A Case of Epstein-Barr Virus-Associated Peripheral T-Cell Lymphoma
A 4-year-old girl developed Epstein-Barr virus (EBV) -associated peripheral T-cell lymphoma (PTCL), showing hemophagocytic lymphohistiocytosis. Bone marrow smears showed an increase of atypical immuno-blasts with mature T-cell antigens and some histiocytic hemophagocytes. Southern blot analyses of b...
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| Published in | The Japanese Journal of Pediatric Hematology Vol. 11; no. 3; pp. 193 - 197 |
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| Main Authors | , , , , , , , , , |
| Format | Journal Article |
| Language | Japanese |
| Published |
THE JAPANESE SOCIETY OF PEDIATRIC HEMATOLOGY/ONCOLOGY
1997
特定非営利活動法人 日本小児血液・がん学会 |
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| ISSN | 0913-8706 1884-4723 |
| DOI | 10.11412/jjph1987.11.193 |
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| Abstract | A 4-year-old girl developed Epstein-Barr virus (EBV) -associated peripheral T-cell lymphoma (PTCL), showing hemophagocytic lymphohistiocytosis. Bone marrow smears showed an increase of atypical immuno-blasts with mature T-cell antigens and some histiocytic hemophagocytes. Southern blot analyses of bone marrow cells by TCR C-β-1 and EBV terminal probes revealed the monoclonal proliferation of EBV-infected T-cells. The patient received HLH-94 protocol and achieved remission in a month. At 50 days after the onset, however, the disease recurred. Combined chemotherapy with cyclosporin A improved her condition transiently. At the 7th month, she received an allogeneic bone marrow transplantation (BMT) from her HLA-identical brother. She is doing well 6 months after the transplantation. It is suggested that allogeneic BMT is an effective treatment for refractory EBV/PTCL. |
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| AbstractList | A 4-year-old girl developed Epstein-Barr virus (EBV) -associated peripheral T-cell lymphoma (PTCL), showing hemophagocytic lymphohistiocytosis. Bone marrow smears showed an increase of atypical immuno-blasts with mature T-cell antigens and some histiocytic hemophagocytes. Southern blot analyses of bone marrow cells by TCR C-β-1 and EBV terminal probes revealed the monoclonal proliferation of EBV-infected T-cells. The patient received HLH-94 protocol and achieved remission in a month. At 50 days after the onset, however, the disease recurred. Combined chemotherapy with cyclosporin A improved her condition transiently. At the 7th month, she received an allogeneic bone marrow transplantation (BMT) from her HLA-identical brother. She is doing well 6 months after the transplantation. It is suggested that allogeneic BMT is an effective treatment for refractory EBV/PTCL. A 4-year-old girl developed Epstein-Barr virus (EBV) -associated peripheral T-cell lymphoma (PTCL), showing hemophagocytic lymphohistiocytosis. Bone marrow smears showed an increase of atypical immuno-blasts with mature T-cell antigens and some histiocytic hemophagocytes. Southern blot analyses of bone marrow cells by TCR C-β-1 and EBV terminal probes revealed the monoclonal proliferation of EBV-infected T-cells. The patient received HLH-94 protocol and achieved remission in a month. At 50 days after the onset, however, the disease recurred. Combined chemotherapy with cyclosporin A improved her condition transiently. At the 7th month, she received an allogeneic bone marrow transplantation (BMT) from her HLA-identical brother. She is doing well 6 months after the transplantation. It is suggested that allogeneic BMT is an effective treatment for refractory EBV/PTCL. 血球貧食症候群を伴うEpstein-Barrウイルス関連末梢T細胞性悪性リンパ腫 (EBV/PTCL) を発症した4歳女児について報告した.骨髄に成熟T細胞表面マーカーを持つ異型なimmunoblastが増加し, 同時にCβ1 probeおよびEBV terminal probeによる骨髄細胞のサザン解析によりEBV/PTCLと診断した.当初HLH-94プロトコールに良好な反応が得られ寛解を得たが, 第50病日に再燃した.その後種々の多剤併用療法を行うも効果は一時的で, 徐々に治療抵抗性となった.Cyclosporin A併用により一時全身状態は改善するも, その後も再燃を繰り返すため, 発症後7カ月でHLA一致の弟をドナーとして同種骨髄移植を行った.移植後6カ月を経過した現在も再燃を認めず, 同種骨髄移植は治療抵抗性EBV/PTCLに有効な治療法であると思われた. |
| Author | NAYA, Mayumi IMASHUKU, Shinsaku OSAWA, Minako SAWADA, Tadashi NAKABAYASHI, Yoshinobu TABATA, Yasuhiro HIBI, Shigeyoshi TODO, Shinjiro KIHARA, Akio HOJO, Makoto |
| Author_FL | 中林 佳信 木原 明生 田畑 恭裕 納谷 真由美 今宿 晋作 澤田 淳 北條 誠 東道 伸二郎 日比 成美 大澤 美奈子 |
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| References | 15) Watson HG, Goulden NJ, Manson LM, et al : Virusassociated haemophagocytic syndrome : further evidence for a T-cell mediated disorder. Br J Haematol 86 : 213-215, 1994 14) Imashuku S, Hibi S, Fujiwara F, et al : Haemophagocytic lymphohistiocytosis, interferon-gammanaemia and Epstein-Barr virus involvement. Br J Haematol 88 : 656-658, 1994 10) Korbjuhn P, Anagnostopoulos I, Hummel M, et al : Frequent latent Epstein-Barr virus infection of neoplastic T cells and bystander B cells in human immunodeficiency virus-negative European peripheral pleomorphic T-cell lymphomas. Blood 82 : 217-223, 1993 9) Hart DNJ, Baker BW, Inglis MJ, et al : Epstein-Barr viral DNA in acute large granular lymphocyte (natural killer) leukemic cells. Blood 79 : 2116-2123, 1992 12) Esumi N, Ikushima S, Hibi S, et al : High serum ferritin level as a marker of malignant histiocytosis and virus-associated hemophagocytic syndrome. Cancer 61 : 2071-2076, 1988 5) Falini B, Pileri S, De Solas I, et al : Peripheral T-cell lymphoma associated with hemophagocytic syndrome. Blood 75 : 434-444, 1990 8) Yao M, Cheng AL, Su IJ, et al : Clinicopathologicalspectrum of haemophagocytic syndrome in Epstein-Barr virus-associated peripheral T-cell lymphoma. Br J Haematol 87 : 535-543, 1994 2) 今宿晋作, 日比成美 : *Histiocyte SocietyによるHLH-94 (hemophagocytic lymphohistiocytosisの治療に関する国際統一プロトコール) について.日児誌99 : 281, 1995 17) Oyama Y, Amano T, Hirakawa S, et al : Haemophagocytic syndrome treated with cyclosporin A : A T cell disorder ? Br J Haematol 73 : 276-278, 1989 11) Hibi S, Ikushima S, Fujiwara F, et al : Serum and urine beta-2-microglobulin in hemophagocytic syndrome. Cancer 75 : 1700-1705, 1995 6) Lee SH, Su IJ, Chen RL, et al : A pathologic study of childhood lymphoma in Taiwan with special reference to peripheral T-cell lymphoma and the association with Epstein-Barr viral infection. Cancer 68 : 1954-1962, 1991 19) Saeki T, Ueda K, Tanigawara Y, et al : Human Pglycoprotein transports cyclosporin A and FK506. J Biol Chem 268 : 6077-6080, 1993 16) Imashuku S, Hibi S : Cytokines in haemophagocytic syndrome. Br J Haematol 77 : 438-440, 1991 21) Teshima T, Miyaji R, Fukuda M, et al : Bone-marrow transplantation for Epstein-Barr-virus-associated natural killer cell-large granular lymphocyte leukaemia. Lancet 347 : 1124, 1996 7) Cheng AL, Su IJ, Chen YC, et al : Characteristic clinicopathologic features of Epstein-Barr virus-associated peripheral T-cell lymphoma. Cancer 72 : 909-916, 1993 20) 今宿晋作 : HPS, 治療抵抗性LCH, EBV関連免疫異常症例に対する同種骨髄移植.日本医事新報3736 : 13-20, 1995 18) Cooper DL, Braverman IM, Sarris AH, et al : Cyclosporine treatment of refractory T-cell lymphomas. Cancer 71 : 2335-2341, 1993 13) Imashuku S, Hibi S, Sako M, et al : Soluble interleukin-2 recepter : a useful prognostic factor for patients with hemophagocytic lymphohistiocytosis. Blood 86 : 4706-4707, 1995 4) Su IJ, Hsieh HC, Lin KH, et al : Aggressive peripheral T-cell lymphomas containing Epstein-Barr viral DNA : a clinicopathologic and molecular analysis. Blood 77 : 799-808, 1991 3) Henter JI, Arico M, Egeler RM, et al : HLH-94 : a treatment protocol for hemophagocytic lymphohistiocytosis. Med Pediatr Oncol 28 : 342-347, 1997 1) 今宿晋作 : リンパ系腫瘍に伴う血球貧食症候群におけるサイトカインの関与.臨床医22 : 103-107, 1996 |
| References_xml | – reference: 10) Korbjuhn P, Anagnostopoulos I, Hummel M, et al : Frequent latent Epstein-Barr virus infection of neoplastic T cells and bystander B cells in human immunodeficiency virus-negative European peripheral pleomorphic T-cell lymphomas. Blood 82 : 217-223, 1993 – reference: 3) Henter JI, Arico M, Egeler RM, et al : HLH-94 : a treatment protocol for hemophagocytic lymphohistiocytosis. Med Pediatr Oncol 28 : 342-347, 1997 – reference: 9) Hart DNJ, Baker BW, Inglis MJ, et al : Epstein-Barr viral DNA in acute large granular lymphocyte (natural killer) leukemic cells. Blood 79 : 2116-2123, 1992 – reference: 16) Imashuku S, Hibi S : Cytokines in haemophagocytic syndrome. Br J Haematol 77 : 438-440, 1991 – reference: 18) Cooper DL, Braverman IM, Sarris AH, et al : Cyclosporine treatment of refractory T-cell lymphomas. Cancer 71 : 2335-2341, 1993 – reference: 7) Cheng AL, Su IJ, Chen YC, et al : Characteristic clinicopathologic features of Epstein-Barr virus-associated peripheral T-cell lymphoma. Cancer 72 : 909-916, 1993 – reference: 4) Su IJ, Hsieh HC, Lin KH, et al : Aggressive peripheral T-cell lymphomas containing Epstein-Barr viral DNA : a clinicopathologic and molecular analysis. Blood 77 : 799-808, 1991 – reference: 17) Oyama Y, Amano T, Hirakawa S, et al : Haemophagocytic syndrome treated with cyclosporin A : A T cell disorder ? Br J Haematol 73 : 276-278, 1989 – reference: 8) Yao M, Cheng AL, Su IJ, et al : Clinicopathologicalspectrum of haemophagocytic syndrome in Epstein-Barr virus-associated peripheral T-cell lymphoma. Br J Haematol 87 : 535-543, 1994 – reference: 12) Esumi N, Ikushima S, Hibi S, et al : High serum ferritin level as a marker of malignant histiocytosis and virus-associated hemophagocytic syndrome. Cancer 61 : 2071-2076, 1988 – reference: 19) Saeki T, Ueda K, Tanigawara Y, et al : Human Pglycoprotein transports cyclosporin A and FK506. J Biol Chem 268 : 6077-6080, 1993 – reference: 1) 今宿晋作 : リンパ系腫瘍に伴う血球貧食症候群におけるサイトカインの関与.臨床医22 : 103-107, 1996 – reference: 2) 今宿晋作, 日比成美 : *Histiocyte SocietyによるHLH-94 (hemophagocytic lymphohistiocytosisの治療に関する国際統一プロトコール) について.日児誌99 : 281, 1995 – reference: 13) Imashuku S, Hibi S, Sako M, et al : Soluble interleukin-2 recepter : a useful prognostic factor for patients with hemophagocytic lymphohistiocytosis. Blood 86 : 4706-4707, 1995 – reference: 15) Watson HG, Goulden NJ, Manson LM, et al : Virusassociated haemophagocytic syndrome : further evidence for a T-cell mediated disorder. Br J Haematol 86 : 213-215, 1994 – reference: 21) Teshima T, Miyaji R, Fukuda M, et al : Bone-marrow transplantation for Epstein-Barr-virus-associated natural killer cell-large granular lymphocyte leukaemia. Lancet 347 : 1124, 1996 – reference: 6) Lee SH, Su IJ, Chen RL, et al : A pathologic study of childhood lymphoma in Taiwan with special reference to peripheral T-cell lymphoma and the association with Epstein-Barr viral infection. Cancer 68 : 1954-1962, 1991 – reference: 11) Hibi S, Ikushima S, Fujiwara F, et al : Serum and urine beta-2-microglobulin in hemophagocytic syndrome. Cancer 75 : 1700-1705, 1995 – reference: 14) Imashuku S, Hibi S, Fujiwara F, et al : Haemophagocytic lymphohistiocytosis, interferon-gammanaemia and Epstein-Barr virus involvement. Br J Haematol 88 : 656-658, 1994 – reference: 20) 今宿晋作 : HPS, 治療抵抗性LCH, EBV関連免疫異常症例に対する同種骨髄移植.日本医事新報3736 : 13-20, 1995 – reference: 5) Falini B, Pileri S, De Solas I, et al : Peripheral T-cell lymphoma associated with hemophagocytic syndrome. Blood 75 : 434-444, 1990 |
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| Snippet | A 4-year-old girl developed Epstein-Barr virus (EBV) -associated peripheral T-cell lymphoma (PTCL), showing hemophagocytic lymphohistiocytosis. Bone marrow... |
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| SubjectTerms | bone marrow transplantation Epstein-Barr virus hemophagocytic lymphohistiocytosis peripheral T-cell lymphoma |
| Title | A Case of Epstein-Barr Virus-Associated Peripheral T-Cell Lymphoma |
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