Premium
Novel association of haemophagocytic syndrome with Kaposi's sarcoma‐associated herpesvirus‐related primary effusion lymphoma
Author(s) -
Pastore Raymond D.,
Chadburn Amy,
Kripas Christopher,
Schattner Elaine J.
Publication year - 2000
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.2000.02478.x
Subject(s) - primary effusion lymphoma , medicine , ganciclovir , fulminant , lymphoma , ascites , malignancy , chemotherapy , pathology , sarcoma , effusion , dermatology , immunology , virus , surgery , human cytomegalovirus
Haemophagocytic syndrome (HPS) is a fulminant, often fatal, systemic illness that occurs in association with infection and malignancy. We provide the first report of HPS that heralded a primary effusion lymphoma (PEL), a rare neoplasm linked to Kaposi's sarcoma‐associated herpesvirus. The patient was a 38‐year‐old man with acquired immunodeficiency syndrome who presented with fever, sweats, lymphadenopathy, splenomegaly and refractory anaemia. Examination of the spleen demonstrated haemophagocytosis; analysis of ascites revealed PEL. Treatment with chemotherapy and ganciclovir resulted in complete remission of both conditions. This case illustrates the diagnostic challenges posed by HPS and supports the trial of antiviral agents in combination with chemotherapy in patients with PEL.