*
keywords: Intravascular lymphoma; Lymphome intravasculaire; Macrophage activation syndrome; Syndrome d’activation macrophagique