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Intravascular lymphoma associated with haemophagocytic syndrome: a very rare entity in western countries
Author(s) -
Terrier Benjamin,
Aouba Achille,
Vasiliu Viorel,
Charlier Caroline,
Delarue Richard,
Buzyn Agnès,
Hermine Olivier
Publication year - 2005
Publication title -
european journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0902-4441
DOI - 10.1111/j.1600-0609.2005.00519.x
Subject(s) - medicine , bone marrow , pathology , lymphoma , rituximab , septic shock , medullary cavity , biopsy , intravascular large b cell lymphoma , infiltration (hvac) , sepsis , physics , thermodynamics
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.