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Bence Jones

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
I G Rekhtina, L P Mendeleeva, E S Stolyarevich, I V Gal'tseva, 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
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
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
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
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
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
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
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.
March 24, 2016: Clinical Chemistry and Laboratory Medicine: CCLM
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
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
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
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
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
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
Stanley S Levinson
BACKGROUND: Serum free light chain (FLC) analysis with ratio and urine immunofixation electrophoresis (IFE) are both available for routine use in helping to detect plasma cell dyscrasia and related diseases. CASES: Case reports showing one serum positive for serum FLC but that showed a hook effect and overestimated the amount of monoclonal FLC while urine IFE was negative for Bence Jones protein, and a second serum that showed elevated FLC κ and λ but a normal κ/λ ratio, while urine IFE was positive for Bence Jones protein...
June 1, 2016: Clinical Chemistry and Laboratory Medicine: CCLM
Manasi Agrawal, Olga C Aroniadis, Lawrence J Brandt, Colleen Kelly, Sarah Freeman, Christina Surawicz, Elizabeth Broussard, Neil Stollman, Andrea Giovanelli, Becky Smith, Eugene Yen, Apurva Trivedi, Levi Hubble, Dina Kao, Thomas Borody, Sarah Finlayson, Arnab Ray, Robert Smith
OBJECTIVES: Clostridium difficile infection (CDI) in the elderly has a higher prevalence, greater morbidity and mortality, and lower response to conventional treatment than the general population. Fecal microbiota transplant (FMT) is highly effective therapy for CDI but has not been studied specifically in the elderly. This study aims to determine the long-term efficacy and safety of FMT for recurrent (RCDI), severe (SCDI), and complicated (CCDI) CDI in elderly patients. METHODS: A multicenter, long-term follow-up study was performed with demographic, pre-FMT, and post-FMT data collected from elderly patients with RCDI, SCDI, and CCDI, through a 47-item questionnaire...
May 2016: Journal of Clinical Gastroenterology
Yasushi Takamatsu, Tsuyoshi Muta
We carried out a cohort study of smoldering multiple myeloma (SMM) in Japan. The clinical data of 207 patients with SMM, median age 69 years (range: 27-90), were collected from 63 institutions. The subtype of myeloma was IgG type in 168, IgA type in 30, and Bence Jones type in 9 patients. At a median follow-up of 39 months, 53% of the patients had progressed to symptomatic MM (within 5 years of the initial diagnosis). As previously described, a serum free light chain ratio>8 or <0.125, along with the number of bone marrow plasma cells being 10% or more and the serum M-protein level being at least 3 g/dl, was a significant predictor of rapid progression...
August 2015: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
R Djidjik, Y Lounici, K Chergeulaïne, Y Berkouk, S Mouhoub, S Chaib, M Belhani, M Ghaffor
IgD multiple myeloma (MM) is a rare subtype of myeloma, it affects less than 2% of patients with MM. To evaluate the clinical and prognostic attributes of serum free light chains (sFLCs) analysis, we examined 17 cases of IgD MM. From 1998 to 2012, we obtained 1250 monoclonal gammapathies including 590 multiple myeloma and 17 patients had IgD MM. With preponderance of men patients with a mean age at diagnosis of: 59±12years. Patients with IgD MM have a short survival (Median survival=9months). The presenting features included: bone pain (75%), lymphadenopathy (16%), hepatomegaly (25%), splenomegaly (8%), associated AL amyloidosis (6%), renal impairment function (82%), infections (47%), hypercalcemia (37%) and anemia (93%)...
September 2015: Pathologie-biologie
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