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Paraproteinemia

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https://www.readbyqxmd.com/read/27775221/light-chain-podocytopathy-mimicking-recurrent-focal-segmental-glomerulosclerosis
#1
M A Khalighi, M P Revelo, J D Abraham, F Shihab, F Ahmed
Kidney injury related to paraproteinemia is common and typically occurs after the fourth decade of life in association with an underlying plasma cell dyscrasia or other lymphoproliferative disease. Kidney transplantation in paraprotein-related kidney disease can be successful in conjunction with treatment of the underlying hematopoietic process; however, when hematologic response to therapy is not achieved, recurrent kidney injury is frequently seen. We describe a young male patient who presented at the age of 23 years with end-stage kidney disease thought to be secondary to focal segmental glomerulosclerosis; this patient ultimately received two kidney allografts...
October 24, 2016: American Journal of Transplantation
https://www.readbyqxmd.com/read/27734522/detection-of-l265p-myd-88-mutation-in-a-series-of-clonal-b-cell-lymphocytosis-of-marginal-zone-origin-cbl-mz
#2
Christina Kalpadakis, Gerassimos A Pangalis, Theodoros P Vassilakopoulos, Maria Roumelioti, Sotirios Sachanas, Penelope Korkolopoulou, Efstathios Koulieris, Maria Moschogiannis, Xanthi Yiakoumis, Pantelis Tsirkinidis, Charalampos Pontikoglou, Dimitra Rondoyianni, Helen A Papadaki, Panayiotidis Panayiotidis, Maria K Angelopoulou
Clonal B-cell lymphocytosis of marginal zone origin (CBL-MZ) is a recently described entity characterized by the presence of clonal B cells in the blood and/or bone marrow (BM) with morphologic and immunophenotypic features consistent with marginal zone derivation in otherwise healthy individuals. CBL-MZ is commonly associated with paraproteinemia, usually immunoglobulin M (IgM), raising diagnostic difficulties from Waldenstrom macroglobulinemia (WM). The aim of the present study was to determine the presence of MYD-88 L265P mutation in a well-characterized series of CBL-MZ to identify cases that may in fact represent WM...
October 13, 2016: Hematological Oncology
https://www.readbyqxmd.com/read/27582537/evaluation-of-in-vitro-effects-of-various-targeted-drugs-on-plasma-cells-and-putative-neoplastic-stem-cells-in-patients-with-multiple-myeloma
#3
Katharina Blatt, Harald Herrmann, Gabriele Stefanzl, Wolfgang R Sperr, Peter Valent
Multiple myeloma (MM) is a malignancy characterized by monoclonal paraproteinemia and tissue plasmocytosis. In advanced MM cytopenia and osteopathy may occur. Although several effective treatment strategies have been developed in recent years, there is still a need to identify new drug targets and to develop more effective therapies for patients with advanced MM. We examined the effects of 15 targeted drugs on growth and survival of primary MM cells and 5 MM cell lines (MM.1S, NCI-H929, OPM-2, RPMI-8226, U-266)...
August 25, 2016: Oncotarget
https://www.readbyqxmd.com/read/27526707/paraprotein-related-kidney-disease-kidney-injury-from-paraproteins-what-determines-the-site-of-injury
#4
Mona Doshi, Amit Lahoti, Farhad R Danesh, Vecihi Batuman, Paul W Sanders
Disorders of plasma and B cells leading to paraproteinemias are associated with a variety of renal diseases. Understanding the mechanisms of injury and associated nephropathies provides a framework that aids clinicians in prompt diagnosis and appropriate adjunctive treatment of these disorders. Glomerular diseases that may be associated with paraproteinemias include amyloid deposition, monoclonal Ig deposition disease, proliferative GN with monoclonal Ig deposits, C3 glomerulopathy caused by alterations in the complement pathway, immunotactoid glomerulopathy, fibrillary GN, and cryoglobulinemia...
August 15, 2016: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/27526706/paraprotein-related-kidney-disease-glomerular-diseases-associated-with-paraproteinemias
#5
Shveta S Motwani, Leal Herlitz, Divya Monga, Kenar D Jhaveri, Albert Q Lam
Paraproteins are monoclonal Igs that accumulate in blood as a result of abnormal excess production. These circulating proteins cause a diversity of kidney disorders that are increasingly being comanaged by nephrologists. In this review, we discuss paraprotein-related diseases that affect the glomerulus. We provide a broad overview of diseases characterized by nonorganized deposits, such as monoclonal Ig deposition disease (MIDD), proliferative GN with monoclonal Ig deposits (PGNMID), and C3 glomerulopathy, as well as those characterized by organized deposits, such as amyloidosis, immunotactoid glomerulopathy, fibrillary GN, and cryoglobulinemic GN, and rarer disorders, such as monoclonal crystalline glomerulopathies, paraprotein-related thrombotic microangiopathies, and membranous-like glomerulopathy with masked IgGκ deposits...
August 15, 2016: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/27504090/nodular-cutaneous-amyloidosis-at-the-temple
#6
Kathrin Schucht, Josef Schröder, Heiko Siegmund, Claudia Grafe, Stephan Schreml
A 52-year-old woman presented with a large partially yellow and erythematous tumor on her right temple. She reported that it had grown over the last 4 years. Regional lymph nodes were impalpable. A punch biopsy showed eosinophilic material in the dermis and subcutis. Immunohistochemistry showed positive staining for kappa and lambda light chains. Electron microscopy showed the typical amyloid fibrils (7-10 nm in diameter). There was no evidence of systemic amyloidosis, paraproteinemia or underlying plasmacytoma...
May 2016: Case Reports in Dermatology
https://www.readbyqxmd.com/read/27436448/treatment-of-necrobiotic-xanthogranuloma-a-systematic-review
#7
D Miguel, J Lukacs, T Illing, P Elsner
Necrobiotic xanthogranuloma (NXG) is an uncommon non-Langerhans cell histiocytosis involving skin and extracutaneous tissues. The lesions are usually asymptomatic and commonly appear in the periorbital area. Paraproteinemia is closely associated with NXG and its pathogenesis remains unclear. NXG prognosis is poor with several treatments showing variable results. Treatment of monoclonal gammopathy with alkylating agents does not necessarily influence the activity of the skin disease and vice versa. The aim of this systematic review is to summarize all reported treatments of necrobiotic xanthogranuloma of the skin, with or without underlying malignant condition and based on articles from the PubMed database using the query 'necrobiotic xanthogranuloma treatment', both in English and German, about 'human' subjects and published between 1980 and 2014, documenting adequate treatment for NXG...
July 19, 2016: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/27424703/fanconi-syndrome-due-to-light-chain-proximal-tubulopathy-in-a-patient-with-multiple-myeloma
#8
Mohit Mathur, Bobby Chacko, Mahesha Vankalakunti, Channappa Patil
Fanconi syndrome (FS) in an adult patient is an unusual finding and it merits thorough evaluation. Paraproteinemias are one of the common etiologies in adult FS and need to be ruled out. Among the various forms of renal involvement in multiple myeloma, light chain proximal tubulopathy (LCPT) is the rarest. Usually, it causes proximal tubular dysfunction which is characterized by intracytoplasmic deposition of crystallized, mostly kappa monoclonal light chains in proximal tubules; however, glomerular crystal deposition is unusual...
July 2016: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/27408378/immunoglobulin-m-flare-seen-in-a-case-of-waldenstrom-s-macroglobulinemia-successfully-managed-by-therapeutic-plasma-exchange
#9
Suvro Sankha Datta, Somnath Mukherjee, Biplabendu Talukder, Prasun Bhattacharya
Therapeutic plasma exchange (TPE) is a conjunctive modality of treatment along with rituximab to decrease paraproteinemia associated with hyperviscosity. Here we narrate our experience in treating a diagnosed case of Waldenstrom's macroglobulinemia in 70 years old male patient with moderate anemia and severe features of hyperviscosity syndrome by serial TPE and rituximab combined with bortezomib. The patient was relieved of his symptoms after initial two TPE procedures performed on alternative day. However he again developed signs and symptoms of the disease within 6 weeks following second TPE and starting of rituximab (375 mg/m(2) weekly for 4 weeks) therapy with bortezomib...
June 2016: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/27329639/lymphoplasmacytic-lymphoma-with-a-non-igm-paraprotein-shows-clinical-and-pathologic-heterogeneity-and-may-harbor-myd88-l265p-mutations
#10
Rebecca L King, Wilson I Gonsalves, Stephen M Ansell, Patricia T Greipp, Lori A Frederick, David S Viswanatha, Rong He, Robert A Kyle, Morie A Gertz, Prashant Kapoor, William G Morice, Matthew T Howard
OBJECTIVES: Lymphoplasmacytic lymphoma (LPL) with non-immunoglobulin M (IgM) paraproteinemia remains poorly understood. The goal of this study was to investigate the clinicopathologic features of LPL in the bone marrow in patients with immunoglobulin G (IgG) or immunoglobulin A (IgA) paraproteins and evaluate MYD88 L265P mutation status to determine the relationship of these cases to Waldenström macroglobulinemia (WM). METHODS: Bone marrows from LPL cases with IgG or IgA paraproteins diagnosed between January 1, 2007, and June 30, 2014, were retrieved from the clinical archive...
June 2016: American Journal of Clinical Pathology
https://www.readbyqxmd.com/read/27293389/nodular-cutaneous-amyloidosis-resembling-a-giant-tumor
#11
Sonja Haverkampf, Katja Evert, Josef Schröder, Stephan Schreml
A 79-year-old man presented with a large tumor on the left side of his head, which had grown over 5 years. Regional lymph nodes were impalpable and computed tomography revealed no signs of bone infiltration. Histology showed that the entire dermis was filled with amorphous eosinophilic material. Immunohistochemistry was negative for cytokeratin, but showed that the dermis and parts of the subcutis were filled with amyloid consisting of immunoglobulin light chains. There were no signs of paraproteinemia or underlying plasmocytoma...
January 2016: Case Reports in Dermatology
https://www.readbyqxmd.com/read/27098634/pseudohyperferremia-in-paraproteinemia
#12
Bin Fu, Tong-Yan Zhang, Ji Xiao, Bo Zhang, Lin Meng
No abstract text is available yet for this article.
2016: Annals of Clinical and Laboratory Science
https://www.readbyqxmd.com/read/27078247/-xanthinuria-type-1-in-a-woman-with-arthralgias-a-combined-clinical-and-molecular-genetic-investigation
#13
Roland Wolfgang Därr, Steffen Lenzner, Thomas Eggermann, Wolfgang Hermann Därr
HISTORY AND CLINICAL PRESENTATION: A 53-year old woman with recurrent polyarthralgias, negative test results in a recent rheumatologic work-up and an unmeasurably low uric acid serum concentration presented for suspected IgM paraproteinemia. INVESTIGATIONS: Physical examination, abdominal ultrasound and routine laboratory test results were unremarkable. Repeat determination confirmed a markedly decreased uric acid (UA) serum concentration. Urinary xanthine and hypoxanthine concentrations were increased by 14-fold and 7...
April 2016: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/26747296/viral-co-infections-and-paraproteins-in-hiv-effect-on-development-of-hematological-malignancies
#14
Erin Jou, Oleg Gligich, Alvita C Y Chan, Diwakar Mohan, Uriel R Felsen, Sabarish Ayyappan, Henny H Billett, Edwin P Hui, Anthony T C Chan, Radha Raghupathy
The role of viral co-infections and paraproteins in the development of hematological malignancies (HMs) in HIV remains unclear. Using our large database of HIV+ patients, we investigated whether co-infection and paraproteinemia increase the risk of HM. Data on demographics, hepatitis B (HBV) and hepatitis C virus (HCV) co-infections, paraproteinemia, HIV characteristics, and biopsy proven malignant hematological disorders for HIV+ patients were collected over a 10-year period in a large urban hospital setting...
March 2016: Annals of Hematology
https://www.readbyqxmd.com/read/26663802/poems-syndrome-with-igg-%C3%AE-iga-%C3%AE%C2%BA-biclonal-gammopathy-and-abnormal-serum-free-light-chain-ratio-a-case-report
#15
Ji Yeon Ham, Jang Soo Suh, Won-Kil Lee, Kyung Eun Song
BACKGROUND: POEMS syndrome is a rare paraneoplastic disorder with atypical plasma cell proliferation. Cases of POEMS syndrome presented with either biclonal gammopathy or an abnormal serum free light chain ratio are considered uncommon. The present authors encountered a case of POEMS syndrome with IgG-λ/IgA-κ biclonal gammopathy with dominant κ free light chain and abnormal serum free light chain ratio. CASE: A 56-year-old man with a history of Castleman disease was suspected with POEMS syndrome and admitted for further evaluation for B-cell proliferative disease to rule out multiple myeloma...
2015: Annals of Clinical and Laboratory Science
https://www.readbyqxmd.com/read/26435658/paraproteinemia-and-central-retinal-vein-occlusion
#16
E I Agorogiannis, V Kotamarthi
No abstract text is available yet for this article.
January 2015: Hippokratia
https://www.readbyqxmd.com/read/26312681/scleredema-associated-with-sj%C3%A3-gren-s-syndrome
#17
João Alves, Tiago Judas, Tiago Ferreira, Diogo Matos, Elvira Bártolo
Scleredema adultorum of Buschke is a rare disorder characterized by diffuse swelling and non-pitting induration of the skin usually involving the face, neck, arms and upper trunk. It has been associated with previous infectious diseases, diabetes, paraproteinemia and, more rarely, malignant neoplasms or autoimmune disorders. We report the case of a 30-year-old man who presented with a 2-year history of scleredema. Further investigation led to the diagnosis of primary Sjögren's syndrome. The association between scleredema and autoimmune disorders has been rarely seen...
May 2015: Anais Brasileiros de Dermatologia
https://www.readbyqxmd.com/read/26299085/lymphoplasmacytic-lymphoma-with-iga-paraproteinemia
#18
Jing Guan, Fang Fang, Zonghong Shao
BACKGROUND: Lymphoplasmacytic lymphoma is a type of B-cell Non-Hodgkin's lymphoma. The monoclonal immunoglobulin of IgA type is rarely seen in the clinical practice. METHODS: We report a patient with anemia, thrombocytopenia, hepatomegaly, and splenomegaly. Serum immuno-electrophoresis, bone marrow morphology, and flow cytometry assays were used for the diagnosis of this patient. RESULTS: The monoclonal gammopathy was detected in the serum protein electrophoresis...
2015: Clinical Laboratory
https://www.readbyqxmd.com/read/26137042/concurrent-chronic-neutrophilic-leukemia-blast-crisis-and-multiple-myeloma-a-case-report-and-literature-review
#19
Jinning Shi, Ying Ni, Jianyong Li, Hairong Qiu, Kourong Miao
The current study presents the case of a 78-year-old male with concurrent chronic neutrophilic leukemia (CNL) and multiple myeloma (MM) who developed acute myeloid leukemia after two years of treatment with hydroxyurea, cyclophosphamide, prednisone and thalidomide. The patient presented with mature neutrophilic leukocytosis, hepatosplenomegaly, a high neutrophil alkaline phosphatase score and an absence of the Philadelphia chromosome or the BCR-ABL fusion gene. A bone marrow aspirate smear and biopsy indicated that the CNL coexisted with a plasma cell neoplasm...
May 2015: Oncology Letters
https://www.readbyqxmd.com/read/26120184/scleromyxedema-an-atypical-case
#20
Emy Thomas, Anisha George, Divya Deodhar, Mary John
Scleromyxedema is a rare, chronic and persistent idiopathic disorder characterized by a generalized papular eruption due to dermal mucin deposition with an increase in dermal collagen. Patients usually have associated paraproteinemia. We describe the case of a 59-year-old gentleman with features of scleromyxedema, who had severe pruritus, scalp involvement, unrestricted mobility and associated peripheral eosinophilia, but no monoclonal gammopathy.
May 2015: Indian Journal of Dermatology
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