메뉴 건너뛰기




Volumn 161, Issue 5, 2013, Pages 609-622

Advances in understanding the pathogenesis of HLH

Author keywords

Haemophagocytic syndrome; Immunodeficiency; Macrophages; Mutations; NK cells

Indexed keywords

ANEMIA; AP3B1 GENE; CHEDIAK HIGASHI SYNDROME; CLINICAL FEATURE; DISEASE ASSOCIATION; EPSTEIN BARR VIRUS INFECTION; FAMILIAL HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS TYPE 1; FAMILIAL HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS TYPE 2; FAMILIAL HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS TYPE 3; FAMILIAL HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS TYPE 4; FAMILIAL HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS TYPE 5; FEVER; GENE; GENE FUNCTION; GENE IDENTIFICATION; GENE MUTATION; GRISCELLI SYNDROME; HEMOPHAGOCYTIC SYNDROME; HUMAN; IMMUNE DEFICIENCY; LIVER DISEASE; LUNG DISEASE; LYMPHOPROLIFERATIVE DISEASE; LYST GENE; MACROPHAGE ACTIVATION SYNDROME; NEUROLOGIC DISEASE; OCULAR ALBINISM; PATHOPHYSIOLOGY; PHENOTYPE; PREVALENCE; PRF1 GENE; PRIORITY JOURNAL; RAB27A GENE; RETICULOENDOTHELIOSIS; REVIEW; SKIN DISEASE; STX11 GENE; STXBP2 GENE; SYNDROME DELINEATION; THROMBOCYTOPENIA; UNC13D GENE; X LINKED LYMPHOPROLIFERATIVE SYNDROME;

EID: 84877819974     PISSN: 00071048     EISSN: 13652141     Source Type: Journal    
DOI: 10.1111/bjh.12293     Document Type: Review
Times cited : (167)

References (131)
  • 1
    • 34548401192 scopus 로고    scopus 로고
    • Childhood acute myeloid leukemia with hemophagocytosis by the blasts and inv (8) (p11q13) with MOZ-TIF2 fusion transcripts
    • Abdelhaleem, M., Shago, M., Beimnet, K., Sayeh, E., Bartakke, S. & Weitzman, S. (2007) Childhood acute myeloid leukemia with hemophagocytosis by the blasts and inv (8) (p11q13) with MOZ-TIF2 fusion transcripts. Journal of Pediatric Hematology/Oncology, 29, 643-645.
    • (2007) Journal of Pediatric Hematology/Oncology , vol.29 , pp. 643-645
    • Abdelhaleem, M.1    Shago, M.2    Beimnet, K.3    Sayeh, E.4    Bartakke, S.5    Weitzman, S.6
  • 3
    • 42349095543 scopus 로고    scopus 로고
    • Highly elevated ferritin levels and the diagnosis of hemophagocytic lymphohistiocytosis
    • Allen, C.E., Yu, X., Kozinetz, C.A. & McClain, K.L. (2008) Highly elevated ferritin levels and the diagnosis of hemophagocytic lymphohistiocytosis. Pediatric Blood & Cancer, 50, 1227-1235.
    • (2008) Pediatric Blood & Cancer , vol.50 , pp. 1227-1235
    • Allen, C.E.1    Yu, X.2    Kozinetz, C.A.3    McClain, K.L.4
  • 4
    • 0029879812 scopus 로고    scopus 로고
    • Hemophagocytic lymphohistiocytosis. Report of 122 children from the international registry. FHL study group of the histiocyte society
    • Arico, M., Janka, G., Fischer, A., Henter, J.I., Blanche, S., Elinder, G., Martinetti, M. & Rusca, M.P. (1996) Hemophagocytic lymphohistiocytosis. Report of 122 children from the international registry. FHL study group of the histiocyte society. Leukemia, 10, 197-203.
    • (1996) Leukemia , vol.10 , pp. 197-203
    • Arico, M.1    Janka, G.2    Fischer, A.3    Henter, J.I.4    Blanche, S.5    Elinder, G.6    Martinetti, M.7    Rusca, M.P.8
  • 6
    • 34548406426 scopus 로고    scopus 로고
    • Use of rituximab in conjunction with immunosuppressive chemotherapy as a novel therapy for Epstein Barr virus-associated hemophagocytic lymphohistiocytosis
    • Balamuth, N.J., Nichols, K.E., Paessler, M. & Teachey, D.T. (2007) Use of rituximab in conjunction with immunosuppressive chemotherapy as a novel therapy for Epstein Barr virus-associated hemophagocytic lymphohistiocytosis. Journal of Pediatric Hematology/Oncology, 29, 569-573.
    • (2007) Journal of Pediatric Hematology/Oncology , vol.29 , pp. 569-573
    • Balamuth, N.J.1    Nichols, K.E.2    Paessler, M.3    Teachey, D.T.4
  • 9
    • 35848953001 scopus 로고    scopus 로고
    • Late onset of familial hemophagocytic lymphohistiocytosis
    • Bertaina, C., Bezzio, S. & Riva, C. (2007) Late onset of familial hemophagocytic lymphohistiocytosis. Minerva Pediatrica, 59, 416-417.
    • (2007) Minerva Pediatrica , vol.59 , pp. 416-417
    • Bertaina, C.1    Bezzio, S.2    Riva, C.3
  • 10
    • 67649909185 scopus 로고    scopus 로고
    • Infection of T lymphocytes in Epstein Barr virus-associated hemophagocytic lymphohistiocytosis in children of non-asian origin
    • Beutel, K., Gross-Wieltsch, U., Wiesel, T., Stadt, U.Z., Janka, G. & Wagner, H.J. (2009) Infection of T lymphocytes in Epstein Barr virus-associated hemophagocytic lymphohistiocytosis in children of non-asian origin. Pediatric Blood & Cancer, 53, 184-190.
    • (2009) Pediatric Blood & Cancer , vol.53 , pp. 184-190
    • Beutel, K.1    Gross-Wieltsch, U.2    Wiesel, T.3    Stadt, U.Z.4    Janka, G.5    Wagner, H.J.6
  • 11
    • 13544273826 scopus 로고    scopus 로고
    • Macrophage activation syndrome: characteristic findings on liver biopsy illustrating the key role of activated, IFN-gamma-producing lymphocytes and IL-6- and TNF-alpha-producing macrophages
    • Billiau, A.D., Roskams, T., Van Damme-Lombaerts, R., Matthys, P. & Wouters, C. (2005) Macrophage activation syndrome: characteristic findings on liver biopsy illustrating the key role of activated, IFN-gamma-producing lymphocytes and IL-6- and TNF-alpha-producing macrophages. Blood, 105, 1648-1651.
    • (2005) Blood , vol.105 , pp. 1648-1651
    • Billiau, A.D.1    Roskams, T.2    Van Damme-Lombaerts, R.3    Matthys, P.4    Wouters, C.5
  • 14
    • 78651365935 scopus 로고    scopus 로고
    • Rapid and sustained remission of systemic juvenile idiopathic arthritis-associated macrophage activation syndrome through treatment with anakinra and corticosteroids
    • Bruck, N., Suttorp, M., Kabus, M., Heubner, G., Gahr, M. & Pessler, F. (2011) Rapid and sustained remission of systemic juvenile idiopathic arthritis-associated macrophage activation syndrome through treatment with anakinra and corticosteroids. Journal of Clinical Rheumatology, 17, 23-27.
    • (2011) Journal of Clinical Rheumatology , vol.17 , pp. 23-27
    • Bruck, N.1    Suttorp, M.2    Kabus, M.3    Heubner, G.4    Gahr, M.5    Pessler, F.6
  • 16
    • 52249116745 scopus 로고    scopus 로고
    • High elevated ferritin levels and the diagnosis of HLH/sepsis/SIRS/MODS/MAS
    • author reply 710-1.
    • Castillo, L. (2008) High elevated ferritin levels and the diagnosis of HLH/sepsis/SIRS/MODS/MAS. Pediatric Blood & Cancer, 51, 710; author reply 710-1.
    • (2008) Pediatric Blood & Cancer , vol.51 , pp. 710
    • Castillo, L.1
  • 18
    • 79952143776 scopus 로고    scopus 로고
    • Hemophagocytic lymphohistiocytosis with MUNC13-4 gene mutation or reduced natural killer cell function prior to onset of childhood leukemia
    • Chang, T.Y., Jaffray, J., Woda, B., Newburger, P.E. & Usmani, G.N. (2011) Hemophagocytic lymphohistiocytosis with MUNC13-4 gene mutation or reduced natural killer cell function prior to onset of childhood leukemia. Pediatric Blood & Cancer, 56, 856-858.
    • (2011) Pediatric Blood & Cancer , vol.56 , pp. 856-858
    • Chang, T.Y.1    Jaffray, J.2    Woda, B.3    Newburger, P.E.4    Usmani, G.N.5
  • 19
    • 35348993736 scopus 로고    scopus 로고
    • Cytophagic histiocytic panniculitis with fatal haemophagocytic lymphohistiocytosis in a paediatric patient with perforin gene mutation
    • Chen, R.L., Hsu, Y.H., Ueda, I., Imashuku, S., Takeuchi, K., Tu, B.P. & Chuang, S.S. (2007) Cytophagic histiocytic panniculitis with fatal haemophagocytic lymphohistiocytosis in a paediatric patient with perforin gene mutation. Journal of Clinical Pathology, 60, 1168-1169.
    • (2007) Journal of Clinical Pathology , vol.60 , pp. 1168-1169
    • Chen, R.L.1    Hsu, Y.H.2    Ueda, I.3    Imashuku, S.4    Takeuchi, K.5    Tu, B.P.6    Chuang, S.S.7
  • 22
    • 27644592071 scopus 로고    scopus 로고
    • Epstein Barr virus LMP1 inhibits the expression of SAP gene and upregulates Th1 cytokines in the pathogenesis of hemophagocytic syndrome
    • Chuang, H.C., Lay, J.D., Hsieh, W.C., Wang, H.C., Chang, Y., Chuang, S.E. & Su, I.J. (2005) Epstein Barr virus LMP1 inhibits the expression of SAP gene and upregulates Th1 cytokines in the pathogenesis of hemophagocytic syndrome. Blood, 106, 3090-3096.
    • (2005) Blood , vol.106 , pp. 3090-3096
    • Chuang, H.C.1    Lay, J.D.2    Hsieh, W.C.3    Wang, H.C.4    Chang, Y.5    Chuang, S.E.6    Su, I.J.7
  • 25
    • 46149083954 scopus 로고    scopus 로고
    • Understanding organ dysfunction in hemophagocytic lymphohistiocytosis
    • Creput, C., Galicier, L., Buyse, S. & Azoulay, E. (2008) Understanding organ dysfunction in hemophagocytic lymphohistiocytosis. Intensive Care Medicine, 34, 1177-1187.
    • (2008) Intensive Care Medicine , vol.34 , pp. 1177-1187
    • Creput, C.1    Galicier, L.2    Buyse, S.3    Azoulay, E.4
  • 26
    • 77950213863 scopus 로고    scopus 로고
    • Neonatal liver failure and haemophagocytic lymphohistiocytosis caused by a new perforin mutation
    • (Oslo, Norway: 1992)
    • Danhaive, O., Caniglia, M., Devito, R., Piersigilli, F., Corchia, C. & Auriti, C. (2010) Neonatal liver failure and haemophagocytic lymphohistiocytosis caused by a new perforin mutation. Acta Paediatrica (Oslo, Norway: 1992), 99, 778-780.
    • (2010) Acta Paediatrica , vol.99 , pp. 778-780
    • Danhaive, O.1    Caniglia, M.2    Devito, R.3    Piersigilli, F.4    Corchia, C.5    Auriti, C.6
  • 27
    • 4844227884 scopus 로고    scopus 로고
    • Demyelinating peripheral neuropathy associated with hemophagocytic lymphohistiocytosis. An immuno-electron microscopic study
    • De Armas, R., Sindou, P., Gelot, A., Routon, M.C., Ponsot, G. & Vallat, J.M. (2004) Demyelinating peripheral neuropathy associated with hemophagocytic lymphohistiocytosis. An immuno-electron microscopic study. Acta Neuropathologica, 108, 341-344.
    • (2004) Acta Neuropathologica , vol.108 , pp. 341-344
    • De Armas, R.1    Sindou, P.2    Gelot, A.3    Routon, M.C.4    Ponsot, G.5    Vallat, J.M.6
  • 31
    • 33750433594 scopus 로고    scopus 로고
    • Infectious triggers of hemophagocytic syndrome in children
    • Domachowske, J.B. (2006) Infectious triggers of hemophagocytic syndrome in children. The Pediatric Infectious Disease Journal, 25, 1067-1068.
    • (2006) The Pediatric Infectious Disease Journal , vol.25 , pp. 1067-1068
    • Domachowske, J.B.1
  • 35
    • 36049020585 scopus 로고    scopus 로고
    • Gene expression profiling of peripheral blood from patients with untreated new-onset systemic juvenile idiopathic arthritis reveals molecular heterogeneity that may predict macrophage activation syndrome
    • Fall, N., Barnes, M., Thornton, S., Luyrink, L., Olson, J., Ilowite, N.T., Gottlieb, B.S., Griffin, T., Sherry, D.D., Thompson, S., Glass, D.N., Colbert, R.A. & Grom, A.A. (2007) Gene expression profiling of peripheral blood from patients with untreated new-onset systemic juvenile idiopathic arthritis reveals molecular heterogeneity that may predict macrophage activation syndrome. Arthritis & Rheumatism, 56, 3793-3804.
    • (2007) Arthritis & Rheumatism , vol.56 , pp. 3793-3804
    • Fall, N.1    Barnes, M.2    Thornton, S.3    Luyrink, L.4    Olson, J.5    Ilowite, N.T.6    Gottlieb, B.S.7    Griffin, T.8    Sherry, D.D.9    Thompson, S.10    Glass, D.N.11    Colbert, R.A.12    Grom, A.A.13
  • 37
    • 0037528195 scopus 로고    scopus 로고
    • Imaging characteristics of hemophagocytic lymphohistiocytosis
    • Fitzgerald, N.E. & MacClain, K.L. (2003) Imaging characteristics of hemophagocytic lymphohistiocytosis. Pediatric Radiology, 33, 392-401.
    • (2003) Pediatric Radiology , vol.33 , pp. 392-401
    • Fitzgerald, N.E.1    MacClain, K.L.2
  • 38
    • 33747128842 scopus 로고    scopus 로고
    • An exclusive case of juvenile myelomonocytic leukemia in association with kikuchi's disease and hemophagocytic lymphohistiocytosis and a review of the literature
    • Gerritsen, A., Lam, K., Marion Schneider, E. & van den Heuvel-Eibrink, M.M. (2006) An exclusive case of juvenile myelomonocytic leukemia in association with kikuchi's disease and hemophagocytic lymphohistiocytosis and a review of the literature. Leukemia Research, 30, 1299-1303.
    • (2006) Leukemia Research , vol.30 , pp. 1299-1303
    • Gerritsen, A.1    Lam, K.2    Marion Schneider, E.3    van den Heuvel-Eibrink, M.M.4
  • 39
    • 84857719455 scopus 로고    scopus 로고
    • Sensitivity and specificity of bone marrow hemophagocytosis in hemophagocytic lymphohistiocytosis
    • Goel, S., Polski, J.M. & Imran, H. (2012) Sensitivity and specificity of bone marrow hemophagocytosis in hemophagocytic lymphohistiocytosis. Annals of Clinical and Laboratory Science, 42, 21-25.
    • (2012) Annals of Clinical and Laboratory Science , vol.42 , pp. 21-25
    • Goel, S.1    Polski, J.M.2    Imran, H.3
  • 40
    • 35248868303 scopus 로고    scopus 로고
    • A spectrum of neuroradiological findings in children with haemophagocytic lymphohistiocytosis
    • Goo, H.W. & Weon, Y.C. (2007) A spectrum of neuroradiological findings in children with haemophagocytic lymphohistiocytosis. Pediatric Radiology, 37, 1110-1117.
    • (2007) Pediatric Radiology , vol.37 , pp. 1110-1117
    • Goo, H.W.1    Weon, Y.C.2
  • 41
    • 0037338925 scopus 로고    scopus 로고
    • Natural killer cell dysfunction in patients with systemic-onset juvenile rheumatoid arthritis and macrophage activation syndrome
    • Grom, A.A., Villanueva, J., Lee, S., Goldmuntz, E.A., Passo, M.H. & Filipovich, A. (2003) Natural killer cell dysfunction in patients with systemic-onset juvenile rheumatoid arthritis and macrophage activation syndrome. The Journal of Pediatrics, 142, 292-296.
    • (2003) The Journal of Pediatrics , vol.142 , pp. 292-296
    • Grom, A.A.1    Villanueva, J.2    Lee, S.3    Goldmuntz, E.A.4    Passo, M.H.5    Filipovich, A.6
  • 42
    • 48249124990 scopus 로고    scopus 로고
    • The role of the initial bone marrow aspirate in the diagnosis of hemophagocytic lymphohistiocytosis
    • Gupta, A., Tyrrell, P., Valani, R., Benseler, S., Weitzman, S. & Abdelhaleem, M. (2008) The role of the initial bone marrow aspirate in the diagnosis of hemophagocytic lymphohistiocytosis. Pediatric Blood & Cancer, 51, 402-404.
    • (2008) Pediatric Blood & Cancer , vol.51 , pp. 402-404
    • Gupta, A.1    Tyrrell, P.2    Valani, R.3    Benseler, S.4    Weitzman, S.5    Abdelhaleem, M.6
  • 45
    • 77951705321 scopus 로고    scopus 로고
    • Kawasaki disease preceding haemophagocytic lymphohistiocytosis: challenges for developing world practitioners
    • Hendricks, M., Pillay, S., Davidson, A., De Decker, R. & Lawrenson, J. (2010) Kawasaki disease preceding haemophagocytic lymphohistiocytosis: challenges for developing world practitioners. Pediatric Blood & Cancer, 54, 1023-1025.
    • (2010) Pediatric Blood & Cancer , vol.54 , pp. 1023-1025
    • Hendricks, M.1    Pillay, S.2    Davidson, A.3    De Decker, R.4    Lawrenson, J.5
  • 46
    • 0036013414 scopus 로고    scopus 로고
    • Biology and treatment of familial hemophagocytic lymphohistiocytosis: importance of perforin in lymphocyte-mediated cytotoxicity and triggering of apoptosis
    • Henter, J.I. (2002) Biology and treatment of familial hemophagocytic lymphohistiocytosis: importance of perforin in lymphocyte-mediated cytotoxicity and triggering of apoptosis. Medical and Pediatric Oncology, 38, 305-309.
    • (2002) Medical and Pediatric Oncology , vol.38 , pp. 305-309
    • Henter, J.I.1
  • 47
    • 42449140697 scopus 로고    scopus 로고
    • Pronounced hyperferritinemia: expanding the field of hemophagocytic lymphohistiocytosis
    • Henter, J.I. (2008) Pronounced hyperferritinemia: expanding the field of hemophagocytic lymphohistiocytosis. Pediatric Blood & Cancer, 50, 1127-1129.
    • (2008) Pediatric Blood & Cancer , vol.50 , pp. 1127-1129
    • Henter, J.I.1
  • 53
    • 37349104881 scopus 로고    scopus 로고
    • Systemic type Epstein Barr virus-related lymphoproliferative diseases in children and young adults: challenges for pediatric hemato-oncologists and infectious disease specialists
    • Imashuku, S. (2007) Systemic type Epstein Barr virus-related lymphoproliferative diseases in children and young adults: challenges for pediatric hemato-oncologists and infectious disease specialists. Pediatric Hematology and Oncology, 24, 563-568.
    • (2007) Pediatric Hematology and Oncology , vol.24 , pp. 563-568
    • Imashuku, S.1
  • 57
    • 84855932987 scopus 로고    scopus 로고
    • Familial and acquired hemophagocytic lymphohistiocytosis
    • Janka, G.E. (2012) Familial and acquired hemophagocytic lymphohistiocytosis. Annual Review of Medicine, 63, 233-246.
    • (2012) Annual Review of Medicine , vol.63 , pp. 233-246
    • Janka, G.E.1
  • 58
    • 54349115607 scopus 로고    scopus 로고
    • Hematopoietic cell transplantation for hemophagocytic lymphohistiocytosis: a journey of a thousand miles begins with a single (big) step
    • Jordan, M.B. & Filipovich, A.H. (2008) Hematopoietic cell transplantation for hemophagocytic lymphohistiocytosis: a journey of a thousand miles begins with a single (big) step. Bone Marrow Transplantation, 42, 433-437.
    • (2008) Bone Marrow Transplantation , vol.42 , pp. 433-437
    • Jordan, M.B.1    Filipovich, A.H.2
  • 59
    • 3242752040 scopus 로고    scopus 로고
    • An animal model of hemophagocytic lymphohistiocytosis (HLH): CD8+ T cells and interferon gamma are essential for the disorder
    • Jordan, M.B., Hildeman, D., Kappler, J. & Marrack, P. (2004) An animal model of hemophagocytic lymphohistiocytosis (HLH): CD8+ T cells and interferon gamma are essential for the disorder. Blood, 104, 735-743.
    • (2004) Blood , vol.104 , pp. 735-743
    • Jordan, M.B.1    Hildeman, D.2    Kappler, J.3    Marrack, P.4
  • 64
    • 79954506709 scopus 로고    scopus 로고
    • A novel single point mutation of the LYST gene in two siblings with different phenotypic features of chediak higashi syndrome
    • Kaya, Z., Ehl, S., Albayrak, M., Maul-Pavicic, A., Schwarz, K., Kocak, U., Ergun, M.A. & Gursel, T. (2011) A novel single point mutation of the LYST gene in two siblings with different phenotypic features of chediak higashi syndrome. Pediatric Blood & Cancer, 56, 1136-1139.
    • (2011) Pediatric Blood & Cancer , vol.56 , pp. 1136-1139
    • Kaya, Z.1    Ehl, S.2    Albayrak, M.3    Maul-Pavicic, A.4    Schwarz, K.5    Kocak, U.6    Ergun, M.A.7    Gursel, T.8
  • 65
    • 84859782246 scopus 로고    scopus 로고
    • Treatment of hemophagocytic lymphohistiocytosis with alemtuzumab in systemic lupus erythematosus
    • Keith, M.P., Pitchford, C. & Bernstein, W.B. (2012) Treatment of hemophagocytic lymphohistiocytosis with alemtuzumab in systemic lupus erythematosus. Journal of Clinical Rheumatology, 18, 134-137.
    • (2012) Journal of Clinical Rheumatology , vol.18 , pp. 134-137
    • Keith, M.P.1    Pitchford, C.2    Bernstein, W.B.3
  • 66
    • 34548359264 scopus 로고    scopus 로고
    • Recognition and management of macrophage activation syndrome in juvenile arthritis
    • Kelly, A. & Ramanan, A.V. (2007) Recognition and management of macrophage activation syndrome in juvenile arthritis. Current Opinion in Rheumatology, 19, 477-481.
    • (2007) Current Opinion in Rheumatology , vol.19 , pp. 477-481
    • Kelly, A.1    Ramanan, A.V.2
  • 67
    • 77949745791 scopus 로고    scopus 로고
    • Hyperferritinemia as the diagnostic clue in life-threatening hemophagocytic lymphohistiocytosis
    • Kerzel, S., Zemlin, M., Komhoff, M., Klaus, G. & Maier, R.F. (2009) Hyperferritinemia as the diagnostic clue in life-threatening hemophagocytic lymphohistiocytosis. Klinische Padiatrie, 221, 318-321.
    • (2009) Klinische Padiatrie , vol.221 , pp. 318-321
    • Kerzel, S.1    Zemlin, M.2    Komhoff, M.3    Klaus, G.4    Maier, R.F.5
  • 69
    • 0036095443 scopus 로고    scopus 로고
    • Perforin expression in cytotoxic lymphocytes from patients with hemophagocytic lymphohistiocytosis and their family members
    • Kogawa, K., Lee, S.M., Villanueva, J., Marmer, D., Sumegi, J. & Filipovich, A.H. (2002) Perforin expression in cytotoxic lymphocytes from patients with hemophagocytic lymphohistiocytosis and their family members. Blood, 99, 61-66.
    • (2002) Blood , vol.99 , pp. 61-66
    • Kogawa, K.1    Lee, S.M.2    Villanueva, J.3    Marmer, D.4    Sumegi, J.5    Filipovich, A.H.6
  • 70
    • 33646776086 scopus 로고    scopus 로고
    • Peripheral hemophagocytosis: an early indicator of advanced systemic inflammatory response syndrome/hemophagocytic syndrome
    • Kuwata, K., Yamada, S., Kinuwaki, E., Naito, M. & Mitsuya, H. (2006) Peripheral hemophagocytosis: an early indicator of advanced systemic inflammatory response syndrome/hemophagocytic syndrome. Shock (Augusta, Ga.), 25, 344-350.
    • (2006) Shock (Augusta, Ga.) , vol.25 , pp. 344-350
    • Kuwata, K.1    Yamada, S.2    Kinuwaki, E.3    Naito, M.4    Mitsuya, H.5
  • 71
    • 77953111101 scopus 로고    scopus 로고
    • Primary Epstein Barr virus infection associated with kikuchi's disease and hemophagocytic lymphohistiocytosis: a case report and review of the literature
    • Lee, H.Y., Huang, Y.C., Lin, T.Y., Huang, J.L., Yang, C.P., Hsueh, T., Wu, C.T. & Hsia, S.H. (2010) Primary Epstein Barr virus infection associated with kikuchi's disease and hemophagocytic lymphohistiocytosis: a case report and review of the literature. Journal of Microbiology, Immunology, and Infection, 43, 253-257.
    • (2010) Journal of Microbiology, Immunology, and Infection , vol.43 , pp. 253-257
    • Lee, H.Y.1    Huang, Y.C.2    Lin, T.Y.3    Huang, J.L.4    Yang, C.P.5    Hsueh, T.6    Wu, C.T.7    Hsia, S.H.8
  • 72
    • 45449104413 scopus 로고    scopus 로고
    • Hemophagocytic lymphohistiocytosis preceded by kikuchi disease in children
    • Lim, G.Y., Cho, B. & Chung, N.G. (2008) Hemophagocytic lymphohistiocytosis preceded by kikuchi disease in children. Pediatric Radiology, 38, 756-761.
    • (2008) Pediatric Radiology , vol.38 , pp. 756-761
    • Lim, G.Y.1    Cho, B.2    Chung, N.G.3
  • 73
    • 37149047480 scopus 로고    scopus 로고
    • Recurrent hemophagocytic lymphohistiocytosis accompanied by kikuchi's disease
    • Lin, Y.W., Horiuchi, H., Ueda, I. & Nambu, M. (2007) Recurrent hemophagocytic lymphohistiocytosis accompanied by kikuchi's disease. Leukemia & Lymphoma, 48, 2447-2451.
    • (2007) Leukemia & Lymphoma , vol.48 , pp. 2447-2451
    • Lin, Y.W.1    Horiuchi, H.2    Ueda, I.3    Nambu, M.4
  • 74
    • 84865986803 scopus 로고    scopus 로고
    • Clinical analysis of macrophage activation syndrome in pediatric patients with autoimmune diseases
    • Lin, C.I., Yu, H.H., Lee, J.H., Wang, L.C., Lin, Y.T., Yang, Y.H. & Chiang, B.L. (2012) Clinical analysis of macrophage activation syndrome in pediatric patients with autoimmune diseases. Clinical Rheumatology, 31, 1223-1230.
    • (2012) Clinical Rheumatology , vol.31 , pp. 1223-1230
    • Lin, C.I.1    Yu, H.H.2    Lee, J.H.3    Wang, L.C.4    Lin, Y.T.5    Yang, Y.H.6    Chiang, B.L.7
  • 77
    • 79960714121 scopus 로고    scopus 로고
    • Perforin is a critical physiologic regulator of T-cell activation
    • Lykens, J.E., Terrell, C.E., Zoller, E.E., Risma, K. & Jordan, M.B. (2011) Perforin is a critical physiologic regulator of T-cell activation. Blood, 118, 618-626.
    • (2011) Blood , vol.118 , pp. 618-626
    • Lykens, J.E.1    Terrell, C.E.2    Zoller, E.E.3    Risma, K.4    Jordan, M.B.5
  • 79
    • 79551628425 scopus 로고    scopus 로고
    • Unusual functional manifestations of a novel STX11 frameshift mutation in two infants with familial hemophagocytic lymphohistiocytosis type 4 (FHL4)
    • Macartney, C.A., Weitzman, S., Wood, S.M., Bansal, D., Steele, M., Meeths, M., Abdelhaleem, M. & Bryceson, Y.T. (2011) Unusual functional manifestations of a novel STX11 frameshift mutation in two infants with familial hemophagocytic lymphohistiocytosis type 4 (FHL4). Pediatric Blood & Cancer, 56, 654-657.
    • (2011) Pediatric Blood & Cancer , vol.56 , pp. 654-657
    • Macartney, C.A.1    Weitzman, S.2    Wood, S.M.3    Bansal, D.4    Steele, M.5    Meeths, M.6    Abdelhaleem, M.7    Bryceson, Y.T.8
  • 83
    • 33747618010 scopus 로고    scopus 로고
    • Perforin polymorphism A91V and susceptibility to B-precursor childhood acute lymphoblastic leukemia: a report from the children's oncology group
    • Children's Oncology Group.
    • Mehta, P.A., Davies, S.M., Kumar, A., Devidas, M., Lee, S., Zamzow, T., Elliott, J., Villanueva, J., Pullen, J., Zewge, Y., Filipovich, A. & Children's Oncology Group. (2006) Perforin polymorphism A91V and susceptibility to B-precursor childhood acute lymphoblastic leukemia: a report from the children's oncology group. Leukemia, 20, 1539-1541.
    • (2006) Leukemia , vol.20 , pp. 1539-1541
    • Mehta, P.A.1    Davies, S.M.2    Kumar, A.3    Devidas, M.4    Lee, S.5    Zamzow, T.6    Elliott, J.7    Villanueva, J.8    Pullen, J.9    Zewge, Y.10    Filipovich, A.11
  • 89
    • 0037311129 scopus 로고    scopus 로고
    • Macrophage activation syndrome following initiation of etanercept in a child with systemic onset juvenile rheumatoid arthritis
    • Ramanan, A.V. & Schneider, R. (2003) Macrophage activation syndrome following initiation of etanercept in a child with systemic onset juvenile rheumatoid arthritis. The Journal of Rheumatology, 30, 401-403.
    • (2003) The Journal of Rheumatology , vol.30 , pp. 401-403
    • Ramanan, A.V.1    Schneider, R.2
  • 90
    • 84861685616 scopus 로고    scopus 로고
    • Macrophage activation syndrome as part of systemic juvenile idiopathic arthritis: diagnosis, genetics, pathophysiology and treatment
    • Ravelli, A., Grom, A.A., Behrens, E.M. & Cron, R.Q. (2012) Macrophage activation syndrome as part of systemic juvenile idiopathic arthritis: diagnosis, genetics, pathophysiology and treatment. Genes and Immunity, 13, 289-298.
    • (2012) Genes and Immunity , vol.13 , pp. 289-298
    • Ravelli, A.1    Grom, A.A.2    Behrens, E.M.3    Cron, R.Q.4
  • 91
    • 0018656157 scopus 로고
    • Virus-associated hemophagocytic syndrome A benign histiocytic proliferation distinct from malignant histiocytosis
    • Risdall, R.J., McKenna, R.W., Nesbit, M.E., Krivit, W., Balfour, H.H., Simmons, R.L. & Brunning, R.D. (1979) Virus-associated hemophagocytic syndrome A benign histiocytic proliferation distinct from malignant histiocytosis. Cancer, 44, 993-1002.
    • (1979) Cancer , vol.44 , pp. 993-1002
    • Risdall, R.J.1    McKenna, R.W.2    Nesbit, M.E.3    Krivit, W.4    Balfour, H.H.5    Simmons, R.L.6    Brunning, R.D.7
  • 92
    • 31044453814 scopus 로고    scopus 로고
    • Aberrant maturation of mutant perforin underlies the clinical diversity of hemophagocytic lymphohistiocytosis
    • Risma, K.A., Frayer, R.W., Filipovich, A.H. & Sumegi, J. (2006) Aberrant maturation of mutant perforin underlies the clinical diversity of hemophagocytic lymphohistiocytosis. The Journal of Clinical Investigation, 116, 182-192.
    • (2006) The Journal of Clinical Investigation , vol.116 , pp. 182-192
    • Risma, K.A.1    Frayer, R.W.2    Filipovich, A.H.3    Sumegi, J.4
  • 93
    • 33745052933 scopus 로고    scopus 로고
    • Spectrum and clinical implications of syntaxin 11 gene mutations in familial haemophagocytic lymphohistiocytosis: association with disease-free remissions and haematopoietic malignancies
    • Rudd, E., Goransdotter Ericson, K., Zheng, C., Uysal, Z., Ozkan, A., Gurgey, A., Fadeel, B., Nordenskjold, M. & Henter, J.I. (2006) Spectrum and clinical implications of syntaxin 11 gene mutations in familial haemophagocytic lymphohistiocytosis: association with disease-free remissions and haematopoietic malignancies. Journal of Medical Genetics, 43, e14.
    • (2006) Journal of Medical Genetics , vol.43
    • Rudd, E.1    Goransdotter Ericson, K.2    Zheng, C.3    Uysal, Z.4    Ozkan, A.5    Gurgey, A.6    Fadeel, B.7    Nordenskjold, M.8    Henter, J.I.9
  • 97
    • 20344362940 scopus 로고    scopus 로고
    • A single amino acid change A91V in perforin: a novel, frequent predisposing factor to childhood acute lymphoblastic leukemia?
    • amp; ()
    • Santoro, A., Cannella, S., Trizzino, A., Lo Nigro, L., Corsello, G. & Arico, M. (2005) A single amino acid change A91V in perforin: a novel, frequent predisposing factor to childhood acute lymphoblastic leukemia? Haematologica, 90, 697-698.
    • (2005) Haematologica , vol.90 , pp. 697-698
    • Santoro, A.1    Cannella, S.2    Trizzino, A.3    Lo Nigro, L.4    Corsello, G.5    Arico, M.6
  • 99
    • 0037108557 scopus 로고    scopus 로고
    • Hemophagocytic lymphohistiocytosis is associated with deficiencies of cellular cytolysis but normal expression of transcripts relevant to killer-cell-induced apoptosis
    • Schneider, E.M., Lorenz, I., Muller-Rosenberger, M., Steinbach, G., Kron, M. & Janka-Schaub, G.E. (2002) Hemophagocytic lymphohistiocytosis is associated with deficiencies of cellular cytolysis but normal expression of transcripts relevant to killer-cell-induced apoptosis. Blood, 100, 2891-2898.
    • (2002) Blood , vol.100 , pp. 2891-2898
    • Schneider, E.M.1    Lorenz, I.2    Muller-Rosenberger, M.3    Steinbach, G.4    Kron, M.5    Janka-Schaub, G.E.6
  • 100
    • 0000041771 scopus 로고
    • Histiocytic medullary reticulosis
    • Scott, R.B. & Robb-Smith, A.H.T. (1939) Histiocytic medullary reticulosis. Lancet, 2, 194-198.
    • (1939) Lancet , vol.2 , pp. 194-198
    • Scott, R.B.1    Robb-Smith, A.H.T.2
  • 102
    • 58149240252 scopus 로고    scopus 로고
    • Hemophagocytic lymphohistiocytosis associated with uncontrolled inflammatory cytokinemia and chemokinemia was caused by systemic anaplastic large cell lymphoma: a case report and review of the literature
    • Shimada, A., Kato, M., Tamura, K., Hirato, J., Kanegane, H., Takechi, Y., Park, M.J., Sotomatsu, M., Hatakeyama, S. & Hayashi, Y. (2008) Hemophagocytic lymphohistiocytosis associated with uncontrolled inflammatory cytokinemia and chemokinemia was caused by systemic anaplastic large cell lymphoma: a case report and review of the literature. Journal of Pediatric Hematology/Oncology, 30, 785-787.
    • (2008) Journal of Pediatric Hematology/Oncology , vol.30 , pp. 785-787
    • Shimada, A.1    Kato, M.2    Tamura, K.3    Hirato, J.4    Kanegane, H.5    Takechi, Y.6    Park, M.J.7    Sotomatsu, M.8    Hatakeyama, S.9    Hayashi, Y.10
  • 106
    • 29944442846 scopus 로고    scopus 로고
    • Mutation spectrum in children with primary hemophagocytic lymphohistiocytosis: molecular and functional analyses of PRF1, UNC-13D STX11, and RAB27A
    • zur Stadt, U., Beutel, K., Kolberg, S., Schneppenheim, R., Kabisch, H., Janka, G. & Hennies, H.C. (2006) Mutation spectrum in children with primary hemophagocytic lymphohistiocytosis: molecular and functional analyses of PRF1, UNC-13D STX11, and RAB27A. Human Mutation, 27, 62-68.
    • (2006) Human Mutation , vol.27 , pp. 62-68
    • Zur Stadt, U.1    Beutel, K.2    Kolberg, S.3    Schneppenheim, R.4    Kabisch, H.5    Janka, G.6    Hennies, H.C.7
  • 108
    • 0033520970 scopus 로고    scopus 로고
    • Perforin gene defects in familial hemophagocytic lymphohistiocytosis
    • Stepp, S.E. (1999) Perforin gene defects in familial hemophagocytic lymphohistiocytosis. Science (New York, N.Y.), 286, 1957-1959.
    • (1999) Science (New York, N.Y.) , vol.286 , pp. 1957-1959
    • Stepp, S.E.1
  • 110
    • 84874100105 scopus 로고    scopus 로고
    • Gene expression signatures differ between different clinical forms of familial hemophagocytic lymphohistiocytosis
    • Sumegi, J., Nestheide, S.V., Barnes, M.G., Villanueva, J., Zhang, K., Grom, A.A. & Filipovich, A.H. (2012) Gene expression signatures differ between different clinical forms of familial hemophagocytic lymphohistiocytosis. Blood, 121, e14-24.
    • (2012) Blood , vol.121
    • Sumegi, J.1    Nestheide, S.V.2    Barnes, M.G.3    Villanueva, J.4    Zhang, K.5    Grom, A.A.6    Filipovich, A.H.7
  • 111
    • 17044424680 scopus 로고    scopus 로고
    • Rapid detection of intracellular SH2D1A protein in cytotoxic lymphocytes from patients with X-linked lymphoproliferative disease and their family members
    • Tabata, Y., Villanueva, J., Lee, S.M., Zhang, K., Kanegane, H., Miyawaki, T., Sumegi, J. & Filipovich, A.H. (2005) Rapid detection of intracellular SH2D1A protein in cytotoxic lymphocytes from patients with X-linked lymphoproliferative disease and their family members. Blood, 105, 3066-3071.
    • (2005) Blood , vol.105 , pp. 3066-3071
    • Tabata, Y.1    Villanueva, J.2    Lee, S.M.3    Zhang, K.4    Kanegane, H.5    Miyawaki, T.6    Sumegi, J.7    Filipovich, A.H.8
  • 112
    • 33747454224 scopus 로고    scopus 로고
    • Hemophagocytic syndrome preceding acute myeloid leukemia with der t [7:17] [q12; q11], monosomy, 17 and 5p-
    • Tadmor, T., Vadazs, Z., Dar, H., Laor, R. & Attias, D. (2006) Hemophagocytic syndrome preceding acute myeloid leukemia with der t [7:17] [q12; q11], monosomy, 17 and 5p-. Journal of Pediatric Hematology/Oncology, 28, 544-546.
    • (2006) Journal of Pediatric Hematology/Oncology , vol.28 , pp. 544-546
    • Tadmor, T.1    Vadazs, Z.2    Dar, H.3    Laor, R.4    Attias, D.5
  • 114
    • 52249093922 scopus 로고    scopus 로고
    • Increased serum monocyte chemoattractant protein-1, macrophage inflammatory protein-1beta, and interleukin-8 concentrations in hemophagocytic lymphohistiocytosis
    • Tamura, K., Kanazawa, T., Tsukada, S., Kobayashi, T., Kawamura, M. & Morikawa, A. (2008) Increased serum monocyte chemoattractant protein-1, macrophage inflammatory protein-1beta, and interleukin-8 concentrations in hemophagocytic lymphohistiocytosis. Pediatric Blood & Cancer, 51, 662-668.
    • (2008) Pediatric Blood & Cancer , vol.51 , pp. 662-668
    • Tamura, K.1    Kanazawa, T.2    Tsukada, S.3    Kobayashi, T.4    Kawamura, M.5    Morikawa, A.6
  • 115
    • 0036119678 scopus 로고    scopus 로고
    • Quantitative analysis of cell-free Epstein Barr virus genome copy number in patients with EBV-associated hemophagocytic lymphohistiocytosis
    • Teramura, T., Tabata, Y., Yagi, T., Morimoto, A., Hibi, S. & Imashuku, S. (2002) Quantitative analysis of cell-free Epstein Barr virus genome copy number in patients with EBV-associated hemophagocytic lymphohistiocytosis. Leukemia & Lymphoma, 43, 173-179.
    • (2002) Leukemia & Lymphoma , vol.43 , pp. 173-179
    • Teramura, T.1    Tabata, Y.2    Yagi, T.3    Morimoto, A.4    Hibi, S.5    Imashuku, S.6
  • 118
    • 33644669225 scopus 로고    scopus 로고
    • Correlation between phenotypic heterogeneity and gene mutational characteristics in familial hemophagocytic lymphohistiocytosis (FHL)
    • Ueda, I., Ishii, E., Morimoto, A., Ohga, S., Sako, M. & Imashuku, S. (2006) Correlation between phenotypic heterogeneity and gene mutational characteristics in familial hemophagocytic lymphohistiocytosis (FHL). Pediatric Blood & Cancer, 46, 482-488.
    • (2006) Pediatric Blood & Cancer , vol.46 , pp. 482-488
    • Ueda, I.1    Ishii, E.2    Morimoto, A.3    Ohga, S.4    Sako, M.5    Imashuku, S.6
  • 120
    • 21744452702 scopus 로고    scopus 로고
    • Natural killer cell dysfunction is a distinguishing feature of systemic onset juvenile rheumatoid arthritis and macrophage activation syndrome
    • Villanueva, J., Lee, S., Giannini, E.H., Graham, T.B., Passo, M.H., Filipovich, A. & Grom, A.A. (2005) Natural killer cell dysfunction is a distinguishing feature of systemic onset juvenile rheumatoid arthritis and macrophage activation syndrome. Arthritis Research & Therapy, 7, R30-R37.
    • (2005) Arthritis Research & Therapy , vol.7
    • Villanueva, J.1    Lee, S.2    Giannini, E.H.3    Graham, T.B.4    Passo, M.H.5    Filipovich, A.6    Grom, A.A.7
  • 122
    • 58049168372 scopus 로고    scopus 로고
    • Diagnostic challenges in a child with familial hemophagocytic lymphohistiocytosis type 3 (FHLH3) presenting with fulminant neurological disease
    • Weisfeld-Adams, J.D., Frank, Y., Havalad, V., Hojsak, J.M., Posada, R., Kaicker, S.M. & Wistinghausen, B. (2009) Diagnostic challenges in a child with familial hemophagocytic lymphohistiocytosis type 3 (FHLH3) presenting with fulminant neurological disease. Child's Nervous System, 25, 153-159.
    • (2009) Child's Nervous System , vol.25 , pp. 153-159
    • Weisfeld-Adams, J.D.1    Frank, Y.2    Havalad, V.3    Hojsak, J.M.4    Posada, R.5    Kaicker, S.M.6    Wistinghausen, B.7
  • 126
    • 84872429599 scopus 로고    scopus 로고
    • Characterization of Epstein Barr virus (EBV)-infected cells in EBV-associated hemophagocytic lymphohistiocytosis in two patients with X-linked lymphoproliferative syndrome type 1 and type 2
    • Yang, X., Wada, T., Imadome, K., Nishida, N., Mukai, T., Fujiwara, M., Kawashima, H., Kato, F., Fujiwara, S., Yachie, A., Zhao, X., Miyawaki, T. & Kanegane, H. (2012) Characterization of Epstein Barr virus (EBV)-infected cells in EBV-associated hemophagocytic lymphohistiocytosis in two patients with X-linked lymphoproliferative syndrome type 1 and type 2. Herpesviridae, 3, 1.
    • (2012) Herpesviridae , vol.3 , pp. 1
    • Yang, X.1    Wada, T.2    Imadome, K.3    Nishida, N.4    Mukai, T.5    Fujiwara, M.6    Kawashima, H.7    Kato, F.8    Fujiwara, S.9    Yachie, A.10    Zhao, X.11    Miyawaki, T.12    Kanegane, H.13
  • 127
    • 84874207708 scopus 로고    scopus 로고
    • Efficacy, safety and tolerability of tocilizumab in patients with systemic juvenile idiopathic arthritis
    • Yokota, S., Tanaka, T. & Kishimoto, T. (2012) Efficacy, safety and tolerability of tocilizumab in patients with systemic juvenile idiopathic arthritis. Therapeutic Advances in Musculoskeletal Disease, 4, 387-397.
    • (2012) Therapeutic Advances in Musculoskeletal Disease , vol.4 , pp. 387-397
    • Yokota, S.1    Tanaka, T.2    Kishimoto, T.3
  • 128
    • 77950540046 scopus 로고    scopus 로고
    • Cytotoxic T-lymphocyte-associated antigen 4 gene polymorphisms in Japanese children with infection-associated hemophagocytic lymphohistiocytosis
    • Yoshiyama, M., Kounami, S., Nakayama, K., Okutani, T., Aoyagi, N. & Yoshikawa, N. (2010) Cytotoxic T-lymphocyte-associated antigen 4 gene polymorphisms in Japanese children with infection-associated hemophagocytic lymphohistiocytosis. Acta Haematologica, 123, 186-190.
    • (2010) Acta Haematologica , vol.123 , pp. 186-190
    • Yoshiyama, M.1    Kounami, S.2    Nakayama, K.3    Okutani, T.4    Aoyagi, N.5    Yoshikawa, N.6
  • 130
    • 46949093535 scopus 로고    scopus 로고
    • Syntaxin-11 is expressed in primary human monocytes/macrophages and acts as a negative regulator of macrophage engulfment of apoptotic cells and IgG-opsonized target cells
    • Zhang, S., Ma, D., Wang, X., Celkan, T., Nordenskjold, M., Henter, J.I., Fadeel, B. & Zheng, C. (2008b) Syntaxin-11 is expressed in primary human monocytes/macrophages and acts as a negative regulator of macrophage engulfment of apoptotic cells and IgG-opsonized target cells. British Journal of Haematology, 142, 469-479.
    • (2008) British Journal of Haematology , vol.142 , pp. 469-479
    • Zhang, S.1    Ma, D.2    Wang, X.3    Celkan, T.4    Nordenskjold, M.5    Henter, J.I.6    Fadeel, B.7    Zheng, C.8


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.