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Paraproteinemia

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https://www.readbyqxmd.com/read/28781859/hematuria-as-the-first-sign-of-multiple-myeloma
#1
Mariana Alves, Raul Moreno, Fátima Rodrigues, Anabela Rodrigues, Teresa Fonseca
Patients with multiple myeloma may present with several signs and symptoms, inclusive of hemorrhagic diathesis. This case emphasizes the need to suspect uncommon etiologies for common signs and to be aware of the atypical effects of paraproteinemia.
August 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28761239/sequential-autologous-hematopoietic-stem-cell-transplant-followed-by-renal-transplant-in-multiple-myeloma
#2
D Bhowmik, S Yadav, L Kumar, S Agarwal, S K Agarwal, S Gupta
A 30-year-old female was symptomatic with headache, fatigue, and weakness since October 2011 and was told to have anemia. In January 2012, she was admitted outside with pulmonary edema. Investigations revealed advanced azotemia, anemia, and hypercalcemia. Urine showed 2 + proteins and 30-35 red blood cells. There was no history of oral ulcers, rash, Raynaud's phenomenon, or hemoptysis. She was evaluated for causes of rapidly progressive "renal failure." Hemolytic work-up; antinuclear antibody, double-stranded DNA, and anti-neutrophil cytoplasmic antibody were negative...
July 2017: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/28716288/uric-acid-levels-correlate-with-the-severity-of-diabetic-sensorimotor-polyneuropathy
#3
Alon Abraham, Ari Breiner, Carolina Barnett, Hans D Katzberg, Leif E Lovblom, Bruce A Perkins, Vera Bril
INTRODUCTION: Diabetic sensorimotor polyneuropathy (DSP) is the most frequent complication in patients with diabetes mellitus (DM), and is associated with age, DM duration, and HbA1c levels. In addition, higher uric acid (UA) levels are reported in patients with DSP. OBJECTIVES: To explore whether UA levels correlate with DSP severity. METHODS: We extracted the demographic data, clinical history, neurological and electrophysiological examinations and laboratory findings of 115 patients diagnosed with DSP from January 2012 to December 2015...
August 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28709864/the-role-of-human-natural-killer-1-hnk-1-carbohydrate-in-neuronal-plasticity-and-disease
#4
REVIEW
Jyoji Morise, Hiromu Takematsu, Shogo Oka
BACKGROUND: The human natural killer-1 (HNK-1) carbohydrate, a unique trisaccharide possessing sulfated glucuronic acid in a non-reducing terminus (HSO3-3GlcAß1-3Galß1-4GlcNAc-), is highly expressed in the nervous system and its spatiotemporal expression is strictly regulated. Mice deficient in the gene encoding a key enzyme, GlcAT-P, of the HNK-1 biosynthetic pathway exhibit almost complete disappearance of the HNK-1 epitope in the brain, significant reduction of long-term potentiation, and aberration of spatial learning and memory formation...
July 12, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28571810/predictive-role-of-neutrophil-to-lymphocyte-ratio-and-platelet-to-lymphocyte-ratio-in-normal-tension-glaucoma
#5
Kursat Atalay, Havva Kaldirim Erdogan, Ahmet Kirgiz, Senay Asik Nacaroglu
As a type of primary open-angle glaucoma, normal-tension glaucoma (NTG) is a health problem that manifests itself with low intraocular pressure (IOP) levels and preserves its importance for public health care. In population-based screenings, which take the IOP levels of 21mmHg as a cut-off for the diagnosis of glaucoma, the diagnosis is usually delayed, and the treatment is started in an advanced stage by the referred ophthalmologists. NTG is associated with some autoimmune conditions and systemic diseases, such as paraproteinemia, nocturnal hypotension, and migraine...
June 2017: Medical Hypotheses
https://www.readbyqxmd.com/read/28570324/neurologic-complications-of-lymphoma-leukemia-and-paraproteinemias
#6
Michelle L Mauermann
PURPOSE OF REVIEW: This article reviews the spectrum of neurologic complications associated with lymphoma, leukemia, and paraproteinemic disorders. While leptomeningeal metastasis is the most common complication of lymphoma and leukemia and peripheral neuropathy is the most common complication of paraproteinemic disorders, clinicians need to be familiar with the diverse neurologic complications of these disorders. RECENT FINDINGS: Lymphomatous nervous system involvement can be difficult to diagnose, especially when it is the presenting symptom...
June 2017: Continuum: Lifelong Learning in Neurology
https://www.readbyqxmd.com/read/28509128/a-case-of-rapid-amelioration-of-hepatitis-c-virus-associated-cryoglobulinemic-membranoproliferative-glomerulonephritis-treated-by-interferon-free-directly-acting-antivirals-for-hcv-in-the-absence-of-immunosuppressant
#7
Fumiaki Obata, Taichi Murakami, Junko Miyagi, Sayo Ueda, Taizo Inagaki, Masanori Minato, Hiroyuki Ono, Kenji Nishimura, Eriko Shibata, Masanori Tamaki, Sakiya Yoshimoto, Fumi Kishi, Seiji Kishi, Motokazu Matsuura, Kojiro Nagai, Hideharu Abe, Toshio Doi
Mixed cryoglobulinemic syndrome, which is a systemic vasculitis characterized by the immune complex deposition in small- and medium-sized arteries and most often due to chronic hepatitis C virus (HCV) infection, sometimes clinically manifests as refractory glomerulonephritis or nephritic syndrome. Patients with mixed cryoglobulinemic nephropathy who have a rapidly progressive glomerulonephritis should receive immunosuppressive therapy. After disease stabilization, patients should receive concurrent therapy for the underlying HCV infection...
May 2017: CEN Case Reports
https://www.readbyqxmd.com/read/28490364/sporadic-late-onset-nemaline-myopathy-clinico-pathological-characteristics-and-review-of-76-cases
#8
REVIEW
Lukas J Schnitzler, Tobias Schreckenbach, Aleksandra Nadaj-Pakleza, Werner Stenzel, Elisabeth J Rushing, Philip Van Damme, Andreas Ferbert, Susanne Petri, Christian Hartmann, Antje Bornemann, Andreas Meisel, Jens A Petersen, Thomas Tousseyn, Dietmar R Thal, Jens Reimann, Peter De Jonghe, Jean-Jacques Martin, Peter Y Van den Bergh, Jörg B Schulz, Joachim Weis, Kristl G Claeys
BACKGROUND: Sporadic late-onset nemaline myopathy (SLONM) is a rare, late-onset muscle disorder, characterized by the presence of nemaline rods in muscle fibers. Phenotypic characterization in a large cohort and a comprehensive overview of SLONM are lacking. METHODS: We studied the clinico-pathological features, treatment and outcome in a large cohort of 76 patients with SLONM, comprising 10 new patients and 66 cases derived from a literature meta-analysis (PubMed, 1966-2016), and compared these with 15 reported HIV-associated nemaline myopathy (HIV-NM) cases...
May 11, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28469834/ibrutinib-associated-skin-toxicity-a-case-of-maculopapular-rash-in-a-79-year-old-caucasian-male-patient-with-relapsed-waldenstrom-s-macroglobulinemia-and-review-of-the-literature
#9
Anders Bisgaard Jensen, Birgitte Stausbøl-Grøn, Rikke Riber-Hansen, Francesco d'Amore
Waldenstrom's macroglobulinamia (WM) is a rare malignant lymphoproliferative disorder, characterized by monoclonal IgM paraproteinemia and neoplastic proliferation of malignant lymphoplasmacytoid cells in the bone marrow. Traditionally, WM has been treated with modalities similar to those used in the management of other indolent lymphomas. Just recently, based on impressive clinical trial results in heavily pretreated WM patients, a new Bruton Tyrosine Kinase-inhibitor, Ibrutinib, has been approved for the treatment of this disorder...
March 13, 2017: Dermatology Reports
https://www.readbyqxmd.com/read/28446858/a-case-of-autoimmune-polyglandular-syndrome-ype-2-associated-with-atypical-form-of-scleromyxedema
#10
Oleksander Prylutskyi, Olga Prylutska, Anatoliy Degonskyi, Kseniia Tkachenko
BACKGROUND: Autoimmune polyglandular syndrome type 2 represents an uncommon endocrine disorder composed by Addison's disease with autoimmune thyroid disease (Schmidt's syndrome) and/or type 1 diabetes mellitus. Scleromyxedema is a rare progressive cutaneous mucinosis usually associated with systemic involvement and paraproteinemia. To the best of our knowledge, there is no case report of Schmidt's syndrome associated with scleromyxedema. CASE DETAILS: A 34-year-old woman was admitted to Donetsk Clinical Territorial Medical Association due to acute general weakness, reduced vision, dryness of integuments, memory decline, fatigue, weight loss, rash on the face trunk and extremities...
September 2016: Ethiopian Journal of Health Sciences
https://www.readbyqxmd.com/read/28383205/igm-myeloma-a-multicenter-retrospective-study-of-134-patients
#11
MULTICENTER STUDY
Jorge J Castillo, Artur Jurczyszyn, Lucie Brozova, Edvan Crusoe, Jacek Czepiel, Julio Davila, Angela Dispenzieri, Marion Eveillard, Mark A Fiala, Irene M Ghobrial, Alessandro Gozzetti, Joshua N Gustine, Roman Hajek, Vania Hungria, Jiri Jarkovsky, David Jayabalan, Jacob P Laubach, Barbara Lewicka, Vladimir Maisnar, Elisabet E Manasanch, Philippe Moreau, Elizabeth A Morgan, Hareth Nahi, Ruben Niesvizky, Claudia Paba-Prada, Tomas Pika, Ludek Pour, John L Reagan, Paul G Richardson, Jatin Shah, Ivan Spicka, Ravi Vij, Anna Waszczuk-Gajda, Morie A Gertz
IgM myeloma is a rare hematologic malignancy for which the clinicopathological features and patient outcomes have not been extensively studied. We carried out a multicenter retrospective study in patients with diagnosis of IgM myeloma defined by >10% marrow involvement by monoclonal plasma cells, presence of an IgM monoclonal paraproteinemia of any size, and anemia, renal dysfunction, hypercalcemia, lytic lesions and/or t(11;14) identified by FISH. A total of 134 patients from 20 centers were included in this analysis...
August 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28370513/high-dose-intravenous-immunoglobulin-therapy-for-scleromyxoedema-a-prospective-open-label-clinical-trial-using-an-objective-score-of-clinical-evaluation-system
#12
A Guarneri, M Cioni, F Rongioletti
BACKGROUND: Scleromyxoedema is a primary fibro-mucinosis whose therapy is still challenging. OBJECTIVE: To evaluate the safety and efficacy of high-dose intravenous immunoglobulin (IVIg) for the management of scleromyxoedema prospectively using an objective score. METHODS: In a prospective open-label study, IVIg was administered to eight patients with scleromyxoedema in a dose of 2 g/kg per month. The patients were followed-up to a minimum of 6 months, and their disease activity and response to treatment were assessed using the Physician's Global Assessment of disease severity (PGA) and a modified objective skin scoring system for patients with scleroderma (modified Rodnan score system for scleromyxoedema or mRSSS)...
March 29, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28300892/scleromyxedema-clinical-diagnosis-and-autopsy-findings
#13
Ana Carolina Bulhões Sala, Paulo Rowilson Cunha, Clóvis Antônio Lopes Pinto, Célia Antônia Xavier de Moraes Alves, Ingrid Barreto Paiva, Ana Paula Vieira Araujo
Scleromyxedema is a rare chronic cutaneous mucinosis of unknown etiology. It is characterized by papular eruption and scleroderma with microscopic evidence of mucin deposition, fibroblast proliferation, and fibrosis. Most patients with scleromyxedema have monoclonal gammopathy and systemic manifestations resulting in significant morbidity and mortality. Several types of treatment have been reported with partial or inconsistent responses. Despite showing unpredictable evolution, systemic consequences of scleromyxedema and treatment side effects may result in death...
September 2016: Anais Brasileiros de Dermatologia
https://www.readbyqxmd.com/read/28226184/cd117-kit-is-a-useful-marker-in-the-diagnosis-of-plasmablastic-plasma-cell-myeloma
#14
Etan Marks, Yang Shi, Yanhua Wang
AIMS: Plasmablastic plasma cell myeloma (PPCM) is a rare morphological presentation of multiple myeloma, and can resemble plasmablastic lymphoma (PBL), a human immunodeficiency virus (HIV)-related lymphoid neoplasm, morphologically and immunophenotypically. The aim of this study was to retrospectively search for factors that could help to differentiate these two entities. METHODS AND RESULTS: We used clinical looking glass, a data mining tool, to identify patients with a diagnosis of either PPCM or PBL with a CD117 test performed...
July 2017: Histopathology
https://www.readbyqxmd.com/read/28182355/pure-red-cell-aplasia-associated-with-monoclonal-gammopathy-of-undetermined-significance-and-literature-review
#15
Hyunjung Gu, Woo-In Lee, You La Jeon, So Young Kang, Myeong Hee Kim, Tae Sung Park
BACKGROUND: Pure red cell aplasia (PRCA) is an uncommon disease which involves an almost complete absence of the erythroid lineage in bone marrow (BM) and causes severe anemia. Cases due to monoclonal gammopathy occurring in plasma cell disorder have been infrequently reported. Here we report a case of PRCA associated plasma cell disorder, especially monoclonal gammopathy of undetermined significance (MGUS). METHODS: A 55-year-old male visited the ER due to general weakness...
February 1, 2017: Clinical Laboratory
https://www.readbyqxmd.com/read/28153807/amyloidosis-a-cancer-derived-paraproteinemia-and-kidney-involvement
#16
REVIEW
Jolanta Małyszko, Klaudia Kozłowska, Jacek Stanisław Małyszko
Amyloidosis is the general term describing the extracellular tissue deposition of fibrils composed of low molecular weight subunits of a variety of proteins. There are multiple different human protein precursors of amyloid fibrils. Amyloid deposits are stained using Congo Red and show typical apple-green birefringence in polarized microscopy. Nowadays, a novel technique LMD/MS technique or laser microdissection combined with mass spectrometry help to diagnose amyloidosis. Amyloidosis of the kidney is typically classified as being either one of two types: AL or AA...
March 2017: Advances in Medical Sciences
https://www.readbyqxmd.com/read/28101022/necrobiotic-xanthogranuloma-in-a-patient-with-multiple-myeloma
#17
Marlies Wruhs, Robert Feldmann, Isabelle Sawetz, Friedrich Breier, Andreas Steiner
An 82-year-old woman presented with a 9-month history of multiple, well-defined skin lesions on her neck and upper chest, progressively increasing in size. Histological examination of a skin biopsy showed a regular epidermis. In the dermis, granulomatous changes with central necrobiosis were found which extended focally into the subcutaneous fat. The necrobiotic areas were surrounded by Touton cells and foreign-body giant cells. Laboratory analysis revealed leucopenia. Serum electrophoresis and immunofixation disclosed the presence of an IgG-λ paraprotein...
September 2016: Case Reports in Dermatology
https://www.readbyqxmd.com/read/28070468/multiple-myeloma-associated-chronic-inflammatory-demyelinating-polyradiculoneuropathy-the-importance-of-continued-surveillance
#18
Adebayo A Fasanya, Michael F Loncharich, Viral Gandhi, Sandeep Rana, Marvin Balaan
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an autoimmune disease presenting with weakness and numbness in a remitting or chronic progressive course. It is known to have several clinical presentations and several associated diseases. CIDP has been associated with multiple myeloma, monoclonal gammopathy of undetermined significance (MGUS), and other paraproteinemias. We present a case of refractory CIDP in which the initial workup for multiple myeloma was negative, and multiple myeloma was then diagnosed two and half years later...
November 28, 2016: Curēus
https://www.readbyqxmd.com/read/27994856/c3-glomerulopathy-in-adults-a-distinct-patient-subset-showing-frequent-association-with-monoclonal-gammopathy-and-poor-renal-outcome
#19
Isaac E Lloyd, Alexander Gallan, Hunter K Huston, Kalani L Raphael, Dylan V Miller, Monica P Revelo, Mazdak A Khalighi
BACKGROUND: C3 glomerulopathy (C3G) includes both C3 glomerulonephritis (C3GN) and dense deposit disease (DDD) and is defined by C3-dominant deposits on immunofluorescence. Dysfunction of the alternative pathway (AP) of complement is central to the pathophysiology of C3G and young patients often harbor genetic alterations of AP mediators. Recently, a link between C3G and paraproteinemia has been established. We performed this study to better characterize older patients with C3G where this association is more frequently seen...
December 2016: Clinical Kidney Journal
https://www.readbyqxmd.com/read/27990110/scleromyxedema-without-paraproteinemia-treatment-with-thalidomide-and-prednisolone
#20
Sara Saniee, Ghazaleh Davarnia
Scleromyxedema is a rare and distinctive variant of cutaneous mucinoses of unknown etiology. It is presenting with generalized papular eruption and sclerodermoid induration. Numerous treatment modalities have been reported to produce partial or permanent responses. This study reports on a case of scleromyxedema without paraproteinemia in a subject who experienced a partial response to thalidomide and prednisolone.
September 2016: Case Reports in Dermatology
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