title: Intravascular lymphoma associated with haemophagocytic syndrome: a very rare entity in western countries.
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.
authors.affiliation: Department of Adult Haematology, Necker Hospital, 149-161 rue de Sevres, 75743 Paris Cedex 15, France.
authors.forename: Benjamin
authors.initials: B
authors.lastname: Terrier
authors.forename: Achille
authors.initials: A
authors.lastname: Aouba
authors.forename: Viorel
authors.initials: V
authors.lastname: Vasiliu
authors.forename: Caroline
authors.initials: C
authors.lastname: Charlier
authors.forename: Richard
authors.initials: R
authors.lastname: Delarue
authors.forename: Agnès
authors.initials: A
authors.lastname: Buzyn
authors.forename: Olivier
authors.initials: O
authors.lastname: Hermine
mesh_terms: D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D005260:Female; D015616:Histiocytosis, Non-Langerhans-Cell / Q000150:complications* / Q000188:drug therapy / Q000453:epidemiology; D006801:Humans; D008223:Lymphoma / Q000188:drug therapy / Q000209:etiology*; D016403:Lymphoma, Large B-Cell, Diffuse / Q000209:etiology*; D008875:Middle Aged; D009361:Neoplasm Invasiveness; D012074:Remission Induction; D000069283:Rituximab; D012772:Shock, Septic; D019043:Vascular Neoplasms / Q000188:drug therapy / Q000453:epidemiology / Q000209:etiology*; D019366:Western World
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D000069283:Rituximab