The Experts below are selected from a list of 291 Experts worldwide ranked by ideXlab platform

Takamichi Hattori - One of the best experts on this subject based on the ideXlab platform.

  • Generalized myositis mimicking polymyositis associated with chronic active Epstein-Barr Virus Infection
    Journal of Neurology, 2005
    Co-Authors: Tomoyuki Uchiyama, Kimito Arai, Takako Yamamoto-tabata, Kouji Kishimoto, Yoshiko Nakamura, Kanji Hirai†, Takamichi Hattori
    Abstract:

    Background Chronic generalized myositis has not so far been reported as a complication of chronic active Epstein–Barr Virus Infection (CAEBV). We encountered three patients with chronic generalized myositis mimicking polymyositis associated with CAEBV. Methods To clarify the pathological character of this myositis, we investigated the distribution, clonality, and the immunophenotype of EBV–infected cells and lymphocytes infiltrating in muscles. Results Clinically, two patients showed symmetrical proximal weakness and muscle atrophy as the initial and main symptom. Although the condition resembled polymyositis, they had also lingual and/or orbital myositis. The other patient showed generalized myositis at the late phase of CAEBV. In all of them, immunotherapy was ineffective and prognosis was poor. Intramuscular infiltrating lymphocytes in our patients were mainly CD45RO+, CD3+, CD4–, CD8–, TCR βF1–, TCR δTCS1–, CD56–, CD79a–, CD21–, HLA-DR+, ZEBRA –, LMP1–, and EBER+ T cells. Oligoclonal expansion of EBV–infected T cells was shown in the muscles. However, there were no malignant lymphocytes. Conclusions This new form of myositis must be distinguished from polymyositis and the other conventional forms of myositis. Careful investigation of hidden CAEBV is recommended when patients present with steroid non–responsive chronic progressive generalized myositis, in particular, with lingual or orbital involvement.

  • Generalized myositis mimicking polymyositis associated with chronic active Epstein-Barr Virus Infection.
    Journal of Neurology, 2005
    Co-Authors: Tomoyuki Uchiyama, Kimito Arai, Takako Yamamoto-tabata, Kanji Hirai, Kouji Kishimoto, Yoshiko Nakamura, Takamichi Hattori
    Abstract:

    Background Chronic generalized myositis has not so far been reported as a complication of chronic active Epstein–Barr Virus Infection (CAEBV). We encountered three patients with chronic generalized myositis mimicking polymyositis associated with CAEBV.

Keisei Kawa - One of the best experts on this subject based on the ideXlab platform.

  • How we treat chronic active Epstein–Barr Virus Infection
    International Journal of Hematology, 2017
    Co-Authors: Akihisa Sawada, Masami Inoue, Keisei Kawa
    Abstract:

    Chronic active Epstein–Barr Virus Infection (CAEBV) is a prototype of the EBV-associated T- or NK-cell lymphoproliferative diseases, which also include hypersensitivity to mosquito bites and severe-type hydroavacciniforme. The manifestations of CAEBV are often self-limiting with minimum supportive care or only prednisolone and cyclosporine A with or without etoposide. However, allogeneic hematopoietic stem cell transplantation (HSCT) is the only cure, without which patients with CAEBV die within several years. A severe hypercytokinemia and hemophagocytic syndrome, which may occur suddenly, often results in a fatal clinical course. At out institute, we have established a 3-step strategy, including allogeneic HSCT, for the treatment of CAEBV. Seventy-nine patients with CAEBV and related diseases have been treated to date. The 3-year overall survival rate (3y-OS) is currently 87.3 ± 4.2% after planned allogeneic HSCT. However, 3y-OS in patients with uncontrolled active disease is only 16.7 ± 10.8%. To maximize survival rates with minimized late sequelae, we recommend earlier initiation and completion of the 3-step treatment without watchful waiting. We present six illustrative and difficult cases (including severe hypercytokinemia or emergent HSCT) and discuss them together with 73 residual cases.

Tomoyuki Uchiyama - One of the best experts on this subject based on the ideXlab platform.

  • Generalized myositis mimicking polymyositis associated with chronic active Epstein-Barr Virus Infection
    Journal of Neurology, 2005
    Co-Authors: Tomoyuki Uchiyama, Kimito Arai, Takako Yamamoto-tabata, Kouji Kishimoto, Yoshiko Nakamura, Kanji Hirai†, Takamichi Hattori
    Abstract:

    Background Chronic generalized myositis has not so far been reported as a complication of chronic active Epstein–Barr Virus Infection (CAEBV). We encountered three patients with chronic generalized myositis mimicking polymyositis associated with CAEBV. Methods To clarify the pathological character of this myositis, we investigated the distribution, clonality, and the immunophenotype of EBV–infected cells and lymphocytes infiltrating in muscles. Results Clinically, two patients showed symmetrical proximal weakness and muscle atrophy as the initial and main symptom. Although the condition resembled polymyositis, they had also lingual and/or orbital myositis. The other patient showed generalized myositis at the late phase of CAEBV. In all of them, immunotherapy was ineffective and prognosis was poor. Intramuscular infiltrating lymphocytes in our patients were mainly CD45RO+, CD3+, CD4–, CD8–, TCR βF1–, TCR δTCS1–, CD56–, CD79a–, CD21–, HLA-DR+, ZEBRA –, LMP1–, and EBER+ T cells. Oligoclonal expansion of EBV–infected T cells was shown in the muscles. However, there were no malignant lymphocytes. Conclusions This new form of myositis must be distinguished from polymyositis and the other conventional forms of myositis. Careful investigation of hidden CAEBV is recommended when patients present with steroid non–responsive chronic progressive generalized myositis, in particular, with lingual or orbital involvement.

  • Generalized myositis mimicking polymyositis associated with chronic active Epstein-Barr Virus Infection.
    Journal of Neurology, 2005
    Co-Authors: Tomoyuki Uchiyama, Kimito Arai, Takako Yamamoto-tabata, Kanji Hirai, Kouji Kishimoto, Yoshiko Nakamura, Takamichi Hattori
    Abstract:

    Background Chronic generalized myositis has not so far been reported as a complication of chronic active Epstein–Barr Virus Infection (CAEBV). We encountered three patients with chronic generalized myositis mimicking polymyositis associated with CAEBV.

Akihisa Sawada - One of the best experts on this subject based on the ideXlab platform.

  • How we treat chronic active Epstein–Barr Virus Infection
    International Journal of Hematology, 2017
    Co-Authors: Akihisa Sawada, Masami Inoue, Keisei Kawa
    Abstract:

    Chronic active Epstein–Barr Virus Infection (CAEBV) is a prototype of the EBV-associated T- or NK-cell lymphoproliferative diseases, which also include hypersensitivity to mosquito bites and severe-type hydroavacciniforme. The manifestations of CAEBV are often self-limiting with minimum supportive care or only prednisolone and cyclosporine A with or without etoposide. However, allogeneic hematopoietic stem cell transplantation (HSCT) is the only cure, without which patients with CAEBV die within several years. A severe hypercytokinemia and hemophagocytic syndrome, which may occur suddenly, often results in a fatal clinical course. At out institute, we have established a 3-step strategy, including allogeneic HSCT, for the treatment of CAEBV. Seventy-nine patients with CAEBV and related diseases have been treated to date. The 3-year overall survival rate (3y-OS) is currently 87.3 ± 4.2% after planned allogeneic HSCT. However, 3y-OS in patients with uncontrolled active disease is only 16.7 ± 10.8%. To maximize survival rates with minimized late sequelae, we recommend earlier initiation and completion of the 3-step treatment without watchful waiting. We present six illustrative and difficult cases (including severe hypercytokinemia or emergent HSCT) and discuss them together with 73 residual cases.

Kimito Arai - One of the best experts on this subject based on the ideXlab platform.

  • Generalized myositis mimicking polymyositis associated with chronic active Epstein-Barr Virus Infection
    Journal of Neurology, 2005
    Co-Authors: Tomoyuki Uchiyama, Kimito Arai, Takako Yamamoto-tabata, Kouji Kishimoto, Yoshiko Nakamura, Kanji Hirai†, Takamichi Hattori
    Abstract:

    Background Chronic generalized myositis has not so far been reported as a complication of chronic active Epstein–Barr Virus Infection (CAEBV). We encountered three patients with chronic generalized myositis mimicking polymyositis associated with CAEBV. Methods To clarify the pathological character of this myositis, we investigated the distribution, clonality, and the immunophenotype of EBV–infected cells and lymphocytes infiltrating in muscles. Results Clinically, two patients showed symmetrical proximal weakness and muscle atrophy as the initial and main symptom. Although the condition resembled polymyositis, they had also lingual and/or orbital myositis. The other patient showed generalized myositis at the late phase of CAEBV. In all of them, immunotherapy was ineffective and prognosis was poor. Intramuscular infiltrating lymphocytes in our patients were mainly CD45RO+, CD3+, CD4–, CD8–, TCR βF1–, TCR δTCS1–, CD56–, CD79a–, CD21–, HLA-DR+, ZEBRA –, LMP1–, and EBER+ T cells. Oligoclonal expansion of EBV–infected T cells was shown in the muscles. However, there were no malignant lymphocytes. Conclusions This new form of myositis must be distinguished from polymyositis and the other conventional forms of myositis. Careful investigation of hidden CAEBV is recommended when patients present with steroid non–responsive chronic progressive generalized myositis, in particular, with lingual or orbital involvement.

  • Generalized myositis mimicking polymyositis associated with chronic active Epstein-Barr Virus Infection.
    Journal of Neurology, 2005
    Co-Authors: Tomoyuki Uchiyama, Kimito Arai, Takako Yamamoto-tabata, Kanji Hirai, Kouji Kishimoto, Yoshiko Nakamura, Takamichi Hattori
    Abstract:

    Background Chronic generalized myositis has not so far been reported as a complication of chronic active Epstein–Barr Virus Infection (CAEBV). We encountered three patients with chronic generalized myositis mimicking polymyositis associated with CAEBV.