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Monoclonal gammopathy

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https://www.readbyqxmd.com/read/29781263/urticarial-eruption-in-a-patient-with-intermittent-fever-and-monoclonal-igm-gammopathy
#1
Giovanni Genovese, Angelo V Marzano, Silvia M Ferrucci
No abstract text is available yet for this article.
May 16, 2018: Giornale Italiano di Dermatologia e Venereologia: Organo Ufficiale, Società Italiana di Dermatologia e Sifilografia
https://www.readbyqxmd.com/read/29777014/a-case-of-monoclonal-gammopathy-of-renal-significance
#2
Jonathan J Hogan
No abstract text is available yet for this article.
May 18, 2018: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/29766549/leukocytoclastic-vasculitis-associated-with-immunoglobulin-a-lambda-monoclonal-gammopathy-of-undetermined-significance-a-case-report-and-review-of-previously-reported-cases
#3
Hiroshi Umemura, Osamu Yamasaki, Keiji Iwatsuki
Leukocytoclastic vasculitis is often associated with immunoglobulin (Ig)A deposition on the vascular walls. IgA-associated leukocytoclastic vasculitis comprises various underlying diseases. Hematological disorders that can be minor triggers include multiple myeloma and monoclonal gammopathy of undetermined significance. Here, we present the case of a 78-year-old woman with leukocytoclastic vasculitis associated with monoclonal gammopathy of undetermined significance of the IgA lambda chain. Oral steroid administration initially showed remission of vasculitis; however, the condition recurred after four attempts of treatment withdrawal...
May 15, 2018: Journal of Dermatology
https://www.readbyqxmd.com/read/29761076/synchronous-bone-metastasis-from-multiple-myeloma-and-prostate-adenocarcinoma-as-initial-presentation-of-coexistent-malignancies
#4
Diego Andres Adrianzen Herrera, Shlomit Goldberg-Stein, Alexander Sankin, Judy Sarungbam, Janaki Sharma, Benjamin A Gartrell
The radiographic appearance of bone metastases is usually determined by tumor histology and can be osteolytic, osteoblastic, or mixed. We present a patient with coexistent bone metastasis from multiple myeloma and prostate adenocarcinoma who exhibited synchronous bone involvement of both histologies within the same bone lesion, a rare phenomenon that has not been previously reported and led to atypical radiographic findings. The radiograph of a 71-year-old man with thigh swelling and pain demonstrated a lytic femoral lesion...
2018: Frontiers in Oncology
https://www.readbyqxmd.com/read/29759418/a-clone-directed-approach-may-improve-diagnosis-and-treatment-of-proliferative-glomerulonephritis-with-monoclonal-immunoglobulin-deposits
#5
Ramnika Gumber, Jordana B Cohen, Matthew B Palmer, Sidney M Kobrin, Dan T Vogl, Alan G Wasserstein, Sunita D Nasta, Melissa B Bleicher, Roy D Bloom, Laura Dember, Adam Cohen, Brendan M Weiss, Jonathan J Hogan
The optimal treatment for the monoclonal gammopathies of renal significance is not known, but there is consensus among experts that treatment should be specific for the underlying clone. The majority of patients with proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) do not have an identifiable clone, and prior studies have found poor renal outcomes for patients with PGNMID treated with a variety of regimens. Here we present a retrospective case series of 19 patients with PGNMID with a more uniform, clone-directed approach...
May 11, 2018: Kidney International
https://www.readbyqxmd.com/read/29755672/inhibition-of-mtor-complex-2-restrains-tumor-angiogenesis-in-multiple-myeloma
#6
Aurelia Lamanuzzi, Ilaria Saltarella, Vanessa Desantis, Maria Antonia Frassanito, Patrizia Leone, Vito Racanelli, Beatrice Nico, Domenico Ribatti, Paolo Ditonno, Marcella Prete, Antonio Giovanni Solimando, Francesco Dammacco, Angelo Vacca, Roberto Ria
The mammalian Target of Rapamycin (mTOR) is an intracellular serine/threonine kinase that mediates intracellular metabolism, cell survival and actin rearrangement. mTOR is made of two independent complexes, mTORC1 and mTORC2, activated by the scaffold proteins RAPTOR and RICTOR, respectively. The activation of mTORC1 triggers protein synthesis and autophagy inhibition, while mTORC2 activation promotes progression, survival, actin reorganization, and drug resistance through AKT hyper-phosphorylation on Ser473...
April 17, 2018: Oncotarget
https://www.readbyqxmd.com/read/29747867/-pyoderma-gangrenosum-following-hematopoietic-stem-cell-transplantation
#7
H Eddou, A Ennouhi, M Sina, A Zinebi, J El Benaye, M K Moudden, K Doghmi, J-V Malfuson, M Mikdame, M El Baaj
BACKGROUND: Pyoderma gangrenosum (PG) is a rare form of neutrophilic dermatosis and is a potential complication in a number of systemic diseases. These include blood diseases, which represent 3.5% of cases, with the main forms being monoclonal gammopathy and acute myeloid leukemia. PATIENTS AND METHODS: Herein we report a case of pyoderma gangrenosum in a female patient who had undergone haematopoietic stem cell allograft six months earlier as part of her treatment for acute T-cell leukemia...
May 7, 2018: Annales de Dermatologie et de Vénéréologie
https://www.readbyqxmd.com/read/29740794/focal-segmental-glomerulosclerosis-with-heterozygous-apolipoprotein-e5-glu3lys
#8
Masaru Sasaki, Tetsuhiko Yasuno, Kenji Ito, Akira Matsunaga, Satoshi Hisano, Yasuhiro Abe, Katsuhisa Miyake, Kosuke Masutani, Hitoshi Nakashima, Takao Saito
Apolipoprotein (apo) E5 is a rare apoE isoform. The apoE5 (Glu3Lys) variant, which is caused by the substitution of lysine with glutamic acid at codon 3, has a relative frequency of 0.1% in Japan. Previous studies have reported that apoE5 (Glu3Lys) is associated with hyperlipidemia and cardiovascular diseases, but this isoform has higher LDL receptor-binding activity than that of normal apoE3. Nephropathy associated with apoE5 (Glu3Lys) alone has not yet been reported. We present a case of a 51-year-old man with nephrotic syndrome...
May 8, 2018: CEN Case Reports
https://www.readbyqxmd.com/read/29739120/concordance-of-capillary-electrophoresis-and-conventional-gel-electrophoresis-in-two-different-groups-of-patients-with
#9
Suekyeung Kim, Hyung-Seok Yang, Anbok Lee, Sun Young Cho
BACKGROUND: Serum and urinary protein electrophoresis play an important role in the identification of monoclonal gammopathy. Recently, capillary electrophoresis (CE) has been adapted in many clinical laboratories because of several advantages such as short turnaround time, automation, and high reproducibility. However, there have been unsolved concerns for the concordance between conventional gel and automated capillary electrophoresis methods for protein separation in clinical specimens...
March 1, 2018: Clinical Laboratory
https://www.readbyqxmd.com/read/29731279/a-novel-acta1-mutation-causing-progressive-facioscapuloperoneal-myopathy-in-an-adult
#10
Justin C Kao, Teerin Liewluck, Margherita Milone
We report a 58-year-old woman with slowly progressive facio-scapulo-peroneal muscle weakness due to congenital nemaline myopathy (NM) caused by a novel ACTA1 mutation (c.118A>G, p.Met271Val). In adult patients, congenital NM should be distinguished from sporadic late-onset nemaline myopathy (SLONM), which is a treatable acquired muscle disease often associated with monoclonal gammopathy or HIV infection. Both congenital NM and SLONM are characterized by the presence of nemaline rods in muscle. The patient's clinical history of difficulty running since childhood and weakness in other family members favored a congenital NM...
May 3, 2018: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/29729982/c3-glomerulopathy-associated-with-monoclonal-ig-is-a-distinct-subtype
#11
Aishwarya Ravindran, Fernando C Fervenza, Richard J H Smith, Sanjeev Sethi
Monoclonal immunoglobulins (MIg) may play a causal role in C3 glomerulopathy (C3G) by impairing regulation of the alternative pathway of complement. Ninety-five patients with C3G were tested for MIg of which 36 were positive. Their mean age at diagnosis was 60 years and among patient 50 years and older, 65.1% had a MIg. At presentation, median serum creatinine and proteinuria were 1.9 mg/dL and 3.0 g/24 hours. Hematuria was present in 32 (88.9%) patients. Twelve (34.3%) patients had low C3 levels. C3 nephritic factor was detected in 45...
May 2, 2018: Kidney International
https://www.readbyqxmd.com/read/29725501/the-untold-stories-of-the-speech-gene-the-foxp2-cancer-gene
#12
REVIEW
Maria Jesus Herrero, Yorick Gitton
FOXP2 encodes a transcription factor involved in speech and language acquisition. Growing evidence now suggests that dysregulated FOXP2 activity may also be instrumental in human oncogenesis, along the lines of other cardinal developmental transcription factors such as DLX5 and DLX6 [1-4]. Several FOXP familymembers are directly involved during cancer initiation, maintenance and progression in the adult [5-8]. This may comprise either a pro-oncogenic activity or a deficient tumor-suppressor role, depending upon cell types and associated signaling pathways...
January 2018: Genes & Cancer
https://www.readbyqxmd.com/read/29724182/favorable-effect-of-bortezomib-in-dense-deposit-disease-associated-with-monoclonal-gammopathy-a-case-report
#13
Shuma Hirashio, Ayaka Satoh, Takahiro Arima, Kouichi Mandai, Tadasuke Awaya, Kumi Oshima, Shigeo Hara, Takao Masaki
BACKGROUND: Complement component 3 (C3) glomerulopathy, which includes dense deposit disease (DDD) and C3 glomerulonephritis, is caused by dysregulation of the alternative complement pathway. In most cases, C3 glomerulopathy manifests pathologically with membranoproliferative glomerulonephritis-like features. An association between C3 glomerulopathy and monoclonal gammopathy was recently reported in several cases, raising the possibility that C3 glomerulopathy is the underlying pathological process in monoclonal gammopathy of renal significance...
May 3, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29724174/eculizumab-as-salvage-therapy-for-recurrent-monoclonal-gammopathy-induced-c3-glomerulopathy-in-a-kidney-allograft
#14
Philipp Moog, Philipp J Jost, Maike Büttner-Herold
BACKGROUND: Monoclonal gammopathy causes several kinds of renal pathology. A rare and special form is monoclonal gammopathy-induced C3 glomerulopathy (MG-C3G). Like idiopathic C3G, MG-C3G frequently leads to end-stage renal disease. MG-C3G frequently recurs after renal transplantation, leading to graft failure in most of the patients. While there is some evidence for successful treatment of recurrent idiopathic C3 glomerulopathy with eculizumab after renal transplantation, nothing is known about its efficacy in the setting of recurrent MG-C3G...
May 3, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29721239/meningitis-spondylodiscitis-pneumonia-and-septic-shock-with-streptococcus-pneumoniae-in-a-previously-healthy-woman-with-isolated-igg2-igg3-iga-deficiency-and-monoclonal-gammopathy-of-undetermined-significance
#15
Shahin Gaini, David Gudnason, Bjarni Á Steig, Jenny Jónsdóttir Nielsen
A 66 years old Caucasian woman with pneumococcal meningitis was treated and discharged after an uncomplicated course. Five months later she was readmitted with fever and right side abdominal pain and diagnosed with pneumococcal spondylodiscitis. One year later she was treated for a severe chest X-ray confirmed left lobar pneumonia. Two years later she was diagnosed with a pneumococcal pneumonia in her left lung with septic shock. An immune deficiency screen revealed slightly reduced IgA levels, low IgG2 levels, low IgG3 levels and high IgG1 levels...
March 29, 2018: Infectious Disease Reports
https://www.readbyqxmd.com/read/29710195/multiple-myeloma-and-its-precursor-disease-among-firefighters-exposed-to-the-world-trade-center-disaster
#16
Ola Landgren, Rachel Zeig-Owens, Orsolya Giricz, David Goldfarb, Kaznouri Murata, Katie Thoren, Lakshmi Ramanathan, Malin Hultcrantz, Ahmet Dogan, George Nwankwo, Ulrich Steidl, Kith Pradhan, Charles B Hall, Hillel W Cohen, Nadia Jaber, Theresa Schwartz, Laura Crowley, Michael Crane, Shani Irby, Mayris P Webber, Amit Verma, David J Prezant
Importance: The World Trade Center (WTC) attacks on September 11, 2001, created an unprecedented environmental exposure to known and suspected carcinogens suggested to increase the risk of multiple myeloma. Multiple myeloma is consistently preceded by the precursor states of monoclonal gammopathy of undetermined significance (MGUS) and light-chain MGUS, detectable in peripheral blood. Objective: To characterize WTC-exposed firefighters with a diagnosis of multiple myeloma and to conduct a screening study for MGUS and light-chain MGUS...
April 26, 2018: JAMA Oncology
https://www.readbyqxmd.com/read/29707401/a-rare-but-fascinating-disorder-case-collection-of-patients-with-schnitzler-syndrome
#17
Maaman Bashir, Brittany Bettendorf, Richard Hariman
Background: Schnitzler syndrome is a rare disorder characterized by a chronic urticarial rash and monoclonal gammopathy (IgM in more than 90% of the cases). It is difficult to distinguish from other neutrophilic urticarial dermatoses, and diagnosis is based on the Strasbourg criteria. Interleukin-1 is considered the key mediator, and interleukin-1 inhibitors are considered first line treatment. Here, we present two cases of Schnitzler syndrome, both successfully treated with anakinra...
2018: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29706814/proliferative-glomerulonephritis-with-monoclonal-immunoglobulin-deposits-of-lambda-chains
#18
Muhammad A Panezai, Pingchaun Zhang, Gates B Colbert
Increasingly, monoclonal gammopathies of renal significance (MGRS) are being described as unique, distinct disease states. We describe a type of MGRS with proliferative glomerular lesions with monoclonal immunoglobulin deposits of rarely reported IgG2 lambda chains.
April 2018: Proceedings of the Baylor University Medical Center
https://www.readbyqxmd.com/read/29703839/the-complexity-and-heterogeneity-of-monoclonal-immunoglobulin-associated-renal-diseases
#19
Sanjeev Sethi, S Vincent Rajkumar, Vivette D D'Agati
Monoclonal gammopathies are characterized by the overproduction of monoclonal Ig (MIg) detectable in the serum or urine resulting from a clonal proliferation of plasma cells or B lymphocytes. The underlying hematologic conditions range from malignant neoplasms of plasma cells or B lymphocytes, including multiple myeloma and B-cell lymphoproliferative disorders, to nonmalignant small clonal proliferations. The term MGUS implies presence of an MIg in the setting of a "benign" hematologic condition without renal or other end organ damage...
April 27, 2018: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/29703722/-myd88-l265p-mutation-in-lymphoid-malignancies
#20
REVIEW
Xinfang Yu, Wei Li, Qipan Deng, Ling Li, Eric D Hsi, Ken H Young, Mingzhi Zhang, Yong Li
Next-generation sequencing has revealed cancer genomic landscapes, in which over 100 driver genes that, when altered by intragenic mutations, can promote oncogenesis. MYD88 is a driver gene found in hematologic B-cell malignancies. A missense mutation (L265P) changing leucine at position 265 to proline in MYD88 is found in ∼90% of Waldenström macroglobulinemia (WM) cases and in significant portions of activated B-cell diffuse large B-cell lymphomas and IgM monoclonal gammopathy of undetermined significance...
April 27, 2018: Cancer Research
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