| Title | Novel association of haemophagocytic syndrome with Kaposi's sarcoma-associated herpesvirus-related primary effusion lymphoma. |
| Publication Type | Journal Article |
| Year of Publication | 2000 |
| Authors | Pastore RD, Chadburn A, Kripas C, Schattner EJ |
| Journal | Br J Haematol |
| Volume | 111 |
| Issue | 4 |
| Pagination | 1112-5 |
| Date Published | 2000 Dec |
| ISSN | 0007-1048 |
| Keywords | Acquired Immunodeficiency Syndrome, Adult, Anemia, Refractory, Antineoplastic Combined Chemotherapy Protocols, Antiviral Agents, Cyclophosphamide, Ganciclovir, Herpesvirus 8, Human, Histiocytosis, Non-Langerhans-Cell, Humans, Lung Neoplasms, Lymphoma, Large B-Cell, Diffuse, Male, Methylprednisolone, Sarcoma, Kaposi, Vincristine |
| Abstract | 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. |
| DOI | 10.1046/j.1365-2141.2000.02478.x |
| Alternate Journal | Br J Haematol |
| PubMed ID | 11167749 |
Related Faculty:
Amy Chadburn, M.D.