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  • Title: Intravascular lymphoma associated with haemophagocytic syndrome: a very rare entity in western countries.
    Author: Terrier B, Aouba A, Vasiliu V, Charlier C, Delarue R, Buzyn A, Hermine O.
    Journal: Eur J Haematol; 2005 Oct; 75(4):341-5. PubMed ID: 16146541.
    Abstract:
    Intravascular lymphoma (IVL) is a rare and aggressive disorder, characterised by frequent cutaneous and neurological involvement and medullary infiltration. In rare cases particularly in Asia, IVL can be associated with haemophagocytic syndrome (IVL-HS). Here, we report the case of a 61-year-old Caucasian female who presented with IVL-HS. Bone marrow biopsy showed haemophagocytic features and medullary localisation of a diffuse large B-cell lymphoma. Liver biopsy showed exclusive sinusoidal infiltration by large B cells. Treatment by polychemotherapy associated with rituximab induced a rapid complete remission. Unfortunately, death occurred as a consequence of septic shock. Early recognition of IVL-HS by performing bone marrow biopsy is critical to start rapidly appropriate treatment. The role of rituximab in the management of IVL-HS remains to be established.
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