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https://www.readbyqxmd.com/read/28694632/late-onset-familial-amyloidotic-polyneuropathy-with-bence-jones-proteinuria-and-cardiomyopathy
#1
Sira Carrasco García de León, Amalia Hernández González, Carmen Orellana Alonso, Laura Burriel Lobo
Familial amyloidotic polyneuropathy is a genetically determined disease characterized by deposition of an anomalous transthyretin. A high index of suspicion is needed for this multisymptomatic and lethal disease to be diagnosed. The patient was a 70-year-old male examined due to hypesthesia in the hands and feet, plus difficulty walking. A neurophysiological study delivered the diagnosis of axonal sensorimotor polyneuropathy. He later developed cardiac symptoms and diarrhea. Urine laboratory analyses revealed a monoclonal spike of light chains (kappa)...
July 2017: Journal of Neurosciences in Rural Practice
https://www.readbyqxmd.com/read/28544666/identifying-target-reference-points-for-harvesting-assessment-limited-wildlife-populations-a-case-study
#2
Bryan S Stevens, James R Bence, William F Porter, Michael L Jones
Identifying appropriate strategies for sustainable harvest is a challenge for many terrestrial vertebrate species because of uncertain system dynamics, limited data to inform population models, and potentially conflicting objectives that seek to harvest and maintain populations at desirable levels. The absence of monitoring and assessment infrastructure needed to regularly estimate abundance accentuates this challenge for many species, and limits application of rigorous state-dependent frameworks for decision making that are commonly advocated in natural resource management...
May 22, 2017: Ecological Applications: a Publication of the Ecological Society of America
https://www.readbyqxmd.com/read/28278719/restoration-of-renal-function-in-patients-with-newly-diagnosed-multiple-myeloma-is-not-associated-with-improved-survival-a-population-based-study
#3
Joost C de Vries, Berdien Oortgiesen, Marc H Hemmelder, Eric van Roon, Robby E Kibbelaar, Nic Veeger, Mels Hoogendoorn
Renal impairment (RI) in patients with multiple myeloma (MM) is associated with poor prognosis. In this population-based cohort study, we assessed the effects of renal response, evaluated according to the IMWG-criteria, on overall survival (OS) in patients with newly diagnosed MM with RI at presentation. All included patients were diagnosed between January 2005 and January 2014 with MM and RI in Friesland, a province of the Netherlands. Of the 131 included patients, 61% achieved renal response. Using a time-varying exposure Cox model, no difference in OS between renal response and non-response was observed (HR = 1...
September 2017: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/28202864/clinical-presentation-of-tubulointerstitial-nephritis-caused-by-amyloid-light-chain-amyloidosis-in-a-patient-with-sj%C3%A3-gren-s-syndrome
#4
Reiko Inoue, Yoshihide Fujigaki, Kana Kobayashi, Yoshifuru Tamura, Tatsuru Ota, Shigeru Shibata, Tsuyoshi Ishida, Fukuo Kondo, Yutaka Yamaguchi, Shunya Uchida
We report a 70-year-old woman with Sjögren's syndrome who had severe renal dysfunction with mild proteinuria and elevated urinary low-molecular-weight proteins. Based on these clinical presentations, interstitial nephritis due to Sjögren's syndrome was strongly suspected. Unexpectedly, renal pathology revealed amyloid light-chain (AL) lambda-type depositions predominantly in the vasculatures with severe tubulointerstitial damage. Concentrated urine immunofixation was positive for Bence Jones lambda-type monoclonal proteins...
2017: Internal Medicine
https://www.readbyqxmd.com/read/27504298/non-secretory-myeloma-diagnosed-on-renal-biopsy-as-cast-nephropathy
#5
Sumit Grover, Pavneet Kaur Selhi, Neena Sood, Jasvinder Singh Sandhu, Harpreet Kaur
Multiple myeloma is a disorder of plasma cells which can involve kidneys in the form of cast nephropathy. Neoplastic plasma cells produce either complete immunoglobulins or fragments of immunoglobulins leading to a monoclonal spike in the serum and/or Bence Jones proteinuria. Very few patients present as non-secretory myeloma when no immunoglobulins (M spike) are produced or only light chains are secreted which can only be detected in urine. Acute renal failure due to cast nephropathy can rarely be the first presentation of multiple myeloma...
June 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/27296267/-secondary-monoclonal-gammopathy-after-bone-marrow-autotransplantation-as-a-cause-of-worse-renal-function-in-light-chain-immunoglobulin-deposition-disease
#6
I G Rekhtina, L P Mendeleeva, E S Stolyarevich, I V Galtseva, P E Povilaitite, L S Biryukova
The paper describes a clinical case of a female woman with nephropathy due to light chain deposition disease caused by secretion of κ Bence-Jones protein. Complete immunochemical remission was achieved after induction therapy using a bortezomib + cyclophosphamide + dexamethasone regimen. Renal function remained unchanged (glomerular filtration rate 16 ml/min), there was a reduction in proteinuria from 5.8 to 2.6 g/day. High-dose melphalan (200 mg/m2) chemotherapy with peripheral blood stem cell autotransplantation was performed as consolidation of remission...
2016: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/27284363/multiple-myeloma-associated-skin-light-chain-amyloidosis-a-case-of-misdiagnosis
#7
L I Xiao, Fengxia Lin, Rong Xiao, Chun Hu, Mingyang Deng, Daiqiang Li, Xiaoling She, Fuyou Liu, Lin Sun
The present study reports the case of a 42-year-old male with multiple myeloma (MM)-associated skin light chain amyloidosis who presented with skin purpura as the initial symptom, which was misdiagnosis as Henoch-Schönlein purpura nephritis prior to admission to the Second Xiangya Hospital (Changsha, Hunan, China). The patient presented with purpura, papules petechiae and spontaneous ecchymosis, which was located scattered around the neck, chest and limbs, accompanied by a small amount of bleeding in the conjunctival and oral mucosa, and a swollen tongue...
June 2016: Oncology Letters
https://www.readbyqxmd.com/read/27282939/solomon-a-fahey-jl-malmgren-ra-immunohistologic-localization-of-gamma-1-macroglobulins-beta-2a-myeloma-proteins-6-6-s-gamma-myeloma-proteins-and-bence-jones-proteins-blood-1963-21-4-403-423
#8
https://www.readbyqxmd.com/read/27263790/multiple-myeloma-developing-in-a-patient-with-immune-thrombocytopenia
#9
REVIEW
Toshio Itoh
A female diagnosed as having immune thrombocytopenic purpura (ITP) was found to be simultaneously suffering from monoclonal gammopathy of undetermined significance (IgGλ). Urine Bence-Jones protein was negative. During the course, plasma cells accounted for 21.6% of the bone marrow. Based on these clinical features in our case, the second disease was diagnosed as multiple myeloma (MM). Both ITP and MM were successfully treated with corticosteroids, bortezomib, lenalidomide with dexamethasone and eltrombopag olamine...
May 2016: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/27263787/achievement-of-hemodialysis-discontinuation-with-lenalidomide-and-dexamethasone-therapy-in-a-refractory-bjp-type-multiple-myeloma-patient
#10
Tomoyuki Uchida, Morihiro Inoue, Jian Hua, Masao Hagihara
A 63-year-old man with Bence Jones-κ multiple myeloma (MM) presented with renal impairment. First, we administered a bortezomib-containing regimen which is considered to be the first choice among therapeutic approaches for MM patients with renal failure. However, his condition was refractory to bortezomib, and the renal dysfunction worsened (creatinine 12.55mg/dl) necessitating the initiation of hemodialysis. Subsequently, we administered an adjusted dose of lenalidomide and dexamethasone. Dialysis could be discontinued after 3 cycles of lenalidomide therapy...
May 2016: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/27170372/clinicopathological-significance-of-monoclonal-iga-deposition-in-patients-with-iga-nephropathy
#11
Hiroshi Nagae, Akihiro Tsuchimoto, Kazuhiko Tsuruya, Shota Kawahara, Yukiko Shimomura, Hideko Noguchi, Kosuke Masutani, Ritsuko Katafuchi, Takanari Kitazono
BACKGROUND: Clinicopathological significance of monoclonal IgA deposition and its relation to bone marrow abnormalities in IgA nephropathy (IgAN) remains unclear. METHODS: We retrospectively investigated the prevalence and clinicopathological significance of monoclonal IgA deposition in 65 patients with IgAN. Serum-free light chain ratio, and urinary Bence Jones protein were also measured. RESULTS: Thirty-nine percent of patients were men, median age was 40 and median observation period was 31 months...
May 12, 2016: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/27133826/bence-jones-proteinuria-in-smoldering-multiple-myeloma-as-a-predictor-marker-of-progression-to-symptomatic-multiple-myeloma
#12
V González-Calle, J Dávila, F Escalante, A G de Coca, C Aguilera, R López, A Bárez, J M Alonso, R Hernández, J M Hernández, Pilar de la Fuente, N Puig, E M Ocio, N C Gutiérrez, R García-Sanz, M-V Mateos
The diagnosis of smoldering multiple myeloma (SMM) includes patients with a heterogeneous risk of progression to active multiple myeloma (MM): some patients will never progress while others will have a high risk of progression within the first two years. Therefore, it is important to improve risk assessment at diagnosis. We conducted a retrospective study in a large cohort of SMM patients in order to investigate the role of Bence Jones (BJ) proteinuria at diagnosis in the progression to active MM. We found that SMM patients presenting with BJ proteinuria had a significantly shorter median time to progression (TTP) to MM compared to patients without BJ proteinuria (22 vs 88 months, respectively; HR=2...
May 2, 2016: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/27044938/phase-i-study-of-ly2606368-a-checkpoint-kinase-1-inhibitor-in-patients-with-advanced-cancer
#13
David Hong, Jeffrey Infante, Filip Janku, Suzanne Jones, Ly M Nguyen, Howard Burris, Aung Naing, Todd M Bauer, Sarina Piha-Paul, Faye M Johnson, Razelle Kurzrock, Lisa Golden, Scott Hynes, Ji Lin, Aimee Bence Lin, Johanna Bendell
PURPOSE: The primary objective was to determine safety, toxicity, and a recommended phase II dose regimen of LY2606368, an inhibitor of checkpoint kinase 1, as monotherapy. PATIENTS AND METHODS: This phase I, nonrandomized, open-label, dose-escalation trial used a 3 + 3 dose-escalation scheme and included patients with advanced solid tumors. Intravenous LY2606368 was dose escalated from 10 to 50 mg/m(2) on schedule 1 (days 1 to 3 every 14 days) or from 40 to 130 mg/m(2) on schedule 2 (day 1 every 14 days)...
May 20, 2016: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/27010779/evaluation-of-screening-method-for-bence-jones-protein-analysis
#14
LETTER
Umberto Basile, Francesca Gulli, Eleonora Torti, Cecilia Napodano, Maria Teresa Dell'Abate, Elena De Santis, Stefano Angelo Santini, Laura Conti, Cecilia Zuppi, Giovanni Cigliana
No abstract text is available yet for this article.
November 1, 2016: Clinical Chemistry and Laboratory Medicine: CCLM
https://www.readbyqxmd.com/read/26949303/plasmacytoma-of-the-breast-a-report-of-a-rare-disease
#15
Ugare Gabriel, Udosen Joseph, Ima-Abasi Bassey, Ayodele Joshua, Djunda Emmanuel
BACKGROUND: Extramedullary plasma cells tumours are rare. Much more rarer is their occurance in the breast tissue. Our aim is to report a single case of this very rare lesion (at least from an African perspective) that we incidentally diagnosed histopathologically as a primary extramedullary lesion in a 53 year old woman. CASE DETAILS: Clinical records of a 53 year old postmenopausal woman was referred from a secondary health centre to our clinic with a three weeks' history of right breast lump were reviewed...
October 2015: Ethiopian Journal of Health Sciences
https://www.readbyqxmd.com/read/26831021/bence-jones-protein-%C3%AE-type-multiple-myeloma-patient-withdrawn-from-maintenance-hemodialysis-after-long-term-bortezomib-and-dexamethasone-therapy
#16
Wakaba Yamaguchi, Naofumi Yui, Toshikage Nagao, Gaku Oshikawa, Mariko Negi, Soichiro Iimori, Tomokazu Okado, Tatemitsu Rai, Shinichi Uchida
The effectiveness of bortezomib treatment for multiple myeloma (MM) is well established. However, the protocol by which maintenance therapy using bortezomib should be continued for myeloma patients requiring regular hemodialysis remains to be established. We herein report a case of MM with severe renal insufficiency requiring hemodialysis for nearly 30 months which was finally withdrawn from renal replacement therapy during monthly maintenance treatment with bortezomib and dexamethasone for two years. The details of this case are essential for establishing clinical guidelines for applying intermittent low-frequency bortezomib therapy in dialysis-dependent myeloma patients...
2016: Internal Medicine
https://www.readbyqxmd.com/read/26664659/disseminated-plasma-cell-myeloma-presenting-as-massive-pleural-effusion
#17
Kanahasubramanian Anand Babu, Lakshmikanthan Sundararajan, Pandurangan Prabu, Ashok Parameswaran
Plasma cell myeloma (PCM) is a hematologic malignancy of plasma cell origin and usually associated with the presence of lytic bone lesions. Pleural effusions are rarely associated with PCM and most often signify a concurrent disease process. Malignant myelomatous pleural effusions are even more unusual and carry a poor prognosis. We report a unique case of unsuspected PCM with thoracic involvement in the form of massive left side pleural effusion. Pleural fluid cytology revealed numerous atypical plasma cells...
2015: European Clinical Respiratory Journal
https://www.readbyqxmd.com/read/26642715/the-cutoff-level-for-urine-protein-in-urine-immunofixation-electrophoresis
#18
Hamit Yasar Ellidag, Gulten Curek, Esin Eren, Ozgur Aydin, Necat Yilmaz
BACKGROUND: Immunofixation electrophoresis (IFE) maintains its importance in diagnosing monoclonal gammopathies. In particular, urine IFE detects free light chains (FLC) in urine samples even at low concentrations and offers higher sensitivity compared to serum electrophoresis and serum IFE. The aim of the present study was to determine the place and significance of quantitative urinary protein measurement before IFE in interpreting the results of subsequent IFE and to determine the most appropriate protein concentrations for the appearance of bands...
2015: Clinical Laboratory
https://www.readbyqxmd.com/read/26640537/a-voluminous-mass-as-an-initial-clinical-symptom-of-multiple-myeloma-a-case-report
#19
Nicola Sgherza, Angela Iacobazzi, Angelantonio Cramarossa, Giacoma DE Tullio, Attilio Guarini
Extramedullary multiple myeloma (EMM) is a type of multiple myeloma (MM) that is defined by the presence of extraskeletal (soft tissue or visceral) clonal plasma cell infiltrates, which may be present at the time of initial diagnosis or at the time of relapse. Although extramedullary lesions may be present with other clinical features at the time of diagnosis, the onset of a solid formation as a first clinical symptom of MM is unusual. The present study reports the case of a 77-year-old male who was admitted to the Hematology Unit of the National Cancer Research Center, Istituto Tumori 'Giovanni Paolo II' (Bari, Italy) with a mass protruding from the right side of his lower back...
November 2015: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/26484033/fibrillary-glomerulonephritis-combined-with-chronic-inflammatory-demyelinating-polyneuropathy
#20
Woo Kyung Sung, Jin Uk Jeong, Ki Tae Bang, Jong Ho Shin, Ji Hyung Yoo, Nak Min Kim, Jun Hyung Park, Joo Heon Kim
A 58-yr-old man presented with leg edema and subacute weakness of his bilateral lower extremities. Urinary and serum immunoelectrophoresis revealed the presence of lambda-type Bence Jones proteins. He was ultimately diagnosed with monoclonal gammopathy of undetermined significance (MGUS). A renal biopsy specimen showed fibrillary glomerulonephritis (FGN), which was randomly arranged as 12-20 m nonbranching fibrils in the basement membranes. Immunofluorescence studies were negative for immunoglobulin (Ig)G, IgM, IgA, C3, and kappa light chains in the capillary walls and mesangial areas...
June 2015: Kidney Research and Clinical Practice
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