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Shinsaku Imashuku - One of the best experts on this subject based on the ideXlab platform.

  • Hemophagocytic Syndrome and hepatosplenic gammadelta t cell lymphoma with isochromosome 7q and 8 trisomy
    Journal of Pediatric Hematology Oncology, 2004
    Co-Authors: Motoaki Chin, Shinsaku Imashuku, Hideo Mugishima, Mayumi Takamura, Toshihito Nagata, Hiroyuki Shichino, Toshiaki Shimada, Takashi Suzuki, Kensuke Harada, Shouhei Yokota
    Abstract:

    Abstract:The authors describe a 15-year-old boy with hepatosplenic γδ T-cell lymphoma associated with Hemophagocytic Syndrome (HPS) along with isochromosome 7q and trisomy 8. He presented with prolonged fever, mild anemia, thrombocytopenia, and hepatosplenomegaly. Physical examination, radiography,

  • differential diagnosis of Hemophagocytic Syndrome underlying disorders and selection of the most effective treatment
    International Journal of Hematology, 1997
    Co-Authors: Shinsaku Imashuku
    Abstract:

    Hemophagocytic Syndrome consists of primary and secondary HLH. Efficacy of therapeutic measures and prognosis depend on degree of hypercytokinemia-associated organ failure at disease onset and underlying disorders. The underlying diseases related to hemophagocytosis and informative markers useful for differential diagnoses to select the most effective treatment are discussed. Differential diagnosis is difficult between confirmed FEL and familiality-unknown infantile VAHS (or sporadic FEL cases) in primary HLH and also among IAHS, benign EB-VAHS and EBV-related LAHS in secondary HLH. For primary HLH, assay of NK activity and for secondary HLH, studies on the serum cytokine pattern, EBV genomes and clonality determination might prove useful.

  • differential diagnosis of Hemophagocytic Syndrome underlying disorders and selection of the most effective treatment
    International Journal of Hematology, 1997
    Co-Authors: Shinsaku Imashuku
    Abstract:

    Copyright (c) 1997 Elsevier Science Ireland Ltd. All rights reserved. Hemophagocytic Syndrome consists of primary and secondary HLH. Efficacy of therapeutic measures and prognosis depend on degree of hypercytokinemia-associated organ failure at disease onset and underlying disorders. The underlying diseases related to hemophagocytosis and informative markers useful for differential diagnoses to select the most effective treatment are discussed. Differential diagnosis is difficult between confirmed FEL and familiality-unknown infantile VAHS (or sporadic FEL cases) in primary HLH and also among IAHS, benign EB-VAHS and EBV-related LAHS in secondary HLH. For primary HLH, assay of NK activity and for secondary HLH, studies on the serum cytokine pattern, EBV genomes and clonality determination might prove useful. © 1997 Elsevier Science Ireland Ltd.

Tirtha Raj Koirala - One of the best experts on this subject based on the ideXlab platform.

  • therapeutic trials for a rabbit model of ebv associated Hemophagocytic Syndrome hps effects of vidarabine or chop and development of herpesvirus papio hvp negative lymphomas surrounded by hvp infected lymphoproliferative disease
    Histology and Histopathology, 2003
    Co-Authors: Kazuhiko Hayashi, Zaishun Jin, Sachiyo Onoda, Nobuya Ohara, Wakako Oda, Tirtha Raj Koirala, H Joko, Mitsuru Munemasa, Toshio Tanaka, Takashi Oka
    Abstract:

    Summary. Epstein-Barr virus-associated Hemophagocytic Syndrome (EBV-AHS), which is often associated with fatal infectious mononucleosis or T-cell lymphoproliferative diseases (LPD), is a distinct disease characterized by high mortality. Treatment of patients with EBV-AHS has proved challenging. To develop some therapeutic interventions for EBV-AHS, we examined the effectiveness of an antiviral agent (vidarabine) or chemotherapy (CHOP), using a rabbit model for EBV-AHS. Fourteen untreated rabbits were inoculated intravenously with cell-free virions of the EBV-like virus Herpesvirus papio (HVP). All of the rabbits died of HVP-associated (LPD) and Hemophagocytic Syndrome (HPS) between 21 and 31 days after inoculation. Furthermore, three HVP-infected rabbits treated with vidarabine died between days 23 and 28 after inoculation, and their clinicopathological features were no different from those of untreated rabbits, indicating that this drug is not effective at all to treat HVP-induced rabbit LPD and HPS. Three of the infected rabbits that were treated with one course, with an incomplete set of three courses, or with three full courses of CHOP treatment died of HVPinduced LPD and HPS with a bleeding tendency and/or with opportunistic infections. They died on the 26th, 62nd and 105th day after virus inoculation, respectively. CHOP treatment transiently suppressed the HVPinduced LPD and contributed to the prolonged survival time of two infected rabbits. However, it did not remove all of the HVP-infected cells from the infected rabbits, and residual HVP-infected lymphocytes caused recurrences of rabbit LPD and HPS. The most interesting finding of this experiment was observed in the infected rabbit with the longest survival time of 105 days: HVP-negative lymphomas surrounded by HVPinduced LPD developed in the larynx and ileum of this rabbit, causing an obstruction of the lumen. We concluded that these were not secondary lymphomas caused by CHOP treatment, because no suspicious lesions were detected in three uninfected rabbits that were treated with three courses of CHOP for 120 days. It is therefore necessary to clarify the mechanism by which HVP-negative lymphomas associated with HVP-induced LPD can develop. Our data from therapeutic trials using EBV-AHS animal models indicate that vidarabine is not effective as an agent to treat HVP-infected rabbits, and even the cytotoxic chemotherapy of CHOP is not sufficient to cure the HVP-infected rabbits or to prolong the survival time of infected rabbits. Further studies will therefore be required to develop better therapies to treat EBV-AHS.

  • rabbit model for human ebv associated Hemophagocytic Syndrome hps sequential autopsy analysis and characterization of il 2 dependent cell lines established from herpesvirus papio induced fatal rabbit lymphoproliferative diseases with hps
    American Journal of Pathology, 2003
    Co-Authors: Kazuhiko Hayashi, Zaishun Jin, Sachiyo Onoda, Hiromasa Joko, Norihiro Teramoto, Nobuya Ohara, Wakako Oda, Takehiro Tanaka, Yi Xuan Liu, Tirtha Raj Koirala
    Abstract:

    Epstein-Barr virus-associated Hemophagocytic Syndrome (EBV-AHS) is often associated with fatal infectious mononucleosis or T-cell lymphoproliferative diseases (LPD). To elucidate the true nature of fatal LPD observed in Herpesvirus papio (HVP)-induced rabbit hemophagocytosis, reactive or neoplastic, we analyzed sequential development of HVP-induced rabbit LPD and their cell lines. All of the seven Japanese White rabbits inoculated intravenously with HVP died of fatal LPD 18 to 27 days after inoculation. LPD was also accompanied by Hemophagocytic Syndrome (HPS) in five of these seven rabbits. Sequential autopsy revealed splenomegaly and swollen lymph nodes, often accompanied by bleeding, which developed in the last week. Atypical lymphoid cells infiltrated many organs with a “starry sky” pattern, frequently involving the spleen, lymph nodes, and liver. HVP-small RNA-1 expression in these lymphoid cells was clearly demonstrated by a newly developed in situ hybridization (ISH) system. HVP-ISH of immunomagnetically purified lymphoid cells from spleen or lymph nodes revealed HVP-EBER1+ cells in each CD4+, CD8+, or CD79a+ fraction. Hemophagocytic histiocytosis was observed in the lymph nodes, spleen, bone marrow, and thymus. HVP-DNA was detected in the tissues and peripheral blood from the infected rabbits by PCR or Southern blot analysis. Clonality analysis of HVP-induced LPD by Southern blotting with TCR gene probe revealed polyclonal bands, suggesting polyclonal proliferation. Six IL-2-dependent rabbit T-cell lines were established from transplanted scid mouse tumors from LPD. These showed latency type I/II HVP infection and had normal karyotypes except for one line, and three of them showed tumorigenicity in nude mice. These data suggest that HVP-induced fatal LPD in rabbits is reactive polyclonally in nature.

Kazuhiko Hayashi - One of the best experts on this subject based on the ideXlab platform.

  • therapeutic trials for a rabbit model of ebv associated Hemophagocytic Syndrome hps effects of vidarabine or chop and development of herpesvirus papio hvp negative lymphomas surrounded by hvp infected lymphoproliferative disease
    Histology and Histopathology, 2003
    Co-Authors: Kazuhiko Hayashi, Zaishun Jin, Sachiyo Onoda, Nobuya Ohara, Wakako Oda, Tirtha Raj Koirala, H Joko, Mitsuru Munemasa, Toshio Tanaka, Takashi Oka
    Abstract:

    Summary. Epstein-Barr virus-associated Hemophagocytic Syndrome (EBV-AHS), which is often associated with fatal infectious mononucleosis or T-cell lymphoproliferative diseases (LPD), is a distinct disease characterized by high mortality. Treatment of patients with EBV-AHS has proved challenging. To develop some therapeutic interventions for EBV-AHS, we examined the effectiveness of an antiviral agent (vidarabine) or chemotherapy (CHOP), using a rabbit model for EBV-AHS. Fourteen untreated rabbits were inoculated intravenously with cell-free virions of the EBV-like virus Herpesvirus papio (HVP). All of the rabbits died of HVP-associated (LPD) and Hemophagocytic Syndrome (HPS) between 21 and 31 days after inoculation. Furthermore, three HVP-infected rabbits treated with vidarabine died between days 23 and 28 after inoculation, and their clinicopathological features were no different from those of untreated rabbits, indicating that this drug is not effective at all to treat HVP-induced rabbit LPD and HPS. Three of the infected rabbits that were treated with one course, with an incomplete set of three courses, or with three full courses of CHOP treatment died of HVPinduced LPD and HPS with a bleeding tendency and/or with opportunistic infections. They died on the 26th, 62nd and 105th day after virus inoculation, respectively. CHOP treatment transiently suppressed the HVPinduced LPD and contributed to the prolonged survival time of two infected rabbits. However, it did not remove all of the HVP-infected cells from the infected rabbits, and residual HVP-infected lymphocytes caused recurrences of rabbit LPD and HPS. The most interesting finding of this experiment was observed in the infected rabbit with the longest survival time of 105 days: HVP-negative lymphomas surrounded by HVPinduced LPD developed in the larynx and ileum of this rabbit, causing an obstruction of the lumen. We concluded that these were not secondary lymphomas caused by CHOP treatment, because no suspicious lesions were detected in three uninfected rabbits that were treated with three courses of CHOP for 120 days. It is therefore necessary to clarify the mechanism by which HVP-negative lymphomas associated with HVP-induced LPD can develop. Our data from therapeutic trials using EBV-AHS animal models indicate that vidarabine is not effective as an agent to treat HVP-infected rabbits, and even the cytotoxic chemotherapy of CHOP is not sufficient to cure the HVP-infected rabbits or to prolong the survival time of infected rabbits. Further studies will therefore be required to develop better therapies to treat EBV-AHS.

  • rabbit model for human ebv associated Hemophagocytic Syndrome hps sequential autopsy analysis and characterization of il 2 dependent cell lines established from herpesvirus papio induced fatal rabbit lymphoproliferative diseases with hps
    American Journal of Pathology, 2003
    Co-Authors: Kazuhiko Hayashi, Zaishun Jin, Sachiyo Onoda, Hiromasa Joko, Norihiro Teramoto, Nobuya Ohara, Wakako Oda, Takehiro Tanaka, Yi Xuan Liu, Tirtha Raj Koirala
    Abstract:

    Epstein-Barr virus-associated Hemophagocytic Syndrome (EBV-AHS) is often associated with fatal infectious mononucleosis or T-cell lymphoproliferative diseases (LPD). To elucidate the true nature of fatal LPD observed in Herpesvirus papio (HVP)-induced rabbit hemophagocytosis, reactive or neoplastic, we analyzed sequential development of HVP-induced rabbit LPD and their cell lines. All of the seven Japanese White rabbits inoculated intravenously with HVP died of fatal LPD 18 to 27 days after inoculation. LPD was also accompanied by Hemophagocytic Syndrome (HPS) in five of these seven rabbits. Sequential autopsy revealed splenomegaly and swollen lymph nodes, often accompanied by bleeding, which developed in the last week. Atypical lymphoid cells infiltrated many organs with a “starry sky” pattern, frequently involving the spleen, lymph nodes, and liver. HVP-small RNA-1 expression in these lymphoid cells was clearly demonstrated by a newly developed in situ hybridization (ISH) system. HVP-ISH of immunomagnetically purified lymphoid cells from spleen or lymph nodes revealed HVP-EBER1+ cells in each CD4+, CD8+, or CD79a+ fraction. Hemophagocytic histiocytosis was observed in the lymph nodes, spleen, bone marrow, and thymus. HVP-DNA was detected in the tissues and peripheral blood from the infected rabbits by PCR or Southern blot analysis. Clonality analysis of HVP-induced LPD by Southern blotting with TCR gene probe revealed polyclonal bands, suggesting polyclonal proliferation. Six IL-2-dependent rabbit T-cell lines were established from transplanted scid mouse tumors from LPD. These showed latency type I/II HVP infection and had normal karyotypes except for one line, and three of them showed tumorigenicity in nude mice. These data suggest that HVP-induced fatal LPD in rabbits is reactive polyclonally in nature.

Yasunobu Abe - One of the best experts on this subject based on the ideXlab platform.

  • primary mediastinal non seminomatous germ cell tumor associated with Hemophagocytic Syndrome
    Journal of Clinical and Experimental Hematopathology, 2009
    Co-Authors: Eriko Sada, Motoaki Shiratsuchi, Junnichi Kiyasu, Kensaku Idutsu, Rie Ohtsuka, Eriko Nagasawa, Kennosuke Karube, Ryoichi Takayanagi, Yasunobu Abe
    Abstract:

    A 20-year-old man with a primary non-seminomatous mediastinal germ cell tumor (yolk sac tumor and immature teratoma) developed Hemophagocytic Syndrome (HPS) three months after surgical resection. Around the same time, the patient was found to have bone metastases of the germ cell tumor. No other hereditary or acquired diseases related to HPS were found. The thrombocytopenia was refractory to corticosteroid therapy but improved after chemotherapy performed for germ cell tumor progression. Only three cases of germ cell tumor associated with reactive hemophagocytosis have been previously reported. Successful treatment of the present case by chemotherapy for HPS suggests a close relationship between this rare complication and germ cell tumor.

Hirokazu Nagawa - One of the best experts on this subject based on the ideXlab platform.

  • Hemophagocytic Syndrome caused by fulminant ulcerative colitis and cytomegalovirus infection report of a case
    Diseases of The Colon & Rectum, 2004
    Co-Authors: Shinichiro Koketsu, Toshiaki Watanabe, Nobukazu Hori, Naoyuki Umetani, Yutaka Takazawa, Hirokazu Nagawa
    Abstract:

    We report a case of Hemophagocytic Syndrome that developed in a 35-year-old, Japanese male with fulminant ulcerative colitis. The patient underwent an emergency operation, consisting of subtotal colectomy, ileostomy with rectal preservation (suprapubic mucous fistula). After the operation, peripheral blood counts showed progressive pancytopenia and bone marrow aspirate smears revealed hypocellular bone marrow with an increase in histiocytes, indicating Hemophagocytic Syndrome. Viral studies (serum antibody titer and antigenemia of cytomegalovirus) revealed systemic cytomegalovirus infection. The patient was diagnosed with virus-associated Hemophagocytic Syndrome and was successfully treated with antiviral therapy consisting of intravenous ganciclovir, gamma globulin, and granulocyte-colony stimulating factor.