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Original Article

Treatment Strategies for Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis (EBV-HLH)

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Pages 37-49 | Received 12 Dec 1999, Published online: 01 Jul 2009

References

  • Imashuku S. Differential diagnosis of hemophagocytic syndrome: underlying disorders and selection of the most effective treatment. Int J Hematol 1997; 66: 135–151
  • Tsuda H. Hemophagocytic syndrome (HPS) in children and adults. Int J Hematol 1997; 65: 215–226
  • Jones J.F., Shurin S., Abramowsky C., Tubbs R.R., Sciotto C.G., Wahl R., Sands J., Gottman D., Katz B.Z., Sklar J. T-cell lymphomas containing Epstein-Barr viral DNA in patients with chronic Epstein-Barr infections. N Engl J Med 1988; 318: 733–741
  • Chen C.L., Sadler R.H., Walling D.M., Su I.J., Hsieh H.C., Raab-Traub N. Epstein-Barr virus (EBV) gene expression in EBV-positive peripheral T-cell lymphomas. J Virol 1993; 67: 6303–6308
  • Anagnostopoulos I., Hummel M., Kreschel C., Stein H. Morphology, immunophenotype and distribution of latently and/or productively Epstein-Barr virus-infected cells in acute infectious mononucleosis: implication for the interindividual infection route of Epstein-Barr virus. Blood 1995; 85: 744–750
  • Kawaguchi H., Miyashita T., Herbst H., Niedobitek G., Asada M., Tsuchida M., Hanada R., Kinoshita A., Sakurai M., Kobayashi N., Mizutani S. Epstein-Barr virus-infected T lymphocytes in Epstein-Barr virus-associated hemophagocytic syndrome. J Clin Invest 1993; 92: 1444–1450
  • Su I.J., Chen R.L., Lin D.T., Lin K.S., Chen C.C. Epstein-Barr virus infects T-lymphocytes in childhood EBV-associated hemophagocytic syndrome in Taiwan. Am J Pathol 1994; 144: 1219–1225
  • Wong K.F., Chan J.K., Lo E.S., Wong C.S. A study of the possible etiologic association of Epstein-Barr virus with reactive hemophagocytic syndrome in Hong Kong Chinese. Hum Pathol 1996; 27: 1239–1242
  • Dreyfus D.H., Kelleher C.A., Jones J.F., Gelfand E.W. Epstein-Barr virus infection of T cells: Implications for altered T-lymphocyte activation, repertoire development and autoimmunity. Immunol Reviews 1996; 152: 89–110
  • Imashuku S., Hibi S., Todo S. Hemophagocytic lymphohistiocytosis in infancy and childhood. J Pediatr 1997; 130: 352–357
  • Henter J-I., Elinder G., Ost A. Diagnostic guidelines for hemophagocytic lymphohistiocytosis. Semin Oncol 1991; 18: 29–33, the HLH Study Group of the Histiocyte Society.
  • Sumaya C.V., Ench Y. Epstein-Barr virus infectious mononucleosis in children. II. Heterophil antibody and viral-specific responses. Pediatrics 1985; 75: 1011–1019
  • Raab-Traub N., Flynn K. The structure of the termini of the Epstein-Barr virus as a marker of clonal cellular proliferation. Cell 1986; 47: 883–889
  • Imashuku S., Hibi S., Tabata Y., Sako M, Sekine Y., Hirayama K., Sakazaki H., Maeda N., Kito H., Shichino H., Mugishima H. Biomarker and morphological characteristics of Epstein-Barr virus-related hemophagocytic lymphohistiocytosis. Med Pediatr Oncol 1998; 31: 131–137
  • Mroczek E.C., Weisenburger D.D., Grierson H.L., Markin R., Purtilo D.T. Fatal infectious mononucleosis and virus-associated hemophagocytic syndrome. Arch Pathol Lab Med 1987; 111: 530–535
  • Wilson E.R., Malluh A., Stagno S., Crist W.M. Fatal Epstein-Barr virus-associated hemophagocytic syndrome. J Pediatr 1981; 98: 260–262
  • Imashuku S., Hibi S., Ohara T., Iwai A., Sako M., Kato M., Arakawa H., Sotomatsu M., Kataoka S., Asami K., Hasegawa D., Kosaka Y., Sano K., Igarashi N., Maruhashi K., Ichimi R., Kawasaki H., Maeda N., Tanizawa A., Arai K., Abe T., Hisakawa H., Miyashita H, Henter J-I. Effective control of Epstein-Barr virus-related hemophagocytic lymphohistiocytosis with immunochemotherapy. Blood 1999; 93: 1869–1874
  • Gaillard R, Mechinaud-Lacroix F, Papin S., Moreau A., Mollat C., Fiche M., Peltier S., De Faucal P.J., Rousselet M.C., Praloran V., Harousseau J.L. Primary Epstein-Barr virus infection with clonal T-cell lymphoproliferation. Am J Clin Pathol 1992; 98: 324–333
  • Cheng A-L., Su I-J., Chen Y-C, Uen W-C, Wang C-H. Characteristic clinicopathologic features of Epstein-Barr virus associated peripheral T cell lymphoma. Cancer 1993; 72: 909–916
  • Yao M., Cheng A-L., Su I-J., Lin M-T, Uen W-C, Tien H-E, Wang C.H., Chen Y.C. Clinicopathological spectrum of haemophagocytic syndrome in Epstein-Barr virus associated T cell lymphoma. Br J Haematol 1994; 87: 535–543
  • Schwartzmann F., von Baehr R., Jager M., Prang N., Bohm S., Reischl U., Wolf H., Bieger W.P. A case of severe chronic active infection with Epstein-Barr virus: Immunologic deficiencies associated with a lytic virus strain. Clin Infect Dis 1999; 29: 626–631
  • Ishihara S., Okada S., Wakiguchi H., Kurashige T, Hirai K., Kawa Ha K. Clonal lymphoproliferation following chronic active Epstein-Barr virus infection and hypersensitivity to mosquito bites. Am J Hematol 1997; 54: 276–281
  • Kanegane H., Wado T, Nunogami K., Seki H., Taniguchi N., Tosato G. Chronic persistent Epstein-Barr virus infection of natural killer cells and B cells associated with granular lymphocytes expansion. Br J Haematol 1996; 95: 116–122
  • Boyd A.W., Ellis D.W., Kannourakis G., Begley C.G., Mackay I.R., Burns G.F. Activated killer cell lymphoma: An erythrophagocytic syndrome simulating histiocytic medullary reticulosis. Pathology 1988; 20: 265–270
  • Okuda T., Sakamoto S., Deguchi T, Misawa S., Kashima K., Yoshihara T., Ikushima S., Hibi S., Imashuku S. Hemophagocytic syndrome associated with aggressive natural killer cell leukemia. Am J Hematol 1991; 38: 321–323
  • Chan J.K., Ng C.S. Malignant lymphoma, natural killer cells and hemophagocytic syndrome. Pathology 1989; 21: 154–155
  • Tokura Y, Ishihara S., Ohshima K., Hidano A., Koide M., Seo N., Takigawa M. Severe mosquito bite hypersensitivity, natural killer cell leukaemia, latent or chronic active Epstein-Barr virus infection and hydroa vacciniforme-like eruption. Br J Dermatol 1998; 138: 905–906
  • Mizuki M., Ueda S., Tagawa S., Shibayama H., Nishimori Y., Shibano M., Asada H., Tanaka M., Nagata S., Koudera U., Suzuki K., Machii T, Ohsawa M., Aozasa K., Kitani T, Kanakura Y. Natural killer cell-derived large granular lymphocyte lymphoma of lung developed in a patient with hypersensitivity to mosquito bites and reactivated Epstein-Barr virus infection. Am J Hematol 1998; 59: 309–315
  • Mizuno S., Akashi K., Ohshima K., Iwasaki H., Miyamoto T, Uchida N., Shibuya T, Harada M., Kikuchi M., Niho Y. Interferon-γ prevents apoptosis in Epstein-Barr virus-infected natural killer cell leukemia in an autocrine fashion. Blood 1999; 93: 3494–3504
  • Larochelle B., Flamand L., Gourde P., Beauchamp D., Gosselin J. Epstein-Barr virus infects and induces apoptosis in human neutrophils. Blood 1998; 92: 291–299
  • Cohen J.I. The biology of Epstein-Barr virus: lessons learned from the virus and the host. Current Opinion in Immunology 1999; 11: 365–370
  • Fischer N., Kopper B., Graf N., Schlehofer J.R., Grasser F.A., Mueller-Lantzsch N. Functional analysis of different LMP1 proteins isolated from Epstein-Barr virus-positive carriers. Virus Res 1999; 60: 41–54
  • Xu J., Menezes J., Prasad U., Ahmad A. Elevated serum levels of transforming growth factor P 1 in Epstein-Barr virus-associated nasopharyngeal carcinoma patients. Int J Cancer 1999; 84: 396–399
  • Feng P., Chan S.H., Ooi E.E., Soo M.Y., Loh K.S., Wang D., Ren E.C., Hu H. Elevated blood levels of soluble tumor necrosis factor receptors in nasopharyngeal carcinoma: correlation with humoral immune response to lytic replication of Epstein-Barr virus. Int J Oncol 1999; 15: 167–172
  • MacKenzie J., Gray D., Pinto-Paes R., Barrezueta L.F.M., Armstrong A.A., Alexander F.A., McGeoch D.J., Jarrett R.F. Analysis of Epstein-Barr virus (EBV) nuclear antigen 1 subtypes in EBV-associated lymphomas from Brazil and the United Kingdom. J Gen Virol 1999; 80: 2741–2745
  • Al-Homsi A.S., Berger C., van Baarle D., Kersten M.J., Klein M.R., McQuain C., van Oers R., Knecht H. Molecular analysis of critical sequences within the EBNA2 type A gene from EBV isolates from patients with infectious mononucleosis, tonsillar hypertrophy, and HIV infection. Int J Mol Med 1998; 1: 983–987
  • Chu P.G., Chang K.L., Chen W.G., Chen Y.Y., Shibata D., Hayashi K., Bacchi C., Bacchi M., Weiss L.M. Epstein-Barr virus (EBV) nuclear antigen (EBNA)-4 mutation in EBV-associated malignancies in three different populations. Am J Pathol 1999; 155: 941–947
  • Hu L.F., Zabarovsky E.R., Chen R, Cao S.L., Ernberg I., Klein G., Winberg G. Isolation and sequence of the Epstein-Barr virus BNLF-1 gene (LMP1) from a Chinese nasopharyngeal carcinoma. J Gen Virol 1991; 72: 2399–2409
  • Liebowitz D. Epstein-Barr virus and a cellular signaling pathway in lymphomas from immunosuppressed patients. New Engl J Med 1998; 338: 1413–1421
  • Knecht H., Bachmann E., Brousset P., Rothenberger S., Einsele H., Lestou VS., Delsol G., Bachmann F., Ambros P.F., Odermatt B.F. Mutational hot spots within the carboxy terminal region of the LMP1 oncogene of Epstein-Barr virus are frequent in lymphoproliferative disorders. Oncogene 1995; 10: 523–528
  • Kingma D.W., Weiss W.B., Jaffe E.S., Kumar S., Frekko K., Raffeld M. Epstein-Barr virus latent membrane protein-1 oncogene deletions: correlations with malignancy in Epstein-Barr virus-associated lymphoproliferative disorders and malignant lymphomas. Blood 1996; 88: 242–251
  • Tabata Y., Hibi S., Teramura T., Kuriyama K., Yagi T., Todo S., Sawada T., Imashuku S. Molecular analysis of latent membrane protein 1 in patients with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in Japan. Leuk Lymphoma 2000, in press
  • Kimura H., Morita M., Yabuta Y, Kuzushima K., Kato K., Kojima S., Matsuyama T., Morishima T. Quantitative analysis of Epstein-Barr virus load by using a real-time PCR assay. J Clin Microbiol 1999; 37: 132–136
  • Kanegane H., Wakiguchi H., Kanegane C., Kurashige T, Miyawaki T, Tosato G. Increased cell-free viral DNA in fatal cases of chronic active Epstein-Barr virus infection. Clin Infect Dis 1999; 28: 906–909
  • Lo Y.M.D., Chan L.Y.S., Lo K-W., Leung S.-E, Zhang J., Chan A.T.C., Lee J.K.C., Hjelm N.M., Johnson P.J., Huang D.P. Quantitative analysis of cell-free Epstein-Barr virus DNA in plasma of patients with nasopharyngeal carcinoma. Cancer Res 1999; 59: 1188–1191
  • Lo K.W., Lo Y.M., Leung S.F., Tsang Y.S., Chan L.Y., Johnson P.J., Hjelm N.M., Lee J.C., Huang D.P. Analysis of cell-free Epstein-Barr virus-associated RNA in the plasma of patients with nasopharyngeal carcinoma. Clin Chemistry 1999; 45: 1292–1294
  • Esumi N., Ikushima S., Hibi S., Todo S., Imashuku S. High serum ferritin level as a marker of malignant histiocytosis and virus-associated hemophagocytic syndrome. Cancer 1988; 61: 2071–2076
  • Hibi S., Ikushima S., Fujiwara F., Hashida T., Tsunamoto K., Todo S., Imashuku S. Serum and urine beta-2-microglobulin in hemophagocytic syndrome. Cancer 1995; 75: 1700–1705
  • Imashuku S. Treatment of Epstein-Barr virus (EBV)-associated hemophagocytic syndrome -how to apply immunochemotherapy-. Jpn Med J 1998; 3885, 37-45 (in Japanese)
  • Imashuku S., Hibi S., Kuriyama K., Tabata Y., Hashida T, Iwai A., Kato M., Yamashita N., Oda M., Uchida M., Kinugawa N., Sawada M., Konno M. Management of severe neutropenia with cyclosporin during initial treatment of Epstein-Barr virus-related hemophagocytic lymphohistiocytosis (EBV-HLH). Leuk Lymphoma 2000, in press
  • Imashuku S., Hibi S., Sako M., Ishida Y, Mugishima H., Chen J., Tsunematsu Y. Soluble interleukin-2 receptor: a useful prognostic factor for patients with hemophagocytic lymphohistiocytosis. Blood 1995; 86: 4706–4707
  • Imashuku S., Hibi S., Fujiwara F., Ikushima S., Todo S. Haemophagocytic lymphohistiocytosis, infer-feron-gamma-naemia and Epstein-Barr virus involvement. Br J Haematol 1994; 88: 656–658
  • Imashuku S., Hibi S., Tabata Y, Itoh E., Hashida T., Tsunamoto K., Ishimoto K., Kawano F. Outcome of clonal hemophagocytic lymphohistiocytosis: analysis of 32 cases. Leuk Lymphoma 2000, in press)
  • Esumi N., Hashida T, Matsumura T, Takeuchi Y, Arakawa S., Imashuku S. Malignant histiocytosis in childhood. Clinical features and therapeutic results by combination chemotherapy. Am J Pediatr Hematol Oncol 1986; 8: 300–307
  • Nomura S., Koshikawa K., Hamamoto K., Okubo S., Yasunaga K. Steroid and gamma globulin therapy against virus-associated hemophagocytic syndrome. Rinsho Ketsueki 1992; 33: 1242–1247
  • Imashuku S. Case records of pediatric hemophagocytic syndrome. Jpn Med J 1994; 3676, 43-52 (in Japanese)
  • Gill D.S., Spencer A., Cobcroft R.G. High-dose gamma globulin therapy in the reactive hemophagocytic syndrome. Br J Haematol 1994; 88: 204–206
  • Goulder P., Seward D., Hatton C. Intravenous immunoglobulin in virus-associated hemophagocytic syndrome. Arch Dis Child 1990; 65: 1275–1277
  • Freeman B., Rathore M.H., Salman E., Joyce M.J., Pitel P. Intravenously administered immune globulin for the treatment of infection-associated hemophagocytic syndrome. J Pediatr 1993; 123: 479–481
  • Fort D.W., Buchanan G.R. Treatment of infection-associated hemophagocytic syndrome with immune globulin. J Pediatr 1994; 124: 332
  • Chen R.L., Lin K.H., Lin D-T., Su I.J., Huang L.M., Lee P.I., Hseih K.H., Lin K.S., Lee C.Y. Immunomodulation treatment for childhood virus-associated haemophagocytic lymphohistiocytosis. Br J Haematol 1995; 89: 282–290
  • Nagasawa M., Okawa H., Yata J. Deleterious effects of high dose γ-globulin therapy on patients with hemophagocytic syndrome. Int J Hematol 1994; 60: 91–93
  • Osugi Y., Hara J., Tagawa S., Takai K., Hosoi G., Matsuda Y., Ohta H., Fujisaki H., Kobayashi M., Sakata N., Kawa-Ha K., Okada S., Tawa K. Cytokine production regulating Th1 and Th2 cytokines in hemophagocytic lymphohistiocytosis. Blood 1997; 89: 4100–4103
  • Ladisch S., Ho W., Matheson D., Pilkington R., Hartman G. Immunologic and clinical effects of repeated blood exchange in familial erythrophagocytic lymphohistiocytosis. Blood 1982).; 60: 814–821
  • Kizaki Z., Fukumochi H., Sawai T., Ishimura K., Esumi N., Nagai T., Todo S., Imashuku S. Exchange transfusion and combination chemotherapy in the treatment of 4 cases of childhood malignant histiocytosis. Rinsho Ketsueki 1987; 28: 845–851
  • Matsumoto Y, Naniwa D., Banno S., Sugiura Y. The efficacy of therapeutic plasmapheresis for the treatment of fatal hemophagocytic syndrome: two case reports. Ther Apher 1998; 2: 300–304
  • Stegmayr B. Apheresis of plasma compounds as a therapeutic principle in severe sepsis and multiorgan dysfunction syndrome. Clin Chem Lab Med 1999; 37: 327–332
  • Clark W.F., Rock G.A., Buskard N., Shumak K.H., LeBlond P., Anderson D., Sutton D.M. Therapeutic plasma exchange: an update from the Canadian Apheresis Group. Ann Intern Med 1999).; 131: 453–462
  • Novitzky N., Jacobs P., Rosenstrauch W. The treatment of thrombotic thrombocytopenic purpura: plasma infusion or exchange?. Br J Haematol 1994; 87: 317–320
  • Oyama Y, Amano T., Hirakawa S., Hironaka K., Suzuki S., Ota Z. Haemophagocytic syndrome treated with cyclosporin A: T cell disorder?. Br J Haematol 1989; 73: 276–278
  • Brinkman K., van Dongen J.J., van Lorn K., Groeneveld K., Misere J.F., van der Heul C. Induction of clinical remission in T-large granular lymphocyte leukemia with cyclosporin A, monitored by use of immunophenotyping with V beta antibodies. Leukemia 1998; 12: 150–154
  • Perel Y, Alos N., Ansoborlo S., Carrere A., Guillard J.M. Dramatic efficacy of antithymocyte globulins in childhood EBV-associated haemophagocytic syndrome. Acta Paediatr 1997; 86: 911
  • Okamoto M., Ono M., Baba M. Potent inhibition of HIV type 1 replication by an anti-inflammatory alkaloid, cepharanthine, in chronically infected monocytic cells. AIDS Res Hum Retroviruses 1998; 14: 1239–1245
  • Sundaram S., Gikakis N., Hack C.E., Niewiarowski S., Edmunds L.H., Koneti-Rao A., Sun L., Cooper S.L., Colman R.W. Nafamostat mesilate, a broad spectrum protease inhibitor, modulates platelet, neutrophil and contact activation in simulated extracorporeal circulation. Thromb Haemostasis 1996; 75: 76–82
  • Senda S., Fujiyama Y, Bamba T., Hosoda S. Treatment of ulcerative colitis with camostat mesilate, a serine protease inhibitor. Intern Med 1993; 32: 350–354
  • Maeda N., Kitoh H., Imashuku S. Epstein-Barr virus-associated hemophagocytic syndrome (EB-VAHS) with neurological symptoms and transient organic mental disorder. Jpn J Pediatr Hematol 1997; 11: 450–454
  • Iwai A., Iwai T., Suzuya H., Endo S., Nakagawa Y, Watanabe T, Kawano Y, Imashuku S. Development of a clonal and Epstein-Barr virus (EBV)-positive CNS tumor at recurrence in a case of hemophagocytic lymphohistiocytosis. Int J Pediatr Hematol Oncol 1999; 6: 183–188
  • Yamashita S., Murakami C., Izumi Y, Sawada H., Yamazaki Y, Yokota T.A., Matsumoto N., Matsukura S. Severe chronic active Epstein-Barr virus infection accompanied by virus-associated hemophagocytic syndrome, cerebellar ataxia and encephalitis. Psychiatry Clin Neurosci 1998; 52: 449–452
  • Morita M., Tsuge I., Matsuoka H., Ito Y, Itosu T, Yamamoto M, Morishima T. Calcification in the basal ganglia with chronic active Epstein-Barr virus infection. Neurology 1998; 50: 1485–1488
  • Ohga S., Takada H., Honda K., Inamura T., Gondo K., Ohshima K., Yamamoto M., Hara T. Central nervous system T-cell lymphoproliferative disorder in a patient with chronic active Epstein-Barr virus infection. J Pediatr Hematol Oncol 1999; 21: 42–46
  • Kinoshita Y, Hashida T, Otsuka T., Hibi S., Sawada T., Imashuku S. A case of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis that died as a result of sepsis of Candida albicans at 1 month after initial treatment. Jpn J Pediatr Hematol 1999; 13: 46–50
  • Connolly A.A.P., Rowe-Jones J., Leighton S.E.J., Ball S.E., Davies E.G., Moore-Gillon V. Pseudomonal supraglottitis occurring in a patient with profound neutropenia secondary to virus-associated haemophagocytic syndrome. J Laryngol Otol 1992; 106: 739–740
  • Sood R., Stewart C.C., Apian P.D., Murai H., Ward P., Barcos M., Baer M.R. Neutropenia associated with T-cell large granular lymphocyte leukemia: Long-term response to cyclosporine therapy despite persistence of abnormal cells. Blood 1998; 91: 3372–3378
  • Herrod H.G., Wang W.C., Sullivan J.L. Chronic T-cell lymphocytosis with neutropenia. Its association with Epstein-Barr virus infection. Am J Dis Child 1985; 139: 405–407
  • Garipidou V., Tsatalas C., Sinacos Z. Severe neutropenia in a patient with large granular lymphocytosis: prolonged successful control with cyclosporin A. Haematologica 1991; 76: 424–425
  • Wulff H.R. Acute agranulocytosis following infectious mononucleosis. Report of a case. Scand J Haematol 1976; 2: 179–182
  • Neel E.U. Infectious mononucleosis. Death due to an agranulocytosis and pneumonia. JAMA 1976; 236: 1493–1494
  • Imashuku S., Hibi S., Morinaga S., Takagi K., Chen J., Mugishima H., Ishii T., Sako M., Arakawa H., Kato M. Haemophagocytic lymphohistiocytosis in association with granular lymphocyte proliferative disorders in early childhood: characteristic bone marrow morphology. Br J Haematol 1997; 96: 708–714
  • Tsuda H., Shirono K. Successful treatment of virus-associated haemophagocytic syndrome in adults by cyclosporin A supported by granulocyte colony-stimulating factor. Br J Haematol 1996; 93: 572–575
  • Quesnel B., Catteau B., Aznar V., Bauters F., Fenaux P. Successful treatment of juvenile rheumatoid arthritis associated haemophagocytic syndrome by cyclosporin A with transient exacerbation by conventional-dose G-CSF. Br J Haematol 1997; 97: 504–510
  • Kaneko Y., Maseki N., Sakurai M., Ido M., Tsunematsu Y, Mizutani S., Hattori T., Shimizu H., Eguchi H., Oka T., Miyake M., Horikoshi Y, Suchi T. Clonal and non-clonal karyotypically abnormal cells in haemophagocytic lymphohistiocytosis. Br J Haematol 1995; 90: 48–55
  • Dolezal M.V., Kamel O.W., van de Rijn M., Cleary MX., Sibley R.K., Warnke R.A. Virus-associated hemophagocytic syndrome characterized by clonal Epstein-Barr virus genome. Am J Clin Pathol 1995; 103: 189–194
  • Chen J.S., Tzeng C.C., Tsao C.J., Su W.C., Chen T.Y., Jung Y.C., Su I.J. Clonal karyotype abnormalities in EBV-associated hemophagocytic syndrome. Haematologica 1997; 82: 572–576
  • Bird G., Peel D., McCarthy K., Williams H. Epstein-Barr virus induced virus-associated hemophagocytic syndrome and monoclonal TCR-beta rearrangement: A case report. Hematol Oncol 1997; 15: 47–52
  • Henter J.I., Arico M., Egeler R.M., Elinder G., Favara B.E., Filipovich A.H., Gadner H., Imashuku S., Janka-Schaub G., Komp D., Ladisch S., Webb D. HLH-94: a treatment protocol for hemophagocytic lymphohistiocytosis. Med Pediatr Oncol 1997; 28: 342–347
  • Imashuku S., Naya M., Yamori M., Nakabayashi Y, Hojo M., Kihara A., Tabata Y, Akioka S., Hibi S., Todo S. Chemotherapy and allogeneic bone marrow transplantation for T-cell/NK-cell type EBV-related lymphoprolif-erative disorders. Jpn J Pediatr Oncol 1996; 33, 369 (abstr, in Japanese)
  • Inoue M., Nomura K., Yasui M., Okamura T., Sakata N., Yagi K., Kawa K. (1978) 1998 Treatment strategy for hemophagocytic syndrome in childhood-application of stem cell transplantation. Meeting Program of Fourteenth Annual Meeting of the Histiocyte Society, p40. 1998
  • Canellos G.P., Anderson J.R., Propert K.J., Nissen N., Cooper M.R., Henderson E.S., Green M.R., Gottlieb A., Peterson B.A. Chemotherapy of advanced Hodgkin's disease with MOPP, ABVD, or MOPP alternating with ABVD. N Engl J Med 1992; 327: 1478–1484
  • Obama K., Tara M., Niina K. L-asparaginase induced complete remission in Epstein-Barr virus positive, multidrug resistant, cutaneous T-cell lymphoma. Int J Hematol 1999; 69: 260–262
  • Fischer A., Cerf-Bensussan N., Blanche S., Le Deist F, Bremard-Oury C., Leverger G., Schaison G., Durandy A., Griscelli C. Allogeneic bone marrow transplantation for erythrophagocytic lymphohistiocytosis. J Pediatr 1986; 108: 267–270
  • Bolme P., Henter J.I., Winiarski J, Elinder G., Ljungman P., Lonnerholm G., Ringden O. Allogeneic bone marrow transplantation for hemophagocytic lymphohistiocytosis in Sweden. Bone Marrow Transplant 1995; IS: 331–335
  • Collins P., Watts M., Brocklesby M., Gerritsen B., Veys P. Successful engraftment of haploidentical stem cell transplant for familial haemophagocytic lymphohistiocytosis using both bone marrow and peripheral blood stem cells. Br J Haematol 1997; 96: 644–646
  • Jabado N., de Graeff-Meeder E.R., Cavazzana-Calvo M., Haddad E., Le Deist F., Benkerrou M., Dufourcq R., Caillat S., Blanche S., Fischer A. Treatment of Familial Hemophagocytic Lymphohistiocytosis with bone marrow transplantation from HLA genetically nonidentical donors. Blood 1997; 90: 4743–4748
  • Gratama J.W., Oosterveer M.A., Zwaan F.E., Lepoutre J., Klein G., Ernberg I. Eradication of Epstein-Barr virus by allogeneic bone marrow transplantation: Implications for sites of viral latency. Proc Natl Acad Sci USA 1988; 85: 8693–8696
  • Teshima T., Miyaji R., Fukuda M., Ohshima K. Bone-marrow transplantation for Epstein-Barr-virus-associated natural killer cell-large granular lymphocyte leukaemia. Lancet 1996; 347: 1124
  • Imashuku S., Naya M., Yamori M., Nakabayashi Y, Hojo M., Kihara A., Tabata Y, Akioka C., Hibi S., Todo S. Bone marrow transplantation for Epstein-Barr virus-related clonal T cell proliferation associated with hemophagocytosis. Bone Marrow Transplant 1997; 19: 1059–1060
  • Imashuku S., Hibi S., Todo S., Sako M., Inoue M., Kawa K., Koike K., Iwai A., Tsuchiya S., Akiyama Y, Kotani T., Kawamura Y, Hirosawa M., Hasegawa D., Kosaka Y, Yamaguchi H., Ishii E., Kato K., Ishii M., Kigasawa H. Allogeneic hematopoietic stem cell transplantation for patients with hemophagocytic syndrome (HPS) in Japan. Bone Marrow Transplant 1999; 23: 569–572
  • Chan K.W., Mullen C.A., Korbling M. Allogeneic peripheral blood stem cell transplantation for active hemophagocytic lymphohistiocytosis. Bone Marrow transplant 1998; 22: 301–302
  • Ohga S., Nomura A., Kai T., Matsuzaki A., Inaba S., Suda M., Ueda K. Prolonged resolution of hemophagocytic lymphohistiocytosis following myeloablasive chemotherapy and subsequent autologous peripheral blood stem cell transplantation. Bone Marrow Transplant 1997; 19: 633–635
  • Hasegawa D., Sano K., Kosaka Y, Hayakawa A., Nakamura H. A case of hemophagocytic lymphohistiocytosis with prolonged remission after syngeneic bone marrow transplantation. Bone Marrow Transplant 1999; 2A: 425–427
  • Anderlini P., Riggs S.A., Korbling M., Champlin R. Syngeneic blood stem cell transplantation for infectious mononucleosis-related aplastic anaemia. Br J Haematol 1999; 106: 159–161
  • Williams L.L., Rooney CM., Conley M.E., Brenner M.K., Krance R.A., Heslop H.E. Correction of Duncan's syndrome by allogeneic bone marrow transplantation. Lancet 1993; 342: 587–588
  • Pracher E., Grumayer-Panzer E.R., Zoubek A., Peters C., Gadner H. Bone marrow transplantation during fulminant EBV-infection in Duncan's syndrome. Lancet 1993; 342: 1362
  • Vowels M.R., Tang R.L., Berdoukas V., Ford D., Thierry D., Purtilo D., Gluckman E. Brief report: Correction of X-linked lymphoproliferative disease by transplantation of cord-blood stem cells. N Engl J Med 1993; 329: 1623–1625
  • Natkunam Y, Smoller B.R., Zehnder J.L., Dorfman R.F., Warnke R.A. Aggressive cutaneous NK and NK-like T-cell lymphomas: clinicopathologic, immunohistochemical, and molecular analyses of 12 cases. Am. J. Surg. Pathol 1999; 23: 571–581
  • Drenou B., Amiot L., Lamy T., Le Prise P.Y., Fauchet R. Multidrug resistance in aggressive lymphoproliferative disorders of T and natural-killer origin. Leuk Lymphoma 1998; 30: 381–387
  • Ohnuma K., Toyoda Y., Nishihira H., Iguchi A., Honda K., Nagao T., Kigasawa H., Tanaka Y, Kikuta H. Aggressive natural killer(NK) cell lymphoma: Report of a pediatric case and review of the literature. Leuk Lymphoma 1997; 25: 387–392
  • Mukai H.Y, Kojima H., Suzukawa K., Hori M., Komeno T., Hasegawa Y, Ninomiya H., Mori N., Nagasawa T. High-dose chemotherapy with peripheral blood stem cell rescue in blastoid natural killer cell lymphoma. Leuk Lymphoma 1999; 32: 583–588
  • Liang R., Chen E, Lee C.K., Kwong Y.L., Chim C.S., Yau C.C., Chiu E. Autologous bone marrow transplantation for primary nasal T/NK cell lymphoma. Bone Marrow Transplant 1997; 19: 91–93
  • Imashuku S., Obayashi M., Hosoi G., Sako M., Chen J., Mugishima H., Tsunamoto K., Hibi S., Todo S. Splenectomy in hemophagocytic lymphohistiocytosis: report of histopathologic changes with CD19+ B-cell depletion and therapeutic results. Br J Haematol 2000, In press
  • Demeter J. Persistent lymphocytosis of natural killer cells after splenectomy. Br J Haematol 1995; 91: 253–254
  • Loughran T.P., Starkebaum G., Clark E., Wallace P., Kadin M.E. Evaluation of splenectomy in large granular lymphocyte leukaemia. Br J Haematol 1987; 67: 135–140
  • Syruckova Z., Stary J., Sedlacek P., Smisek P., Vavrinec J., Komrska V., Roubalova K., Vandasova J., Sintakova B., Houskova J., Hassan M. Infection-associated hemophagocytic syndrome complicated by infectious lymphoproliferation: a case report. Pediatr Hematol Oncol 1996; 13: 143–150
  • Papadopoulos E.B., Ladanyi M., Emanuel D., Mackinnon S., Boulad R, Carabasi M.H., Castro-Malaspina H., Childs B.H., Gillio A.P., Small T.N., Young J.W., Kernan N.A., O'Reilly R.J. Infusions of donor leukocytes to treat Epstein-Barr virus-associated lymphoproliferative disorders after allogeneic bone marrow transplantation. N Engl J Med 1994; 330: 1185–1191
  • Moss D.J., Khanna R., Sherritt M., Elliott S.L., Burrows S.R. Developing immunotherapeutic strategies for the control of Epstein-Barr virus-associated malignancies. J AIDS 1999; 21: S80–S83
  • Chiang A.K., Tao Q., Srivastava G., Ho RC. Nasal NK- and T-cell lymphomas share the same type of Epstein-Barr virus latency as nasopharyngeal carcinoma and Hodgkin's disease. Int J Cancer 1996; 68: 285–290
  • Roskrow M.A., Suzuki N., Gan Y.J., Sixbey J.W., Ng C.Y., Kimbrough S., Hudson M., Brenner M.K., Heslop H.E., Rooney C.M. Epstein-Barr virus (EBV)-specific cytotoxic T lymphocytes for the treatment of patients with EBV-positive relapsed Hodgkin's disease. Blood 1998; 91: 2925–2934
  • Seemayer T.A., Grierson H., Pirruccello S.J., Gross T.G., Weisenburger D.D., Davis J., Spiegel K., Brichacek B., Sumegi J. X-linked lymphoproliferative disease. AJDC 1993; 147: 1242–1245
  • Sayos J., Wu C., Morra M., Wang N., Zhang X., Allen D., van Schaik S., Notarangelo L., Geha R., Roncarolo M.G., Oettgen H., De Vries J.E., Aversa G., Terhorst C. The X-linked lymphoproliferative disease gene product SAP regulates signals induced through the co-receptor SLAM. Nature 1998; 395: 462–469
  • Coffey A.J., Brooksbank R.A., Brandau O., Oohashi X, Howell G.R., Bye J.M., Cahn A.P., Durham J., Heath P., Wray P., Pavitt R., Wilkinson J., Leversha M., Huckle E., Shaw-Smith C.J., Dunham A., Rhodes S., Schuster V., Porta G., Yin L., Serafini P., Sylla B., Zollo M., Franco B., Bolino A, Seri M, Lanyi A, Davis JR, Webster D, Harris A, Lenoir G, de St Basile G, Jones A, Behloradsky BH, Achatz H, Murken J, Fassler R, Sumegi J, Romeo G, Vaudin M, Ross MT, Meindl A, Bentley D.R. Host response to EBV infection in X-linked lymphoproliferative disease results from mutations in an SH2-domain encoding gene. Nature Genetics 1998; 20: 129–135
  • Nichols K.E., Harkin D.P., Levitz S., Krainer M., Kolquist K.A., Genovese C., Bernard A., Ferguson M., Zuo L., Snyder E., Buckler A.J., Wise C., Ashley J., Lovett M., Valentine M.B., Look A.T., Gerald W., Housman D.E., Haber D.A. Inactivating mutations in an SH2 domain-encoding gene in X-linked lymphoproliferative syndrome. Proc Natl Acad Sci USA 1998; 95: 13765–13770
  • Nichols K., Arico M., Danesino C., Imashuku S., Haber D. (1978) 1999 Evaluation of DSHP, the gene mutated in X-linked lymphoproliferative disease (XLP), in sporadic EBV-associated hemophagocytic (HLH) or lymphoproliferative (LPD) syndromes. Meeting Program of Fifteenth Annual Meeting of the Histiocyte Society, p40. 1999
  • Ohadi M., Lalloz M.R., Sham P., Zhao J., Dearlove A.M., Shiach C., Kinsey S., Rhodes M., Layton D.M. Localization of a gene for familial hemophagocytic lymphohistiocytosis at chromosome 9q21.3-22 by homozygosity mapping. Am J Hum Genet 1999; 64: 165–171
  • Dufourcq-Lagelouse R., Jabado N., Le Deist F, Stephan J.L., Souillet G., Bruin M., Vilmer E., Schneider M., Janka G., Fischer A., de Saint Basile G. Linkage of familial hemophagocytic lymphohistiocytosis to 10q21-22 and evidence for heterogeneity. Am J Hum Genet 1999; 64: 172–179
  • Stine K.C., Saylors R.L., Sawyer J.R., Becton D.L. Secondary acute myelogenous leukemia following safe exposure to etoposide. J Clin Oncol 1997; 15: 1583–1586
  • Takahashi T., Yagasaki R, Endo K., Murohashi I, Jinnai I, Bessho M, Hirashima K. Treatment of two cases of severe hemophagocytic syndrome. J Jpn Soc Reticuloen-dothel System 1997; 37, 105 (abstr, in Japanese)

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