The Experts below are selected from a list of 267 Experts worldwide ranked by ideXlab platform
Guillermo A Herrera - One of the best experts on this subject based on the ideXlab platform.
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light chain mediated acute tubular interstitial nephritis a poorly recognized pattern of renal disease in patients with Plasma Cell Dyscrasia
Archives of Pathology & Laboratory Medicine, 2009Co-Authors: Xin Gu, Guillermo A HerreraAbstract:Abstract Context.—Acute renal failure may be the first clinical presentation in patients with Plasma Cell Dyscrasia. Recognition of the unusual interstitial inflammatory lesion associated with monoclonal light chains and renal failure described herein is important to guide clinicians in requesting appropriate tests to confirm Plasma Cell Dyscrasia and providing adequate treatment. Objective.—To describe an unusual pattern of tubular interstitial nephritis in patients with underlying Plasma Cell Dyscrasia characterized by an interstitial inflammatory reaction associated with deposition of light chains along tubular basement membranes. Design.—Eight cases of light-chain–mediated acute tubular interstitial nephritis were identified from the archives of 4296 kidney biopsy specimens. In all cases, routine light microscopic examination, direct immunofluorescence, and electron microscopic examination were performed. Ultrastructural immunogold labeling was performed in specimens with inconclusive findings and in ...
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Light-Chain–Mediated Acute Tubular Interstitial Nephritis: A Poorly Recognized Pattern of Renal Disease in Patients With Plasma Cell Dyscrasia
Archives of Pathology & Laboratory Medicine, 2009Co-Authors: Xin Gu, Guillermo A HerreraAbstract:Abstract Context.—Acute renal failure may be the first clinical presentation in patients with Plasma Cell Dyscrasia. Recognition of the unusual interstitial inflammatory lesion associated with monoclonal light chains and renal failure described herein is important to guide clinicians in requesting appropriate tests to confirm Plasma Cell Dyscrasia and providing adequate treatment. Objective.—To describe an unusual pattern of tubular interstitial nephritis in patients with underlying Plasma Cell Dyscrasia characterized by an interstitial inflammatory reaction associated with deposition of light chains along tubular basement membranes. Design.—Eight cases of light-chain–mediated acute tubular interstitial nephritis were identified from the archives of 4296 kidney biopsy specimens. In all cases, routine light microscopic examination, direct immunofluorescence, and electron microscopic examination were performed. Ultrastructural immunogold labeling was performed in specimens with inconclusive findings and in ...
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Plasma Cell Dyscrasia with kappa light chain crystals in proximal tubular Cells a histological immunofluorescent and ultrastructural study
Ultrastructural Pathology, 2006Co-Authors: Gurdip S Sidhu, Xin Gu, Guillermo A Herrera, Rosemary Wieczorek, Olcay Cubukcudimopulo, Thomas KahnAbstract:This is a case report of a 56-year-old man with Plasma Cell Dyscrasia who presented with proximal tubulopathy manifested as kappa light-chain crystal deposition in the proximal convoluted tubular Cells. This was associated with mild Cellular damage. The crystals were seen as negative images with the hematoxylin–eosin and periodic acid–Schiff (PAS) stains. They were identified as kappa light-chains by immunofluorescent imaging and confirmed by immunoelectron microscopy. Ultrastructurally, the crystals appear to be located within lysosomes. No deposits of light-chains were seen elsewhere in the kidney biopsy.
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Light chain crystal deposition as a manifestation of Plasma Cell Dyscrasias: the role of immunoelectron microscopy.
Human Pathology, 2003Co-Authors: Xin Gu, Roberto Barrios, Joiner Cartwright, Ramon L. Font, Luan D. Truong, Guillermo A HerreraAbstract:Light chain crystal deposition disease is a rare and poorly characterized entity that can be confused with a number of different conditions, depending on where the disease process is manifested. The present study explored the role of ultrastructural immunogold labeling in the diagnosis of this condition. Seven cases of light chain crystal deposition (kappa light chain-related) are reported. Immunohistochemistry and immunofluorescence techniques play a rather limited role in the evaluation of these cases, as a result of the inability to detect monoclonal kappa light chains in association with the crystalline structures or high background staining. Ultrastructural labeling is the method of choice to fully characterize these cases. However, surgical pathologists must learn to recognize the findings associated with this condition to avoid misdiagnosis. If the diagnosis is at least suspected, then a complete hematologic workup may identify the underlying Plasma Cell Dyscrasia. It must be emphasized that in some patients the Plasma Cell Dyscrasia does not become clinically manifested until years after the diagnosis of light chain crystal deposition.
Meiyun Fang - One of the best experts on this subject based on the ideXlab platform.
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tempi syndrome erythrocytosis in Plasma Cell Dyscrasia
Clinical Lymphoma Myeloma & Leukemia, 2018Co-Authors: Xianrui Zhang, Meiyun FangAbstract:Abstract TEMPI (telangiectasias, erythrocytosis with elevated erythropoietin, monoclonal gammopathy, perinephric fluid collections, intrapulmonary shunting) syndrome is a newly described clinical entity that is generally considered a Plasma Cell Dyscrasia with multiple system involvement. The etiology and pathophysiology of this condition remains elusive. Nevertheless, clonal Plasma Cells and monoclonal protein appear to be major contributors. The early diagnosis of TEMPI syndrome is essential because therapies targeting the underlying Plasma Cells can lead to a dramatic response. Bortezomib-based chemotherapy, daratumumab monotherapy, and autologous hematopoietic stem Cell transplantation can result in reversal of most manifestations. Nevertheless, the diagnosis of TEMPI syndrome remains a substantial challenge owing to its rarity and the complexity of clinical presentations. TEMPI syndrome is often misdiagnosed as other causes of erythrocytosis, resulting in a delayed diagnosis and further clinical deterioration. The aim of the present review was to present the clinical and biologic features of TEMPI syndrome, highlighting the differential diagnosis and outlining the present understanding of its pathophysiology and treatment.
Xin Gu - One of the best experts on this subject based on the ideXlab platform.
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light chain mediated acute tubular interstitial nephritis a poorly recognized pattern of renal disease in patients with Plasma Cell Dyscrasia
Archives of Pathology & Laboratory Medicine, 2009Co-Authors: Xin Gu, Guillermo A HerreraAbstract:Abstract Context.—Acute renal failure may be the first clinical presentation in patients with Plasma Cell Dyscrasia. Recognition of the unusual interstitial inflammatory lesion associated with monoclonal light chains and renal failure described herein is important to guide clinicians in requesting appropriate tests to confirm Plasma Cell Dyscrasia and providing adequate treatment. Objective.—To describe an unusual pattern of tubular interstitial nephritis in patients with underlying Plasma Cell Dyscrasia characterized by an interstitial inflammatory reaction associated with deposition of light chains along tubular basement membranes. Design.—Eight cases of light-chain–mediated acute tubular interstitial nephritis were identified from the archives of 4296 kidney biopsy specimens. In all cases, routine light microscopic examination, direct immunofluorescence, and electron microscopic examination were performed. Ultrastructural immunogold labeling was performed in specimens with inconclusive findings and in ...
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Light-Chain–Mediated Acute Tubular Interstitial Nephritis: A Poorly Recognized Pattern of Renal Disease in Patients With Plasma Cell Dyscrasia
Archives of Pathology & Laboratory Medicine, 2009Co-Authors: Xin Gu, Guillermo A HerreraAbstract:Abstract Context.—Acute renal failure may be the first clinical presentation in patients with Plasma Cell Dyscrasia. Recognition of the unusual interstitial inflammatory lesion associated with monoclonal light chains and renal failure described herein is important to guide clinicians in requesting appropriate tests to confirm Plasma Cell Dyscrasia and providing adequate treatment. Objective.—To describe an unusual pattern of tubular interstitial nephritis in patients with underlying Plasma Cell Dyscrasia characterized by an interstitial inflammatory reaction associated with deposition of light chains along tubular basement membranes. Design.—Eight cases of light-chain–mediated acute tubular interstitial nephritis were identified from the archives of 4296 kidney biopsy specimens. In all cases, routine light microscopic examination, direct immunofluorescence, and electron microscopic examination were performed. Ultrastructural immunogold labeling was performed in specimens with inconclusive findings and in ...
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Plasma Cell Dyscrasia with kappa light chain crystals in proximal tubular Cells a histological immunofluorescent and ultrastructural study
Ultrastructural Pathology, 2006Co-Authors: Gurdip S Sidhu, Xin Gu, Guillermo A Herrera, Rosemary Wieczorek, Olcay Cubukcudimopulo, Thomas KahnAbstract:This is a case report of a 56-year-old man with Plasma Cell Dyscrasia who presented with proximal tubulopathy manifested as kappa light-chain crystal deposition in the proximal convoluted tubular Cells. This was associated with mild Cellular damage. The crystals were seen as negative images with the hematoxylin–eosin and periodic acid–Schiff (PAS) stains. They were identified as kappa light-chains by immunofluorescent imaging and confirmed by immunoelectron microscopy. Ultrastructurally, the crystals appear to be located within lysosomes. No deposits of light-chains were seen elsewhere in the kidney biopsy.
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Light chain crystal deposition as a manifestation of Plasma Cell Dyscrasias: the role of immunoelectron microscopy.
Human Pathology, 2003Co-Authors: Xin Gu, Roberto Barrios, Joiner Cartwright, Ramon L. Font, Luan D. Truong, Guillermo A HerreraAbstract:Light chain crystal deposition disease is a rare and poorly characterized entity that can be confused with a number of different conditions, depending on where the disease process is manifested. The present study explored the role of ultrastructural immunogold labeling in the diagnosis of this condition. Seven cases of light chain crystal deposition (kappa light chain-related) are reported. Immunohistochemistry and immunofluorescence techniques play a rather limited role in the evaluation of these cases, as a result of the inability to detect monoclonal kappa light chains in association with the crystalline structures or high background staining. Ultrastructural labeling is the method of choice to fully characterize these cases. However, surgical pathologists must learn to recognize the findings associated with this condition to avoid misdiagnosis. If the diagnosis is at least suspected, then a complete hematologic workup may identify the underlying Plasma Cell Dyscrasia. It must be emphasized that in some patients the Plasma Cell Dyscrasia does not become clinically manifested until years after the diagnosis of light chain crystal deposition.
Dong Yeop Shin - One of the best experts on this subject based on the ideXlab platform.
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increased Plasma viscosity in Plasma Cell Dyscrasia and whole blood viscosity in polycythemia vera
Clinical Hemorheology and Microcirculation, 2018Co-Authors: Dong Woo Shin, Ja Yoon Gu, Jae Seol Jung, Dong Yeop ShinAbstract:BACKGROUND: Although hyperviscosity syndrome in Plasma Cell Dyscrasia (PCD) and thrombosis in myeloproliferative neoplasm (MPN) are major causes of morbidity and mortality, blood viscosity measurements are often underutilized. OBJECTIVE: This study aimed to characterize whether whole blood viscosity (WBV) or Plasma viscosity (PV) could be predictive of hyperviscosity syndrome in PCD and could be elevated in subgroups of MPN. METHODS: A total of 75 patients with hematologic diseases: PCD (n = 26), MPN (n = 25) including polycythemia vera (P. vera) and lymphoma (n = 24) were enrolled along with 104 healthy controls. Both WBV and PV were measured using a capillary tube viscometer. Hyperviscosity syndrome was defined as having 2 or more hyperviscosity symptoms. RESULTS: Patients with PCD showed significantly higher PVs at high and low shear rates when compared to healthy controls, especially in those with hyperviscosity syndrome. The sensitivity and specificity of WBV and PV in detecting hyperviscosity syndrome were 28.6% and 94.1%, and 71.4% and 66.7%, respectively. Patients with P. vera exhibited high WBV and RBC counts compared to healthy controls. CONCLUSION: PV is predictive of hyperviscosity syndrome in PCD and WBV is elevated in patients with P. vera. It suggests that hemorheologic disturbances exist in patients with PCD and MPN and that tests of viscosity may be helpful in detecting hemorheological disturbances.
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, J 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.