Intravascular lymphoma associated with hemophagocytic syndrome: a rare but aggressive clinical entity.
We report a 55-year-old male with a diagnosis of intravascular lymphoma and hemophagocytosis. He initially presented with hemolytic anemia and splenomegaly and was successfully treated with oral steroids. His clinical course was later complicated by fever, cytopenias, hypoalbuminemia, disseminated intravascular coagulation, gastrointestinal bleeding and acute tubular necrosis. Results of an extensive investigation for fever of unknown origin were negative. Although the patient was treated aggressively with antimicrobials, transfusion support and dialysis, he expired 3 weeks after hospitalization. Post-mortem analysis revealed large CD30- and CD45-positive lymphoma cells in an intravascular distribution in most of the organs studied. Histopathology of the spleen and bone marrow was significant for fulminant hemophagocytosis.
Bhagwati
Niyati S
NS
Comprehensive Cancer Center, Our Lady of Mercy Medical Center, 600 E 233rd Street, Bronx, NY 10466, USA. nbhagwati@hotmail.com
Oiseth
Stanley J
SJ
Abebe
Lool S
LS
Wiernik
Peter H
PH
D001344:Autopsy; D015616:Histiocytosis, Non-Langerhans-Cell / Q000473:pathology*; D006801:Humans; D016403:Lymphoma, Large B-Cell, Diffuse / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D019043:Vascular Neoplasms / Q000473:pathology*