Intravascular lymphoma associated with haemophagocytic syndrome: a very rare entity in western countries.
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.
Terrier
Benjamin
B
Department of Adult Haematology, Necker Hospital, 149-161 rue de Sevres, 75743 Paris Cedex 15, France.
Aouba
Achille
A
Vasiliu
Viorel
V
Charlier
Caroline
C
Delarue
Richard
R
Buzyn
Agnès
A
Hermine
Olivier
O
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
D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D000069283:Rituximab