The Experts below are selected from a list of 321 Experts worldwide ranked by ideXlab platform
Noriko Iwaki - One of the best experts on this subject based on the ideXlab platform.
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clinicopathologic analysis of tafro syndrome demonstrates a distinct subtype of hhv 8 negative multicentric castleman disease
American Journal of Hematology, 2016Co-Authors: Noriko Iwaki, David C. Fajgenbaum, Mitsuhiro Kawano, Christopher S Nabel, Taro Masunari, Eisei Kondo, Yuka Gion, Isao Yoshida, Hiroshi Moro, Koji NikkuniAbstract:Multicentric Castleman disease (MCD) describes a heterogeneous group of disorders involving systemic inflammation, characteristic lymph node histopathology, and multi-organ dysfunction because of pathologic hypercytokinemia. Whereas Human Herpes Virus-8 (HHV-8) drives the hypercytokinemia in a cohort of immunocompromised patients, the etiology of HHV-8-negative MCD is idiopathic (iMCD). Recently, a limited series of iMCD cases in Japan sharing a constellation of clinical features, including thrombocytopenia (T), Anasarca (A), fever (F), reticulin fibrosis (R), and organomegaly (O) has been described as TAFRO syndrome. Herein, we report clinicopathological findings on 25 patients (14 males and 11 females; 23 Japanese-born and two US-born), the largest TAFRO syndrome case series, including the first report of cases from the USA. The median age of onset was 50 years old (range: 23–72). The frequency of each feature was as follows: thrombocytopenia (21/25), Anasarca (24/25), fever (21/25), organomegaly (25/25), and reticulin fibrosis (13/16). These patients frequently demonstrated abdominal pain, elevated serum alkaline phosphatase levels, and acute kidney failure. Surprisingly, none of the cases demonstrated marked hypergammoglobulinemia, which is frequently reported in iMCD. Lymph node biopsies revealed atrophic germinal centers with enlarged nuclei of endothelial cells and proliferation of endothelial venules in interfollicular zone. 23 of 25 cases were treated initially with corticosteroids; 12 patients responded poorly and required further therapy. Three patients died during the observation period (median: 9 months) because of disease progression or infections. TAFRO syndrome is a unique subtype of iMCD that demonstrates characteristic clinicopathological findings. Further study to clarify prognosis, pathophysiology, and appropriate treatment is needed. Am. J. Hematol. 91:220–226, 2016. © 2015 Wiley Periodicals, Inc.
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Clinicopathologic analysis of TAFRO syndrome demonstrates a distinct subtype of HHV-8-negative multicentric Castleman disease
American Journal of Hematology, 2016Co-Authors: Noriko Iwaki, David C. Fajgenbaum, Mitsuhiro Kawano, Christopher S Nabel, Taro Masunari, Eisei Kondo, Yuka Gion, Isao Yoshida, Hiroshi Moro, Koji NikkuniAbstract:© 2016 Wiley Periodicals, Inc. Multicentric Castleman disease (MCD) describes a heterogeneous group of disorders involving systemic inflammation, characteristic lymph node histopathology, and multi-organ dysfunction because of pathologic hypercytokinemia. Whereas Human Herpes Virus-8 (HHV-8) drives the hypercytokinemia in a cohort of immunocompromised patients, the etiology of HHV-8-negative MCD is idiopathic (iMCD). Recently, a limited series of iMCD cases in Japan sharing a constellation of clinical features, including thrombocytopenia (T), Anasarca (A), fever (F), reticulin fibrosis (R), and organomegaly (O) has been described as TAFRO syndrome. Herein, we report clinicopathological findings on 25 patients (14 males and 11 females; 23 Japanese-born and two US-born), the largest TAFRO syndrome case series, including the first report of cases from the USA. The median age of onset was 50 years old (range: 23-72). The frequency of each feature was as follows: thrombocytopenia (21/25), Anasarca (24/25), fever (21/25), organomegaly (25/25), and reticulin fibrosis (13/16). These patients frequently demonstrated abdominal pain, elevated serum alkaline phosphatase levels, and acute kidney failure. Surprisingly, none of the cases demonstrated marked hypergammoglobulinemia, which is frequently reported in iMCD. Lymph node biopsies revealed atrophic germinal centers with enlarged nuclei of endothelial cells and proliferation of endothelial venules in interfollicular zone. 23 of 25 cases were treated initially with corticosteroids; 12 patients responded poorly and required further therapy. Three patients died during the observation period (median: 9 months) because of disease progression or infections. TAFRO syndrome is a unique subtype of iMCD that demonstrates characteristic clinicopathological findings. Further study to clarify prognosis, pathophysiology, and appropriate treatment is needed.
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atypical hyaline vascular type castleman s disease with thrombocytopenia Anasarca fever and systemic lymphadenopathy
Journal of Clinical and Experimental Hematopathology, 2013Co-Authors: Noriko Iwaki, Eisei Kondo, Yasuharu Sato, Katsuyoshi Takata, Kyotaro Ohno, Mai Takeuchi, Yorihisa Orita, Shinji Nakao, Tadashi YoshinoAbstract:Recently, atypical Castleman’s disease (CD) was reported in Japan. This disease is considered as TAFRO syndrome or nonidiopathic plasmacytic lymphadenopathy (IPL), a constellation of clinical symptoms, namely, thrombocytopenia, Anasarca, fever, reticulin fibrosis, and organomegaly without hyper-g-globulinemia. Histopathologically, this disease is similar to hyaline vascular (HV)-type CD. Here, we present a 43-year-old Japanese woman meeting the clinical criteria of TAFRO syndrome who was successfully treated with combined corticosteroid therapy. She showed a rapidly progressive course of thrombocytopenia, systemic lymphadenopathy, fever, Anasarca, and increase in acute inflammatory proteins without hyper-g-globulinemia. Lymph node biopsy was performed and revealed HV-type CD without human herpes virus 8 infection, which was clinicopathologically compatible with non-IPL. The association of these atypical features with well-known multicentric Castleman’s disease (MCD), namely, HV-type histology with systemic lymphadenopathy, marked thrombocytopenia even with a high level of interleukin-6, and increased acute inflammatory proteins without hyper-g-globulinemia, suggests that TAFRO syndrome as presented in our case is a novel entity, which may have been diagnosed as MCD in the past. To define this novel entity more clearly and to demonstrate its etiology, further nationwide surveys of this syndrome and MCD are needed. 〔J Clin Exp Hematopathol 53(1) : 87-93, 2013〕
John P Cheatham - One of the best experts on this subject based on the ideXlab platform.
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congenital superior vena cava obstruction causing Anasarca and respiratory failure in a newborn successful transcatheter therapy
Catheterization and Cardiovascular Interventions, 2005Co-Authors: Pamela S Ro, Sharon L Hill, John P CheathamAbstract:Superior vena cava (SVC) obstruction is a rare entity in the pediatric population. It usually presents in association with either previous cardiac surgery or external compression from a neoplasm. We present the case of an infant born with congenital SVC obstruction and significant bilateral chylothorax and Anasarca necessitating mechanical ventilation. Successful placement of an intravascular stent led to resolution of the chylothoraces with rapid clinical improvement. © 2005 Wiley-Liss, Inc.
Pamela S Ro - One of the best experts on this subject based on the ideXlab platform.
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congenital superior vena cava obstruction causing Anasarca and respiratory failure in a newborn successful transcatheter therapy
Catheterization and Cardiovascular Interventions, 2005Co-Authors: Pamela S Ro, Sharon L Hill, John P CheathamAbstract:Superior vena cava (SVC) obstruction is a rare entity in the pediatric population. It usually presents in association with either previous cardiac surgery or external compression from a neoplasm. We present the case of an infant born with congenital SVC obstruction and significant bilateral chylothorax and Anasarca necessitating mechanical ventilation. Successful placement of an intravascular stent led to resolution of the chylothoraces with rapid clinical improvement. © 2005 Wiley-Liss, Inc.
Yasuharu Sato - One of the best experts on this subject based on the ideXlab platform.
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TAFRO Syndrome
Hematology Oncology Clinics of North America, 2018Co-Authors: Takuro Igawa, Yasuharu SatoAbstract:TAFRO syndrome is a newly recognized variant of idiopathic multicentric Castleman disease (iMCD) that involves a constellation of syndromes: thrombocytopenia (T), Anasarca (A), fever (F), reticulin fibrosis (R), and organomegaly (O). Thrombocytopenia and severe Anasarca accompanied by relatively low serum immunoglobulin levels are characteristic clinical findings of TAFRO syndrome that are not present in iMCD-not otherwise specified (iMCD-NOS). Lymph node biopsy is recommended to exclude other diseases and to diagnose TAFRO syndrome, which reveals characteristic histopathological findings similar to hyaline vascular-type CD. TAFRO syndrome follows a more aggressive course, compared with iMCD-NOS, and there is no standard treatment.
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atypical hyaline vascular type castleman s disease with thrombocytopenia Anasarca fever and systemic lymphadenopathy
Journal of Clinical and Experimental Hematopathology, 2013Co-Authors: Noriko Iwaki, Eisei Kondo, Yasuharu Sato, Katsuyoshi Takata, Kyotaro Ohno, Mai Takeuchi, Yorihisa Orita, Shinji Nakao, Tadashi YoshinoAbstract:Recently, atypical Castleman’s disease (CD) was reported in Japan. This disease is considered as TAFRO syndrome or nonidiopathic plasmacytic lymphadenopathy (IPL), a constellation of clinical symptoms, namely, thrombocytopenia, Anasarca, fever, reticulin fibrosis, and organomegaly without hyper-g-globulinemia. Histopathologically, this disease is similar to hyaline vascular (HV)-type CD. Here, we present a 43-year-old Japanese woman meeting the clinical criteria of TAFRO syndrome who was successfully treated with combined corticosteroid therapy. She showed a rapidly progressive course of thrombocytopenia, systemic lymphadenopathy, fever, Anasarca, and increase in acute inflammatory proteins without hyper-g-globulinemia. Lymph node biopsy was performed and revealed HV-type CD without human herpes virus 8 infection, which was clinicopathologically compatible with non-IPL. The association of these atypical features with well-known multicentric Castleman’s disease (MCD), namely, HV-type histology with systemic lymphadenopathy, marked thrombocytopenia even with a high level of interleukin-6, and increased acute inflammatory proteins without hyper-g-globulinemia, suggests that TAFRO syndrome as presented in our case is a novel entity, which may have been diagnosed as MCD in the past. To define this novel entity more clearly and to demonstrate its etiology, further nationwide surveys of this syndrome and MCD are needed. 〔J Clin Exp Hematopathol 53(1) : 87-93, 2013〕
Koji Nikkuni - One of the best experts on this subject based on the ideXlab platform.
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clinicopathologic analysis of tafro syndrome demonstrates a distinct subtype of hhv 8 negative multicentric castleman disease
American Journal of Hematology, 2016Co-Authors: Noriko Iwaki, David C. Fajgenbaum, Mitsuhiro Kawano, Christopher S Nabel, Taro Masunari, Eisei Kondo, Yuka Gion, Isao Yoshida, Hiroshi Moro, Koji NikkuniAbstract:Multicentric Castleman disease (MCD) describes a heterogeneous group of disorders involving systemic inflammation, characteristic lymph node histopathology, and multi-organ dysfunction because of pathologic hypercytokinemia. Whereas Human Herpes Virus-8 (HHV-8) drives the hypercytokinemia in a cohort of immunocompromised patients, the etiology of HHV-8-negative MCD is idiopathic (iMCD). Recently, a limited series of iMCD cases in Japan sharing a constellation of clinical features, including thrombocytopenia (T), Anasarca (A), fever (F), reticulin fibrosis (R), and organomegaly (O) has been described as TAFRO syndrome. Herein, we report clinicopathological findings on 25 patients (14 males and 11 females; 23 Japanese-born and two US-born), the largest TAFRO syndrome case series, including the first report of cases from the USA. The median age of onset was 50 years old (range: 23–72). The frequency of each feature was as follows: thrombocytopenia (21/25), Anasarca (24/25), fever (21/25), organomegaly (25/25), and reticulin fibrosis (13/16). These patients frequently demonstrated abdominal pain, elevated serum alkaline phosphatase levels, and acute kidney failure. Surprisingly, none of the cases demonstrated marked hypergammoglobulinemia, which is frequently reported in iMCD. Lymph node biopsies revealed atrophic germinal centers with enlarged nuclei of endothelial cells and proliferation of endothelial venules in interfollicular zone. 23 of 25 cases were treated initially with corticosteroids; 12 patients responded poorly and required further therapy. Three patients died during the observation period (median: 9 months) because of disease progression or infections. TAFRO syndrome is a unique subtype of iMCD that demonstrates characteristic clinicopathological findings. Further study to clarify prognosis, pathophysiology, and appropriate treatment is needed. Am. J. Hematol. 91:220–226, 2016. © 2015 Wiley Periodicals, Inc.
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Clinicopathologic analysis of TAFRO syndrome demonstrates a distinct subtype of HHV-8-negative multicentric Castleman disease
American Journal of Hematology, 2016Co-Authors: Noriko Iwaki, David C. Fajgenbaum, Mitsuhiro Kawano, Christopher S Nabel, Taro Masunari, Eisei Kondo, Yuka Gion, Isao Yoshida, Hiroshi Moro, Koji NikkuniAbstract:© 2016 Wiley Periodicals, Inc. Multicentric Castleman disease (MCD) describes a heterogeneous group of disorders involving systemic inflammation, characteristic lymph node histopathology, and multi-organ dysfunction because of pathologic hypercytokinemia. Whereas Human Herpes Virus-8 (HHV-8) drives the hypercytokinemia in a cohort of immunocompromised patients, the etiology of HHV-8-negative MCD is idiopathic (iMCD). Recently, a limited series of iMCD cases in Japan sharing a constellation of clinical features, including thrombocytopenia (T), Anasarca (A), fever (F), reticulin fibrosis (R), and organomegaly (O) has been described as TAFRO syndrome. Herein, we report clinicopathological findings on 25 patients (14 males and 11 females; 23 Japanese-born and two US-born), the largest TAFRO syndrome case series, including the first report of cases from the USA. The median age of onset was 50 years old (range: 23-72). The frequency of each feature was as follows: thrombocytopenia (21/25), Anasarca (24/25), fever (21/25), organomegaly (25/25), and reticulin fibrosis (13/16). These patients frequently demonstrated abdominal pain, elevated serum alkaline phosphatase levels, and acute kidney failure. Surprisingly, none of the cases demonstrated marked hypergammoglobulinemia, which is frequently reported in iMCD. Lymph node biopsies revealed atrophic germinal centers with enlarged nuclei of endothelial cells and proliferation of endothelial venules in interfollicular zone. 23 of 25 cases were treated initially with corticosteroids; 12 patients responded poorly and required further therapy. Three patients died during the observation period (median: 9 months) because of disease progression or infections. TAFRO syndrome is a unique subtype of iMCD that demonstrates characteristic clinicopathological findings. Further study to clarify prognosis, pathophysiology, and appropriate treatment is needed.
