Novel association of haemophagocytic syndrome with Kaposi's sarcoma-associated herpesvirus-related primary effusion lymphoma

Br J Haematol. 2000 Dec;111(4):1112-5. doi: 10.1046/j.1365-2141.2000.02478.x.


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.

Publication types

  • Case Reports

MeSH terms

  • Acquired Immunodeficiency Syndrome / complications*
  • Acquired Immunodeficiency Syndrome / drug therapy
  • Adult
  • Anemia, Refractory / complications*
  • Anemia, Refractory / drug therapy
  • Anemia, Refractory / virology
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Antiviral Agents / therapeutic use
  • Cyclophosphamide / therapeutic use
  • Ganciclovir / therapeutic use
  • Herpesvirus 8, Human
  • Histiocytosis, Non-Langerhans-Cell / complications*
  • Histiocytosis, Non-Langerhans-Cell / drug therapy
  • Humans
  • Lung Neoplasms / complications*
  • Lung Neoplasms / drug therapy
  • Lung Neoplasms / virology
  • Lymphoma, Large B-Cell, Diffuse / complications*
  • Lymphoma, Large B-Cell, Diffuse / drug therapy
  • Lymphoma, Large B-Cell, Diffuse / virology
  • Male
  • Methylprednisolone / therapeutic use
  • Sarcoma, Kaposi / complications*
  • Sarcoma, Kaposi / drug therapy
  • Sarcoma, Kaposi / virology
  • Vincristine / therapeutic use


  • Antiviral Agents
  • Vincristine
  • Cyclophosphamide
  • Ganciclovir
  • Methylprednisolone