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Paraproteinemia

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https://www.readbyqxmd.com/read/29043147/a-case-report-of-paraproteinemia-associated-pauci-immune-glomerulonephritis-a-new-form-of-monoclonal-gammopathy-of-renal-significance
#1
Robert Rope, Neeraja Kambham, Neiha Arora
BACKGROUND: Renal disease associated with paraproteinemias is classically predicated upon pathologic paraprotein deposition in the kidney. However, growing evidence suggests that paraproteins may be able to systemically activate complement or neutrophils to drive renal damage. This may provide an alternative pathologic mechanism for renal injury in rare cases. CASE REPORT: We report a case of a patient with crescentic pauci-immune glomerulonephritis presenting with rapidly progressive renal failure, polyarthropathy, and a purpuric rash in association with a monoclonal immunoglobulin G κ-light-chain producing multiple myeloma...
2017: Clin Nephrol Case Stud
https://www.readbyqxmd.com/read/29034435/advances-in-the-treatment-of-paraproteinemic-neuropathy
#2
REVIEW
Eduardo Nobile-Orazio, Mariangela Bianco, Andrea Nozza
Purpose of review Several advances have been made on the pathogenesis and therapy of neuropathies associated with paraproteinemia (monoclonal gammopathy). It is important for the neurologist to understand the pathogenetic relevance of this association especially when the hematological disease does not require per se any therapy. Recent findings Treatment of the neuropathy in patients with malignant paraproteinemia is mainly addressed by the hematologist while the neurologist is mainly involved in the initial diagnosis and in deciding whether the neuropathy is caused by the disease or by the chemotherapy used for the disease...
October 16, 2017: Current Treatment Options in Neurology
https://www.readbyqxmd.com/read/28985955/paraprotein-deposits-in-the-skin
#3
REVIEW
Victoria Alegría-Landa, Lorenzo Cerroni, Heinz Kutzner, Luis Requena
Cutaneous manifestations secondary to paraprotein deposits in the skin include a group of different disorders that although rare, may be the first clinical manifestation of the underlying hematologic dyscrasia. In this article we review the clinical manifestations and histopathologic findings of the processes that result from specific deposition of the paraprotein in different structures of the skin. Paraneoplastic processes frequently associated with hematologic malignancies will not be covered in this review...
October 3, 2017: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/28915774/pyoderma-gangrenosum-a-current-problem-as-much-as-an-unknown-one
#4
Valerio Vallini, Roberto Andreini, Angelo Bonadio
Pyoderma gangrenosum (PG) is a rare neutrophilic inflammatory skin disease, characterized by recurrent skin ulcers, which in almost 50% of cases are associated with systemic autoimmune disorders, including rheumatoid arthritis, chronic hepatitis, inflammatory bowel disease, paraproteinemias and hematological malignancies. A systematic search of literature for PG was carried out using the PubMed, Embase, and Google Scholar databases for the purpose of this review and 2780 articles were retrieved up to February 2017...
September 2017: International Journal of Lower Extremity Wounds
https://www.readbyqxmd.com/read/28863117/rituximab-monotherapy-is-effective-in-treating-orbital-necrobiotic-xanthogranuloma
#5
Oded Sagiv, Sudip D Thakar, Gail Morrell, Michael T Tetzlaff, Bita Esmaeli
The authors report a case of a patient with bilateral orbital necrobiotic xanthogranuloma and no associated systemic paraproteinemia. Orbital biopsy showed strong expression of CD20-positive cells. The patient was treated with systemic rituximab monotherapy, with excellent clinical response and marked regression of the orbital lesions on imaging. At the time of writing, the patient has been treated with bimonthly rituximab maintenance therapy for 22 months and has stable clinical and imaging findings with sustained response to treatment and no reported side effects...
August 23, 2017: Ophthalmic Plastic and Reconstructive Surgery
https://www.readbyqxmd.com/read/28781859/hematuria-as-the-first-sign-of-multiple-myeloma
#6
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
#7
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
#8
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
#9
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...
October 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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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