The Experts below are selected from a list of 7089 Experts worldwide ranked by ideXlab platform
Angela Dispenzieri - One of the best experts on this subject based on the ideXlab platform.
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overview of castleman disease
Blood, 2020Co-Authors: Angela Dispenzieri, David C FajgenbaumAbstract:Castleman disease (CD) describes a group of at least 4 disorders that share a spectrum of characteristic histopathological features but have a wide range of etiologies, presentations, treatments, and outcomes. CD includes unicentric CD (UCD) and multicentric CD (MCD), the latter of which is divided into idiopathic MCD (iMCD), human herpes virus-8 (HHV8)-associated MCD (HHV8-MCD), and polyneuropathy, Organomegaly, endocrinopathy, monoclonal plasma cell disorder, skin changes (POEMS)-associated MCD (POEMS-MCD). iMCD can be further subclassified into iMCD-thrombocytopenia, ascites, reticulin fibrosis, renal dysfunction, Organomegaly (iMCD-TAFRO) or iMCD-not otherwise specified (iMCD-NOS). Advances in diagnosis, classification, pathogenesis, and therapy are substantial since the original description of UCD by Benjamin Castleman in 1954. The advent of effective retroviral therapy and use of rituximab in HHV8-MCD have improved outcomes in HHV8-MCD. Anti-interleukin-6-directed therapies are highly effective in many iMCD patients, but additional therapies are required for refractory cases. Much of the recent progress has been coordinated by the Castleman Disease Collaborative Network (CDCN), and further progress will be made by continued engagement of physicians, scientists, and patients. Progress can also be facilitated by encouraging patients to self-enroll in the CDCN's ACCELERATE natural history registry (#NCT02817997; www.CDCN.org/ACCELERATE).
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peripheral blood stem cell transplant for poems syndrome is associated with high rates of engraftment syndrome
European Journal of Haematology, 2008Co-Authors: Angela Dispenzieri, Suzanne R Hayman, Francis K Buadi, Shaji K Kumar, Dennis A Gastineau, David J. Inwards, David Dingli, Martha Q Lacy, Mark R. Litzow, Michelle A ElliottAbstract:Polyneuropathy, Organomegaly, endocrinopathy, M protein and skin changes (POEMS) syndrome is a devastating syndrome, characterized by peripheral neuropathy, Organomegaly, endocrinopathy, monoclonal plasma cells, skin changes, papilledema, volume overload, sclerotic bone lesions, thrombocytosis and high vascular endothelial growth factor (VEGF). High-dose chemotherapy with autologous peripheral blood stem cell transplantation (ASCT) ultimately yields excellent clinical responses, but there can be considerable peritransplant morbidity. We have treated 30 POEMS patients with ASCT at Mayo Clinic, Rochester. During transplant period, patients had high rates of fever, diarrhea, weight gain and rash (93%, 77%, 53% and 43%, respectively). Only 13% remained outpatient, and median time to discharge from hospital was transplant day 17 (range 0–175). Splenomegaly was the baseline factor that best predicted for a complicated peritransplant course. Depending on the definition used, ∼50% of patients satisfied criteria for engraftment syndrome. Earlier and more aggressive use of corticosteroids may be associated with less complicated post-transplant courses. Median overall survival has not been reached; the treatment-related mortality was 3%. In addition, important clinical improvements and reductions in plasma VEGF levels can occur in the absence of significant decrease in the monoclonal protein. Unraveling the mechanisms of the syndrome both in the context of ASCT and in general are challenges for the future.
Naoto Takahashi - One of the best experts on this subject based on the ideXlab platform.
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two cases of thrombocytopenia anasarca fever reticulin fibrosis renal failure and Organomegaly tafro syndrome with high serum procalcitonin levels including the first case complicated with adrenal hemorrhaging
Internal Medicine, 2017Co-Authors: Mizuho Nara, Atsushi Komatsuda, Fumiko Itoh, Masaya Saitoh, Masaru Togashi, Yoshihiro Kameoka, Hajime Kaga, Hideki Wakui, Naoto TakahashiAbstract:Thrombocytopenia, Anasarca, Fever, Reticulin fibrosis/Renal failure, and Organomegaly (TAFRO) syndrome is a recently described systemic inflammatory disorder characterized by thrombocytopenia, anasarca, fever, reticulin fibrosis/renal failure, and Organomegaly. It has an acute or subacute onset of unknown etiology, although some pathological features resemble those of multicentric Castleman disease. We here report two cases of TAFRO syndrome. The symptoms and pathological findings in these cases met the 2015 diagnostic criteria. Our cases showed high serum procalcitonin levels, suggesting bacterial infection as an onset trigger. In addition, Case 1 is the first case complicated with adrenal hemorrhaging. Case 2 is the second case of tocilizumab-resistant TAFRO syndrome successfully treated with rituximab.
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Two Cases of Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis/Renal Failure, and Organomegaly (TAFRO) Syndrome with High Serum Procalcitonin Levels, Including the First Case Complicated with Adrenal Hemorrhaging.
Internal Medicine, 2017Co-Authors: Mizuho Nara, Atsushi Komatsuda, Fumiko Itoh, Masaya Saitoh, Masaru Togashi, Yoshihiro Kameoka, Hajime Kaga, Hideki Wakui, Naoto TakahashiAbstract:: Thrombocytopenia, Anasarca, Fever, Reticulin fibrosis/Renal failure, and Organomegaly (TAFRO) syndrome is a recently described systemic inflammatory disorder characterized by thrombocytopenia, anasarca, fever, reticulin fibrosis/renal failure, and Organomegaly. It has an acute or subacute onset of unknown etiology, although some pathological features resemble those of multicentric Castleman disease. We here report two cases of TAFRO syndrome. The symptoms and pathological findings in these cases met the 2015 diagnostic criteria. Our cases showed high serum procalcitonin levels, suggesting bacterial infection as an onset trigger. In addition, Case 1 is the first case complicated with adrenal hemorrhaging. Case 2 is the second case of tocilizumab-resistant TAFRO syndrome successfully treated with rituximab.
Jonathan D Trobe - One of the best experts on this subject based on the ideXlab platform.
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optic disc edema cystoid macular edema and elevated vascular endothelial growth factor in a patient with poems syndrome
Journal of Neuro-ophthalmology, 2007Co-Authors: Deborah Y Chong, Grant M Comer, Jonathan D TrobeAbstract:Abstract:A 48-year-old man with polyneuropathy, Organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome had bilateral optic disc edema (ODE), bilateral cystoid macular edema (CME), anasarca, and elevated serum vascular endothelial growth factor (VEGF). This is the firs
Michelle A Elliott - One of the best experts on this subject based on the ideXlab platform.
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peripheral blood stem cell transplant for poems syndrome is associated with high rates of engraftment syndrome
European Journal of Haematology, 2008Co-Authors: Angela Dispenzieri, Suzanne R Hayman, Francis K Buadi, Shaji K Kumar, Dennis A Gastineau, David J. Inwards, David Dingli, Martha Q Lacy, Mark R. Litzow, Michelle A ElliottAbstract:Polyneuropathy, Organomegaly, endocrinopathy, M protein and skin changes (POEMS) syndrome is a devastating syndrome, characterized by peripheral neuropathy, Organomegaly, endocrinopathy, monoclonal plasma cells, skin changes, papilledema, volume overload, sclerotic bone lesions, thrombocytosis and high vascular endothelial growth factor (VEGF). High-dose chemotherapy with autologous peripheral blood stem cell transplantation (ASCT) ultimately yields excellent clinical responses, but there can be considerable peritransplant morbidity. We have treated 30 POEMS patients with ASCT at Mayo Clinic, Rochester. During transplant period, patients had high rates of fever, diarrhea, weight gain and rash (93%, 77%, 53% and 43%, respectively). Only 13% remained outpatient, and median time to discharge from hospital was transplant day 17 (range 0–175). Splenomegaly was the baseline factor that best predicted for a complicated peritransplant course. Depending on the definition used, ∼50% of patients satisfied criteria for engraftment syndrome. Earlier and more aggressive use of corticosteroids may be associated with less complicated post-transplant courses. Median overall survival has not been reached; the treatment-related mortality was 3%. In addition, important clinical improvements and reductions in plasma VEGF levels can occur in the absence of significant decrease in the monoclonal protein. Unraveling the mechanisms of the syndrome both in the context of ASCT and in general are challenges for the future.
Jean-paul Fermand - One of the best experts on this subject based on the ideXlab platform.
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High-dose therapy and autologous blood stem cell transplantation in POEMS syndrome.
Blood, 2002Co-Authors: Arnaud Jaccard, Jean-claude Brouet, Bruno Royer, Dominique Bordessoule, Jean-paul FermandAbstract:We treated 5 patients with polyneuropathy, Organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome and multifocal bone lesions or diffuse bone marrow plasmacytic infiltration with high-dose therapy (HDT) and autologous blood stem cell transplantation. In all cases, the treatment produced remission of plasma cell proliferation associated with marked improvement in the patients' performance status, neurologic symptoms, and other manifestations of the syndrome. HDT with stem cell support should be investigated further as a therapeutic option in patients with POEMS syndrome and disseminated plasma cell dyscrasia.