Hemophagocytic syndrome in Epstein-Barr virus-associated T-lymphoproliferative disorders: disease spectrum, pathogenesis, and management

Leukemia & Lymphoma
I J SuR L Chen

Abstract

The Epstein-Barr virus (EBV) has been shown to infect T lymphocytes and is associated with two recently recognized human T-lymphoproliferative disorders: childhood EBV-associated hemophagocytic syndrome (VAHS) representing a primary or active EBV infection of T cells in young children, and the EBV-containing T cell lymphoma in adults predominantly affecting the nose, skin and gastrointestinal tract. In both diseases, hemophagocytic syndrome (HS) accounts for the major cause of mortality. The patients developing HS share common clinicopathologic features such as fever, skin lesions, lung infiltrates, hepatosplenomegaly with jaundice, cytopenias, and coagulopathy. The liver, spleen, lymph nodes, and bone marrow usually show florid histiocytic proliferation with hemophagocytosis in addition to the proliferation of atypical T lymphocytes or immunoblasts. The HS in T cell lymphoma may develop simultaneously with initial lymphoma presentation, at tumor relapse, or even during remission. The cytokines, in particular tumor necrosis factor-alpha, released from the EBV-infected T lymphocytes are presumed to cause the histiocytic activation and the subsequent hemophagocytic process. Chemotherapy or antiviral agents fail to arrest the hemo...Continue Reading

References

Feb 1, 1992·American Journal of Clinical Pathology·F E CraigP M Banks
Feb 1, 1991·British Journal of Orthodontics·N R Smith, I R Reynolds
Jan 1, 1991·British Journal of Haematology·A KhwajaD C Linch
Nov 1, 1990·Archives of Disease in Childhood·P GoulderC Hatton
Oct 1, 1989·British Journal of Haematology·Y OyamaZ Ota
Jun 1, 1987·Immunological Reviews·S SandvigU Andersson
Sep 15, 1988·Annals of Internal Medicine·T J MyersS Schwartz
Oct 1, 1984·Archives of Internal Medicine·M WeintraubM Liron
Dec 15, 1984·Cancer·R J RisdallD H Gordon
Nov 1, 1983·The American Journal of Medicine·E S JaffeM Tsokos
Dec 1, 1952·Archives of Disease in Childhood·J W FARQUHAR, A E CLAIREAUX

❮ Previous
Next ❯

Citations

May 7, 2003·International Journal of Hematology·Tatsuharu OhnoUNKNOWN Kyoto University Hematology/Oncology Study Group
Jul 19, 2008·Virchows Archiv : an International Journal of Pathology·Sanghui ParkYoung-Hyeh Ko
Dec 7, 2002·Critical Reviews in Oncology/hematology·Hirokazu KaneganeGiovanna Tosato
Oct 5, 2002·Microbiology and Immunology·Hiroshi KimuraTsuneo Morishima
May 5, 1999·Therapeutic Apheresis : Official Journal of the International Society for Apheresis and the Japanese Society for Apheresis·Y MatsumotoY Sugiura
Oct 14, 2004·Journal of Virology·Yuling LiShannon Kenney
Aug 11, 2011·Blood·Michael B JordanKenneth L McClain
Jan 18, 2013·BMC Infectious Diseases·Valentina AnsuiniSusanna Esposito
Dec 15, 2005·Allergy, Asthma, and Clinical Immunology : Official Journal of the Canadian Society of Allergy and Clinical Immunology·Nada JabadoGenevieve de Saint Basile
Aug 7, 2007·International Journal of Hematology·Eiichi IshiiKeisei Kawa
Jul 10, 2012·Indian Journal of Pathology & Microbiology·Baijayantimala MishraSubhash Varma
Jun 30, 2014·Best Practice & Research. Clinical Rheumatology·Grant S Schulert, Alexei A Grom
Feb 15, 2013·European Journal of Pediatrics·Ourania AlexandropoulouKyriaki Karavanaki
Oct 5, 2001·Expert Opinion on Pharmacotherapy·S ImashukuS Hibi
Jan 2, 2007·Cancer Letters·Amit W Panwalkar, James O Armitage
Jan 21, 2014·The Journal of Dermatology·Sanghui Park, Young H Ko
Feb 4, 2010·Reviews in Medical Virology·Nadine Rouphael MaakarounHelmut Albrecht
Aug 9, 2002·Pathology, Research and Practice·Shih-Sung Chuang, Chin-Yang Li
Jul 12, 2005·Clinical Immunology : the Official Journal of the Clinical Immunology Society·Alexandra H FilipovichKathleen E Sullivan
Aug 26, 1999·The Korean Journal of Internal Medicine·J Y HanK S Lee
May 1, 1997·Critical Reviews in Oncology/hematology·I J Su, J Y Chen
Aug 16, 2000·Baillière's Best Practice & Research. Clinical Haematology·M TiabJ L Harousseau
Aug 16, 2000·Baillière's Best Practice & Research. Clinical Haematology·M Okano
Oct 27, 2010·International Journal of STD & AIDS·S J Flew, K W Radcliffe

❮ Previous
Next ❯

Related Concepts

Related Feeds

Antivirals

Antivirals are medications that are used specifically for treating viral infections. Discover the latest research on antivirals here.

Antivirals (ASM)

Antivirals are medications that are used specifically for treating viral infections. Discover the latest research on antivirals here.

B-Cell Lymphoma

B-cell lymphomas include lymphomas that affect B cells. This subtype of cancer accounts for over 80% of non-Hodgkin lymphomas in the US. Here is the latest research.

CNS Lymphoma

In CNS lymphoma, cancerous cells from lymph tissues or other parts of the body form tumors in the brain and/or spinal cord. Here is the latest research on this rare non-Hodgkin lymphoma.

Blood Clotting Disorders

Thrombophilia includes conditions with increased tendency for excessive blood clotting. Blood clotting occurs when the body has insufficient amounts of specialized proteins that make blood clot and stop bleeding. Here is the latest research on blood clotting disorders.