摘要
Medical and Pediatric OncologyVolume 28, Issue 5 p. 342-347 HLH-94: A treatment protocol for hemophagocytic lymphohistiocytosis Jan-Inge Henter MD, PhD, Corresponding Author Jan-Inge Henter MD, PhD Department of Pediatrics, Karolinska Hospital, Stockholm, SwedenDepartment of Pediatrics, Karolinska Hospital, S-171 76 Stockholm, Sweden; fax +46-8 7293184, or the Histiocyte Society, 302 North Broadway, Pitman, NJ 08071, fax +1-609 589-6614, tel. +1-609 589-6606Search for more papers by this authorMaurizio Aricò MD, Maurizio Aricò MD Clinica Pediatrica, IRCCS Policlinico S. Matteo, Pavia, ItalySearch for more papers by this authorR. Maarten Egeler MD, PhD, R. Maarten Egeler MD, PhD Sophia Children's Hospital, Rotterdam, the NetherlandsSearch for more papers by this authorGöran Elinder MD, PhD, Göran Elinder MD, PhD Sachs' Children's Hospital, Stockholm, SwedenSearch for more papers by this authorBlaise E. Favara MD, Blaise E. Favara MD National Institutes of Health, Rocky Mountain Laboratory, Hamilton, MontanaSearch for more papers by this authorAlexandra H. Filipovich MD, Alexandra H. Filipovich MD Children's Hospital Medical Center, Cincinnati, OhioSearch for more papers by this authorHelmut Gadner MD, PhD, Helmut Gadner MD, PhD St. Anna Children's Hospital, Vienna, AustriaSearch for more papers by this authorShinsaku Imashuku MD, Shinsaku Imashuku MD Kyoto City Institute of Health and Environmental Sciences, JapanSearch for more papers by this authorGritta Janka-Schaub, Gritta Janka-Schaub Children's University Hospital, Hamburg, GermanySearch for more papers by this authorDiane Komp MD, Diane Komp MD Yale University School of Medicine, New Haven, ConnecticutSearch for more papers by this authorStephan Ladisch MD, Stephan Ladisch MD Center for Cancer and Transplantation Biology, Washington, DCSearch for more papers by this authorDavid Webb MD, David Webb MD Department of Hematology, Great Ormond Street Hospital, London, United KingdomSearch for more papers by this author Jan-Inge Henter MD, PhD, Corresponding Author Jan-Inge Henter MD, PhD Department of Pediatrics, Karolinska Hospital, Stockholm, SwedenDepartment of Pediatrics, Karolinska Hospital, S-171 76 Stockholm, Sweden; fax +46-8 7293184, or the Histiocyte Society, 302 North Broadway, Pitman, NJ 08071, fax +1-609 589-6614, tel. +1-609 589-6606Search for more papers by this authorMaurizio Aricò MD, Maurizio Aricò MD Clinica Pediatrica, IRCCS Policlinico S. Matteo, Pavia, ItalySearch for more papers by this authorR. Maarten Egeler MD, PhD, R. Maarten Egeler MD, PhD Sophia Children's Hospital, Rotterdam, the NetherlandsSearch for more papers by this authorGöran Elinder MD, PhD, Göran Elinder MD, PhD Sachs' Children's Hospital, Stockholm, SwedenSearch for more papers by this authorBlaise E. Favara MD, Blaise E. Favara MD National Institutes of Health, Rocky Mountain Laboratory, Hamilton, MontanaSearch for more papers by this authorAlexandra H. Filipovich MD, Alexandra H. Filipovich MD Children's Hospital Medical Center, Cincinnati, OhioSearch for more papers by this authorHelmut Gadner MD, PhD, Helmut Gadner MD, PhD St. Anna Children's Hospital, Vienna, AustriaSearch for more papers by this authorShinsaku Imashuku MD, Shinsaku Imashuku MD Kyoto City Institute of Health and Environmental Sciences, JapanSearch for more papers by this authorGritta Janka-Schaub, Gritta Janka-Schaub Children's University Hospital, Hamburg, GermanySearch for more papers by this authorDiane Komp MD, Diane Komp MD Yale University School of Medicine, New Haven, ConnecticutSearch for more papers by this authorStephan Ladisch MD, Stephan Ladisch MD Center for Cancer and Transplantation Biology, Washington, DCSearch for more papers by this authorDavid Webb MD, David Webb MD Department of Hematology, Great Ormond Street Hospital, London, United KingdomSearch for more papers by this author First published: 07 December 1998 https://doi.org/10.1002/(SICI)1096-911X(199705)28:5<342::AID-MPO3>3.0.CO;2-HCitations: 331AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL References 1 Henter J-I, Söder O, Öst A, Elinder G: Incidence and clinical features of familial hemophagocytic lymphohistiocytosis in Sweden. Acta Paediatr Scand 80: 428– 35, 1991. 2 The Writing Group of the Histiocyte Society. Histiocytosis syndromes in children. Lancet i: 208– 209, 1987. 3 Henter JI, Elinder G, Öst Å, and the FHL Study Group of the Histiocyte Society. Diagnostic guidelines for hemophagocytic lymphohistiocytosis. Semin Oncol 18: 29– 33, 1991. 4 Risdall RJ, McKenna RW, Nesbit ME, Krivit W, Balfour HH Jr, Simmons RL, Brunning RD: Virus–associated hemophagocytic syndrome. A benign histiocytic proliferation distinct from malignant histiocytosis. Cancer 44: 993– 1002, 1979. 5 Arico M, Janka G, Fischer A, Henter J-I, Blanche S, Elinder G, Martinetti M, Rusca MP, for the FHL Study Group of the Histiocyte Society. Hemophagocytic lymphohistiocytosis: Diagnosis, treatment, and prognostic factors. Report of 122 children from the international registry. Leukaemia, 10: 197– 203, 1996. 6 Imashuku S, Ikushima S, Hibi S, Todo S: Langerhans cell histiocytosis and hemophagocytic syndrome in Japan; epidemiological studies. Int J Pediatr Hematol/Oncol 1: 241– 246, 1994. 7 Janka GE: Familial hemophagocytic lymphohistiocytosis. Eur J Pediatr 140: 221– 230, 1983. 8 Perez N, Virelizier J, Arenzana-Seisdedos F, Fischer A, Criscelli C: Impaired natural killer cell activity in lymphohistiocytosis syndrome. J Pediatr 104: 569– 573, 1984. 9 Eife R, Janka G, Belohradsky B, Holtmann BH: Defective natural killer cell function and interferon production in familial hemophagocytic lymphohistiocytosis. Med Pediatr Oncol 14: 111, 1986. 10 Aricó M, Nespoli L, Maccario R, Montagna D, Bonetti F, Caselli D, Burgio GR: Natural cytotoxicity impairment in familial haemo-phagocytic lymphohistiocytosis. Arch Dis Child 63: 292– 296, 1988. 11 Kataoka Y, Todo S, Morioka Y, Sugie K, Nakamura Y, Yidoi J, Imashuku S: Impaired natural killer activity and expression of interleukin-2 receptor antigen in familial erythrophagocytic lymphohistiocytosis. Cancer 65: 1937– 1941, 1990. 12 Esumi N, Ikushima S, Todo S, Imashuku S: Hyperferritinemia in malignant histiocytosis, virus-associated hemophagocytic syndrome and familial hemophagocytic lymphohistiocytosis. Acta Paediatr Scand 78: 268– 270, 1989. 13 Henter J-I, Carlson LA, Nilsson-Ehle P, Soder O, Elinder G: Lipoprotein alterations and plasma lipoprotein lipase reduction in familial hemophagocytic lymphohistiocytosis. Acta Paediatr Scand 80: 675– 681, 1991. 14 Komp DM, McNamara J, Buckley P: Elevated soluble interleukin-2 receptor in childhood hemophagocytic histiocytic syndromes. Blood 73: 2128– 2132, 1989. 15 Howells DW, Strobel S, Smith I, Levinsky RJ, Hyland K: Central nervous system involvement in the erythrophagocytic disorders of infancy: The role of cerebrospinal fluid neopterins in their differential diagnosis and clinical management. Pediatr Res 28: 116– 119, 1990. 16 Henter J-I, Elinder G, Söder O, Hansson M, Andersson B, Anders-son U: Hypercytokinemia in familial hemophagocytic lymphohistiocytosis. Blood 78: 2918– 2922, 1991. 17 Imashuku S, Ikushima S, Esumi N, Todo S, Saito M: Serum levels of interferon-gamma, cytotoxic factor and soluble interleukin-2 receptor in childhood hemophagocytic syndrome. Leukemia Lymphoma 3: 287– 292, 1991. 18 Fujiwara F, Hibi S, Imashuku S: Hypercytokinemia in hemophagocytic syndrome. Amer J Pediatr Hematol Oncol 15: 92– 98, 1993. 19 Ladisch S, Jaffe ES: The histiocytosis. In: PA Pizzo, DG Poplack eds. Principles and Practice of Pediatric Oncology, pp 491– 504. Philadelphia: Lippincott, 1989. 20 Favara B: Hemophagocytic lymphohistiocytosis: A hemophagocytic syndrome. Semin Diagn Pathol 9: 63– 74, 1992. 21 Seemayer TA, Grierson H, Pirruccello SJ, Gross TG, Weisen–burger DD, Davis J, Spiegel K, Brichacek B, Sumegi J: X–linked lymphoproliferative disease. Am J Dis Child 147: 1242– 1245, 1993. 22 Imashuku S, Hibi S, Fujiwara F, Ikushima S, Todo S: Hemophagocytic lymphohistiocytosis, interferon-gamma-nemia and Epstein-Barr virus involvement. Br J Hematol 88: 656– 658, 1994. 23 Henter J-I, Elinder G: Cerebromeningeal hemophagocytic lymphohistiocytosis. Lancet i: 104– 107, 1992. 24 Henter J-I, Ehrnst A, Andersson J, Elinder G: Familial hemophagocytic lymphohistiocytosis and viral infections. Acta Paediatr 82: 369– 372, 1993. 25 Shapiro RS, Egeler RM, Loechelt BJ, Filipovich AH: Immune dysfunction in hemophagocytic lymphohistiocytosis. Abstract: 9th Annual Meeting of the Histiocyte Society, San Francisco, 1993. Med Pediatr Oncol 25: 143, 1995. 26 Ladisch S, Ho W, Matheson D, Pilkington R, Hartman G: Immunologic and clinical effects of repeated blood exchange in familial erythrophagocytic lymphohistiocytosis. Blood 60: 814– 821, 1982. 27 Ambruso DR, Hays T, Zwartjes WJ, Tubergen DG, Favara BE: Successful treatment of lymphohistiocytic reticulosis with phagocytosis with epipodophyllotoxin VP 16–213. Cancer 45: 2516– 2520, 1980. 28 Henter J-I, Elinder G, Finkel Y, Soder O: Successful induction with chemotherapy including teniposide in familial erythrophagocytic lymphohistiocytosis. Lancet ii: 1402, 1986. 29 Pui C-H, Ribeiro RC, Hancock ML, Rivera GK, Evans WE, Raimondi SC, Head DR, Behm FG, Mahmoud MH, Sandlund JT, Crist WM: Acute myeloid leukemia in children treated with epipodophyllotoxins for acute lymphoblastic leukemia. N Engl J Med 325: 1682– 1687, 1991. 30 Henter J-I, Elinder G, Liibeck P-O, Öst Å: Myelodysplastic syndrome following epipodophyllotoxin therapy in familial hemophagocytic lymphohistiocytosis. Pediatr Hematol Oncol 10: 163– 168, 1993. 31 Stephan JL, Donadieu J, Ledeist F, Blanche S, Griscelli C, Fischer A: Treatment of familial hemophagocytic lymphohistiocytosis with antithymocyte globulins, steroids and cyclosporin A. Blood 82: 2319– 2323, 1993. 32 Loechelt BJ, Egeler RM, Filipovich AH, Jyonouchi H, Shapiro RS: Immunosuppression: an alternative maintenance therapy for familial hemophagocytic lymphohistiocytosis (FHL): Preliminary results. Med Pediatr Oncol 22: 325– 328, 1994. 33 Akima M, Sumi SM: Neuropathy of familial erythrophagocytic lymphohistiocytosis. Six cases and review of the literature. Hum Pathol 15: 161– 168, 1984. 34 Fischer A, Virelizier JL, Arenzana-Seisdedos F, Perez A, Nezelof C, Griscelli C: Treatment of four patients with erythrophagocytic lymphohistiocytosis by a combination of epipodophyllotoxin, steroids, intrathecal methotrexate, and cranial irradiation. Pediatrics 76: 263– 268, 1985. 35 Henter J-I, Elinder G: Familial hemophagocytic lymphohistiocytosis. Clinical review based on the findings in seven children. Acta Paediatr Scand 80: 269– 277, 1991. 36 Fischer A, Cerf-Bensussan N, Blanche S, LeDeist F, Bremard-Oury C, Leverger G, Schaison G, Durandy A, Griscelli C: Allo-geneic bone marrow transplantation for erythrophagocytic lymphohistiocytosis. J Pediatr 108: 267– 270, 1986. 37 Todo S, Fujiwara F, Ikushima S, Yoshihara T, Kataoka Y, Sakurai-Shimada Y, Sakata K, Kasubuchi Y, Morioka Y, Imashuku S: Allogeneic bone marrow transplantation for familial erythrophagocytic lymphohistiocytosis with high dose VP16-containing conditioning regimen. Leukemia Lymphoma 1: 361– 364, 1990. 38 Blanche S, Caniglia M, Girault D, Landman J, Griscelli C, Fischer A: Treatment of hemophagocytic lymphohistiocytosis with chemotherapy and bone marrow transplantation: a single center study of 22 cases. Blood 78: 51– 54, 1991. 39 Locatelli F, Amoroso O, Arico M, Zecca M, Gambaana D, Mazzola G, Berrino M, El Borai HM, Severi F: BMT from a MUD in a patient affected by familial haemophagocytic lymphohistiocytosis: RFLP and PCR–fingerprinting monitoring of post–transplant chimerism. Proceedings EBMT meeting Stockholm 1992; abstract no 361, p 193. 40 Filipovich AH, Shapiro RS, Ramsay NKC, Kim T, Blazar B, Kersey J, McGlave P: Unrelated donor bone marrow transplantation for correction of lethal congenital immunodeficiencies. Blood 80: 270– 276, 1992. 41 Bolme P, Henter J-I, Winiarski J, Elinder G, Ljungman P, Lon–nerholm G, Ringdén O: Allogeneic bone marrow transplantation in hemophagocytic lymphohistiocytosis in Sweden. Bone Marrow Transplant 15: 331– 335, 1995. 42 Baker KS, DeLaat C, Shapiro R, Gross TG, Steinbuch M, Filipovich AH: Successful correction of hemophagocytic lymphohistiocytosis with related and unrelated bone marrow transportation. Blood 86, Suppl 1: 387a, 1995. Citing Literature Volume28, Issue5May 1997Pages 342-347 ReferencesRelatedInformation