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Nicholas J Talley - One of the best experts on this subject based on the ideXlab platform.
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Plasma Cell dyscrasia with polyneuropathy the spectrum of poems syndrome
The New England Journal of Medicine, 1992Co-Authors: G D Miralles, Judith R Ofallon, Nicholas J TalleyAbstract:BACKGROUND: The POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome and osteosclerotic myeloma (polyneuropathy and sclerotic bone lesions) may both be manifestations of Plasma-Cell dyscrasia, but the interrelation of these diseases is not clear. We therefore set out to define the clinical spectrum of disease in patients with Plasma-Cell dyscrasia and polyneuropathy who have the complete or incomplete form of the POEMS syndrome or osteosclerotic myeloma. METHODS: Among 2714 patients with Plasma-Cell dyscrasia who were identified between 1973 and 1989, we reviewed the cases of those with polyneuropathy and Plasma-Cell dyscrasia who fulfilled the criteria for the POEMS syndrome or osteosclerotic myeloma. RESULTS: Thirty-eight patients (1.4 percent) with a median age of 51 years were identified, 22 of whom were male. By definition, all had polyneuropathy (37 combined sensorimotor, and 1 primarily motor). Other findings included osteosclerotic bone lesions (82 percent), skin abnormalities (58 percent), lymphadenopathy (42 percent), papilledema (37 percent), peripheral edema (29 percent), hepatomegaly (24 percent), splenomegaly (21 percent), and ascites (11 percent). Thirty-three patients (87 percent) had an abnormal M protein in serum or urine (17 had IgA lambda, and 12 IgG lambda). Five patients fulfilled all the criteria for the POEMS syndrome. The estimated five-year survival in the 38 patients was 60 percent, which was significantly better than the 20 percent survival in 869 patients with multiple myeloma (P < 0.05). The clinical course was similar among the patients with the complete form of the POEMS syndrome and those with the incomplete form. CONCLUSIONS: Plasma-Cell dyscrasia with polyneuropathy is a rare multisystem disease that often presents with osteosclerotic bone lesions. The differentiation of the POEMS syndrome from so-called osteosclerotic myeloma with peripheral neuropathy appears to have no clinical value.
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Plasma Cell dyscrasia with polyneuropathy the spectrum of poems syndrome
The New England Journal of Medicine, 1992Co-Authors: G D Miralles, Judith R Ofallon, Nicholas J TalleyAbstract:Abstract Background. The POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome and osteosclerotic myeloma (polyneuropathy and sclerotic bone lesions) may both be manifestations of Plasma-Cell dyscrasia, but the interrelation of these diseases is not clear. We therefore set out to define the clinical spectrum of disease in patients with Plasma-Cell dyscrasia and polyneuropathy who have the complete or incomplete form of the POEMS syndrome or osteosclerotic myeloma. Methods. Among 2714 patients with Plasma-Cell dyscrasia who were identified between 1973 and 1989, we reviewed the cases of those with polyneuropathy and Plasma-Cell dyscrasia who fulfilled the criteria for the POEMS syndrome or osteosclerotic myeloma. Results. Thirty-eight patients (1.4 percent) with a median age of 51 years were identified, 22 of whom were male. By definition, all had polyneuropathy (37 combined sensorimotor, and 1 primarily motor). Other findings included osteosclerotic bone le...
Rafael Fonseca - One of the best experts on this subject based on the ideXlab platform.
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Plasma Cell leukemia
Blood Reviews, 2011Co-Authors: Flavio Albarracin, Rafael FonsecaAbstract:Plasma Cell leukemia (PCL) is a rare, yet aggressive Plasma Cell (PC) neoplasm, variant of multiple myeloma (MM), characterized by high levels of PCs circulating in the peripheral blood. PCL can either originate de novo (primary PCL) or as a secondary leukemic transformation of MM (secondary PCL). Presenting signs and symptoms are similar to those seen in MM such as renal insufficiency, hypercalcemia, lytic bone lesions, anemia, and thrombocytopenia, but can also include hepatomegaly and splenomegaly. The diagnostic evaluation of a patient with suspected PCL should include a review of the peripheral blood smear, bone marrow aspiration and biopsy, serum protein electrophoresis (SPEP) with immunofixation, and protein electrophoresis of an aliquot from a 24h urine collection (UPEP). The diagnosis is made when a monoclonal population of PCs is present in the peripheral blood with an absolute PC count exceeding 2000/μL and PC comprising 20% or more of the peripheral blood white Cells. The prognosis of PCL is poor with a median survival of 7 to 11 months. Survival is even shorter (2 to 7 months) when PCL occurs in the context of refractory or relapsing MM. There have been no prospective randomized trials investigating the treatment of PCL. Recommendations are primarily based upon data from small retrospective series, case reports, and extrapolation of data from patients with MM. In general, patients are treated with induction therapy followed by hematopoietic Cell transplantation (HCT) in those who are appropriate candidates for this approach. The best induction regimen for PCL is not known and there is great variability in clinical practice. Newer agents that are being incorporated into frontline and salvage therapy for MM have also demonstrated activity in PCL such as Immunomodulatory agents and the use of bortezomib with different combinations.
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Plasma Cell leukemia
Current Treatment Options in Oncology, 2001Co-Authors: Suzanne R Hayman, Rafael FonsecaAbstract:Plasma Cell leukemia (PCL) is a rare aggressive variant of multiple myeloma (MM) characterized by a fulminant course and poor prognosis. The median survival is measured in months. Therapy and prognosis partially depend on whether the disease presents de novo or as a secondary process involving the leukemic transformation of a previously diagnosed MM. Secondary PCL represents a terminal event for refractory/relapsed MM and is usually not responsive to any treatment modality. The optimal regimens for the treatment of primary PCL have not been firmly established. Induction with combination chemotherapy, followed by high-dose chemotherapy (preferably within the setting of a clinical trial), is the current recommended approach for eligible patients.
Tamotsu Matsuda - One of the best experts on this subject based on the ideXlab platform.
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pulmonary Plasma Cell granuloma improves with corticosteroid therapy
Chest, 1994Co-Authors: Takurna Bando, Masaki Fujimura, Yatsugi Noda, Jinichiro Hirose, Goroku Ohta, Tamotsu MatsudaAbstract:Two cases of pulmonary Plasma Cell granuloma that progressed after respiratory infectious disease are described. The men, 48 and 32 years old, were admitted to the hospital with blood-streaked sputum and mass or nodular shadow on chest radiograph. Close examination revealed that these tumors were Plasma Cell granulomas, which are known as postinflammatory pseudotumors. Biopsy specimens, obtained by way of transbronchial biopsy, demonstrated proliferation of mature Plasma Cells and infiltration of lymphocytes supported by granulation tissue, and there was no evidence of malignancy or tuberculosis. Although surgery is common in the treatment of pulmonary Plasma Cell granuloma, some cases relapse or invade the mediastinum. Therefore, we decided to treat these patients with prednisolone, 30 mg/d, an anti-inflammatory and immunosuppressive agent. Two or 4 weeks later, these tumors disappeared completely and they have never recurred. As middle-dosage corticosteroid therapy is not cytotoxic, it is useful for the treatment of pulmonary Plasma Cell granuloma, especially in multifocal, unresectable, and/or relapsing cases.
G D Miralles - One of the best experts on this subject based on the ideXlab platform.
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Plasma Cell dyscrasia with polyneuropathy the spectrum of poems syndrome
The New England Journal of Medicine, 1992Co-Authors: G D Miralles, Judith R Ofallon, Nicholas J TalleyAbstract:BACKGROUND: The POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome and osteosclerotic myeloma (polyneuropathy and sclerotic bone lesions) may both be manifestations of Plasma-Cell dyscrasia, but the interrelation of these diseases is not clear. We therefore set out to define the clinical spectrum of disease in patients with Plasma-Cell dyscrasia and polyneuropathy who have the complete or incomplete form of the POEMS syndrome or osteosclerotic myeloma. METHODS: Among 2714 patients with Plasma-Cell dyscrasia who were identified between 1973 and 1989, we reviewed the cases of those with polyneuropathy and Plasma-Cell dyscrasia who fulfilled the criteria for the POEMS syndrome or osteosclerotic myeloma. RESULTS: Thirty-eight patients (1.4 percent) with a median age of 51 years were identified, 22 of whom were male. By definition, all had polyneuropathy (37 combined sensorimotor, and 1 primarily motor). Other findings included osteosclerotic bone lesions (82 percent), skin abnormalities (58 percent), lymphadenopathy (42 percent), papilledema (37 percent), peripheral edema (29 percent), hepatomegaly (24 percent), splenomegaly (21 percent), and ascites (11 percent). Thirty-three patients (87 percent) had an abnormal M protein in serum or urine (17 had IgA lambda, and 12 IgG lambda). Five patients fulfilled all the criteria for the POEMS syndrome. The estimated five-year survival in the 38 patients was 60 percent, which was significantly better than the 20 percent survival in 869 patients with multiple myeloma (P < 0.05). The clinical course was similar among the patients with the complete form of the POEMS syndrome and those with the incomplete form. CONCLUSIONS: Plasma-Cell dyscrasia with polyneuropathy is a rare multisystem disease that often presents with osteosclerotic bone lesions. The differentiation of the POEMS syndrome from so-called osteosclerotic myeloma with peripheral neuropathy appears to have no clinical value.
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Plasma Cell dyscrasia with polyneuropathy the spectrum of poems syndrome
The New England Journal of Medicine, 1992Co-Authors: G D Miralles, Judith R Ofallon, Nicholas J TalleyAbstract:Abstract Background. The POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome and osteosclerotic myeloma (polyneuropathy and sclerotic bone lesions) may both be manifestations of Plasma-Cell dyscrasia, but the interrelation of these diseases is not clear. We therefore set out to define the clinical spectrum of disease in patients with Plasma-Cell dyscrasia and polyneuropathy who have the complete or incomplete form of the POEMS syndrome or osteosclerotic myeloma. Methods. Among 2714 patients with Plasma-Cell dyscrasia who were identified between 1973 and 1989, we reviewed the cases of those with polyneuropathy and Plasma-Cell dyscrasia who fulfilled the criteria for the POEMS syndrome or osteosclerotic myeloma. Results. Thirty-eight patients (1.4 percent) with a median age of 51 years were identified, 22 of whom were male. By definition, all had polyneuropathy (37 combined sensorimotor, and 1 primarily motor). Other findings included osteosclerotic bone le...
Chan Ii Park - One of the best experts on this subject based on the ideXlab platform.
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Plasma Cell granuloma of the temporal bone a case report
Head and Neck-journal for The Sciences and Specialties of The Head and Neck, 1994Co-Authors: Boo Hyun Nam, Ki Sang Rha, Jang Yuorl Yoo, Chan Ii ParkAbstract:Background. Plasma Cell granuloma is a rare benign lesion which is found most frequently in the lungs, and a few cases have been described in the head and neck. In the middle ear and mastoid, a case of Plasma Cell granuloma was reported by Benton et al. Methods and Results. A 24-year-old woman was seen with a 7-month history of otalgia and decreased hearing. A computed tomographic (CT) scan showed a soft tissue mass occupying most of the mastoid bone. The mass and the contiguous dura were enhanced homogeneously on magnetic resonance imaging (MRI) scan. Microscopic examination showed Plasma Cell aggregates mixed with other inflammatory Cells and Russell's bodies in a fibrous stroma. Immunoperoxidase studies revealed intracytoplasmic kappa and lambda light chains, and the lesion was confirmed as nonneoplastic and of polyclonal origin (ie, Plasma Cell granuloma). The patient was treated with conservative surgical excision (a canal-down mastoidectomy) and postoperative radiotherapy (5,040 cGy in 28 fractions) and remains free of disease 1 year after treatment. Conclusions. A case of Plasma Cell granuloma is reported, and we believe this is the second case report of Plasma Cell granuloma affecting the temporal bone. © 1994 John Wiley & Sons, Inc.