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https://www.readbyqxmd.com/read/29772037/a-review-of-fabry-disease
#1
A Cinats, E Heck
The class of medications known as Janus kinase inhibitors block cytokine-mediated signaling via the Janus kinase-signal transducer and activator of transcription (JAK-STAT) pathway, which plays an important role in immunoregulation and normal cell growth. This class includes the drugs tofacitinib, approved for the treatment of rheumatoid arthritis, and ruxolitinib, approved for the treatment of myelofibrosis and polycythemia rubra vera. The most common adverse events (AEs) reported in patients taking tofacitinib are infections, whereas the most common AEs in patients taking ruxolitinib are anemia and thrombocytopenia...
May 2018: Skin Therapy Letter
https://www.readbyqxmd.com/read/29742552/subcutaneous-panniculitis-like-t-cell-lymphoma-versus-lupus-erythematosus-panniculitis-distinction-by-means-of-the-periadipocytic-cell-proliferation-index
#2
Panitta Sitthinamsuwan, Penvadee Pattanaprichakul, Jitsupa Treetipsatit, Tawatchai Pongpruttipan, Sanya Sukpanichnant, Laura B Pincus, Timothy H McCalmont
The distinction between subcutaneous panniculitis-like T-cell lymphoma (SPTCL) and lupus erythematosus (LE) panniculitis is remarkably challenging. Rimming by lymphocytes with an elevated Ki-67 cell proliferation index has been forwarded as a potential diagnostic finding in biopsies of SPTCL but has not been rigorously compared with biopsies from patients with LE panniculitis. Nineteen and 17 examples of SPTCL and LE panniculitis, respectively, were evaluated for periadipocytic rimming by lymphocytes expressing Ki-67, CD8, and βF1 and for attributes associated with LE, including clusters of CD123-positive cells...
May 2, 2018: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/29726727/epigenetic-therapy-and-dermatologic-disease-moving-beyond-ctcl
#3
Joshua S Mervis, Jean S McGee
The epigenetic regulation of gene expression is accomplished primarily through DNA methylation, histone modification, and gene silencing via the action of microRNAs. While previously very difficult to study, the field of epigenetics has been greatly facilitated by recent technological innovations. Alterations in the epigenome and epigenetic machinery are now known to be present in a variety of diseases, most notably cancers. Moreover, evidence has emerged that epigenetic dysregulation plays a causative role in disease pathogenesis...
May 4, 2018: Journal of Dermatological Treatment
https://www.readbyqxmd.com/read/29719023/maximizing-the-clinical-utility-of-descriptive-lymphoid-pathology-reporting
#4
Timothy H McCalmont
Dermatopathology reporting can be both exact and inexact. Exact reporting represents the use of terminology that corresponds to a disease sui generis, such as discoid lupus erythematosus or disseminated superficial porokeratosis. Inexact reporting can vary greatly amongst various practitioners-both in terms of the exact semantics used and also stylistically-and can be used habitually by pathologists as a means to provide cover for diagnostic uncertainty or inexperience. This article explores the use of descriptive (inexact) reporting as it applies to cutaneous lymphoma and its differential diagnosis...
March 2018: Seminars in Cutaneous Medicine and Surgery
https://www.readbyqxmd.com/read/29671953/bone-marrow-as-a-target-organ-of-systemic-lupus-erythematosus-analysis-of-cases-with-myelofibrosis
#5
Döndü Üsküdar Cansu, Hava Üsküdar Teke, Serap Işiksoy, Cengiz Korkmaz
AIM: Cytopenia in the course of systemic lupus erythematosus (SLE) may be due to multiple factors. In this study, we aimed primarily to evaluate the detailed results of bone marrow (BM) biopsies of SLE patients, secondly to determine the myelofibrosis (MF) frequency and thirdly to compare BM morphologic findings as well as the clinical and laboratory parameters between groups (with MF and without MF) in cytopenic SLE patients. METHODS: We retrospectively analyzed 224 SLE patients' files...
April 19, 2018: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/29652962/-myasthenia-gravis-and-its-association-with-lymphoproliferative-disorders-a-case-series
#6
Gabriel Cea, Andrés Gallardo V, María Elena Cabrera C
Myasthenia gravis (MG) is a rare autoimmune disease of the neuromuscular junction. It is characterized by variable weakness and excessive fatigability of skeletal muscles. In the last few years, numerous reports have been published showing the association between autoimmune diseases, such as systemic erythematous lupus or rheumatoid arthritis, with lymphoid neoplasias. The association between MG and lymphoid neoplasia seems to be less frequent. To analyze this association we reviewed the MG patients in the Department of Neurology, Hospital Salvador of Santiago, Chile...
December 2017: Revista Médica de Chile
https://www.readbyqxmd.com/read/29651368/synchronous-pulmonary-neoplasms-a-chance-occurrence-or-is-there-more-than-meets-the-eye
#7
Uroosa Ibrahim, Amina Saqib, Manisha Pant, Gwenalyn Garcia, Marcel Odaimi
Primary bronchus-associated lymphoid tissue (BALT) lymphoma comprises 5% of non-Hodgkin's lymphoma (NHL) and usually has an indolent course. Synchronous primary lung cancers with BALT lymphoma are seldom seen in patients with adenocarcinoma of the lung. Synchronous squamous cell carcinoma (SCC) and BALT lymphoma is an extremely rare occurrence. We report an unusual case of stage 4 BALT lymphoma requiring treatment that revealed an underlying ipsilateral mass causing a diagnostic dilemma. An 84-year-old female with a history of systemic lupus erythematosus, deep vein thrombosis, and thrombotic microangiopathy presented to the hospital with cough and dyspnea on exertion...
February 6, 2018: Curēus
https://www.readbyqxmd.com/read/29568865/trifluoperazine-induces-apoptosis-through-the-upregulation-of-bax-bcl%C3%A2-2-and-downregulated-phosphorylation-of-akt-in-mesangial-cells-and-improves-renal-function-in-lupus-nephritis-mice
#8
Baodong Wang, Yankun Luo, Xiaoshuang Zhou, Rongshan Li
The inhibition of mesangial cell (MC) proliferation has become an important therapy in preventing glomerular proliferation diseases. Trifluoperazine (TFP) has been reported to inhibit the proliferation of several types of cancer cell, however, the effects of TFP in renal proliferation diseases remain to be fully elucidated. The present study examined the effects of TFP on the proliferation of MCs and quantified cell apoptosis progression in vivo and in vitro. The effects of various TFP concentrations and treatment durations on cell proliferation and cell apoptosis in vitro were analyzed using flow cytometry in conjunction with a Cell Counting kit‑8 assay...
March 13, 2018: International Journal of Molecular Medicine
https://www.readbyqxmd.com/read/29552450/a-case-of-htlv-1-associated-adult-t-cell-lymphoma-presenting-with-cutaneous-lesions-and-tropical-spastic-paresis
#9
Purva V Sharma, Michael Witteman, Swethika Sundaravel, Tulisa Larocca, Yuanming Zhang, Harry Goldsztajn
Adult T cell lymphoma (ATL), is a peripheral T cell neoplasm associated with infection by human T-lymphotropic virus (HTLV). This is a case of a 28-year-old lady who presented with back pain for the past month and recent onset weakness in her lower extremities bilaterally. She has a history of T-cell lymphoma secondary to HTLV-1 under remission since 2014 and systemic lupus erythematosus complicated by lupus nephritis. On physical examination patient had hyper-reflexia in both knees, ankle clonus bilaterally and spasticity in both her lower extremities...
February 2018: Intractable & Rare Diseases Research
https://www.readbyqxmd.com/read/29546569/a-case-of-duodenal-malignant-lymphoma-presenting-as-acute-pancreatitis-systemic-lupus-erythematosus-and-immunosuppressive-therapy-as-risk-factors
#10
Reiko Yamada, Takashi Sakuno, Hiroyuki Inoue, Hiroshi Miura, Toshifumi Takeuchi, Yasunori Shiono, Hiroaki Okuse, Misaki Nakamura, Masaki Katsurahara, Yasuhiko Hamada, Kyosuke Tanaka, Noriyuki Horiki, Yoshiyuki Takei
A 49-year-old man was admitted to our hospital with pancreatitis. He was diagnosed with systemic lupus erythematosus at 34 years of age and was being treated with oral tacrolimus (3 mg/day) and predonine (10 mg/day) for the past 15 months. The computed tomography (CT) scan showed the mass lesion had invaded the pancreatic head via thickening of the duodenal wall. Upper gastrointestinal endoscopy showed the all-round ulcerative lesion from the superior duodenal angle to the descending portion. Histological examination confirmed the diagnosis of diffuse large B cell lymphoma (DLBCL)...
March 15, 2018: Clinical Journal of Gastroenterology
https://www.readbyqxmd.com/read/29532268/monoclonal-gammopathy-in-rheumatic-diseases
#11
Yue Yang, Long Chen, Yuan Jia, Yang Liu, Lei Wen, Yaoxian Liang, Yuan An, Shi Chen, Yin Su, Zhanguo Li
To analyze the clinical spectrum, laboratory characteristics, and outcomes of monoclonal gammopathy (MG) in patients with rheumatic diseases. Screening for the presence of MG was performed in 872 inpatients with rheumatic diseases from January 2010 to July 2017. A total of 41 patients were enrolled. Their clinical and biological features in addition to outcomes were described. For each patient with primary Sjögren syndrome (pSS), 2 age- and sex-matched pSS patients without MG were selected as controls. Risk factors for the presence of MG and malignant hematological neoplasias were assessed...
March 13, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29514339/false-positive-monospot-in-a-returning-traveler-with-dengue-fever
#12
Kimberly Boyd, Joshua M Harrison, Michael J Kavanaugh
The heterophile antibody (Monospot), initial test of choice for Epstein-Barr virus (EBV)-associated infectious mononucleosis, is both sensitive (70-92%) and specific (96-100%). False positives have been demonstrated in cases of viral hepatitis, human immunodeficiency virus, leukemia, lymphoma, pancreatic cancer, systemic lupus erythematosus, and rubella. We present a case of a 46-yr-old male who developed fever, chills, headaches, myalgia, fatigue, and photophobia 1 d after returning from the Philippines. He demonstrated a mild transaminitis and significant thrombocytopenia (12,000 cells/μL)...
March 1, 2018: Military Medicine
https://www.readbyqxmd.com/read/29495920/multifocal-vertebral-sclerosing-bone-changes-and-soft-tissue-masses-caused-by-hodgkin-s-lymphoma-in-a-patient-with-systemic-lupus-erythematosus-a-case-report
#13
S Krabbe, J Helweg-Larsen, A Loft, S Jacobsen
No abstract text is available yet for this article.
March 2, 2018: Scandinavian Journal of Rheumatology
https://www.readbyqxmd.com/read/29422815/the-role-of-regulatory-t-cells-and-genes-involved-in-their-differentiation-in-pathogenesis-of-selected-inflammatory-and-neoplastic-skin-diseases-part-iii-polymorphisms-of-genes-involved-in-tregs-activation-and-function
#14
REVIEW
Bogusław Nedoszytko, Małgorzata Sokołowska-Wojdyło, Joanna Renke, Magdalena Lange, Piotr Trzonkowski, Michał Sobjanek, Aneta Szczerkowska-Dobosz, Marek Niedoszytko, Aleksandra Górska, Jan Romantowski, Jarosław Skokowski, Leszek Kalinowski, Roman J Nowicki
Regulatory T cells (Tregs) represent a cell type that promotes immune tolerance to autologous components and maintains immune system homeostasis. The abnormal function of Tregs is relevant to the pathogenesis of several skin diseases like psoriasis, atopic dermatitis, systemic lupus erythematosus, cutaneous T-cell lymphomas, and skin cancer and is also important in rheumatoid arthritis, diabetes and other autoimmune diseases. In this review, we will summarize the role of mutations and/or polymorphisms of genes involved in Tregs development, and functions in the pathogenesis of selected skin diseases...
December 2017: Postȩpy Dermatologii i Alergologii
https://www.readbyqxmd.com/read/29417312/lessons-learned-from-splenic-infarcts-with-fever-of-unknown-origin-fuo-culture-negative-endocarditis-cne-or-malignancy
#15
REVIEW
Burke A Cunha, Bertamaria Dieguez, Alena Varantsova
Culture negative endocarditis (CNE) is a common concern in patients with fever, heart murmur, cardiac vegetation, and negative blood cultures. The diagnosis of CNE is not based only on negative blood cultures and a cardiac vegetation. The clinical definition of CNE is based on negative blood cultures plus the findings of culture positive infective endocarditis (IE), e.g., fever, cardiac vegetation, splenomegaly, peripheral manifestations. Because embolic splenic infarcts may occur with culture positive IE, some may assume that splenic infarcts are a sign of CNE...
February 7, 2018: European Journal of Clinical Microbiology & Infectious Diseases
https://www.readbyqxmd.com/read/29394927/quantification-of-cancer-risk-in-glomerulonephritis
#16
James Goya Heaf, Alastair Hansen, Gunnar Hellmund Laier
BACKGROUND: The association of increased cancer risk with glomerulonephritis (GN) is well known, but controversy exists concerning which types of GN are involved, and the size of the association. A national registry survey was performed to assess the size of this association, and the temporal relationship of cancer diagnosis to GN diagnosis. METHODS: All patients with biopsy-proven GN between 1985 and 2015 in Denmark were extracted from The Danish Renal Biopsy Registry and the National Pathology Data Bank...
February 2, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29391916/systemic-lupus-erythematosus-associated-pitfalls-on-18-f-fdg-pet-ct-reactive-follicular-hyperplasia-kikuchi-fujimoto-disease-inflammation-and-lymphoid-hyperplasia-of-the-spleen-mimicking-lymphoma
#17
William Makis, Anthony Ciarallo, Milene Gonzalez-Verdecia, Stephan Probst
Systemic lupus erythematosus (SLE) is associated with a variety of inflammatory processes that can affect the lymph nodes, brain, kidneys, and spleen. We present two patients with SLE in whom SLE-associated conditions complicated interpretation of 18 F-fluoro-2-deoxy-d-glucose (18 F-FDG) positron emission tomography/computed tomography (PET/CT) imaging of the lymph nodes and the spleen. The imaging findings mimicked lymphoma, but histopathological evaluation showed benign processes including reactive follicular hyperplasia in the lymph nodes, Kikuchi-Fujimoto disease in perisplenic lymph nodes, and inflammatory changes and lymphoid hyperplasia in the spleen...
February 2018: Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/29369180/primary-cutaneous-anaplastic-large-cell-lymphoma-a-case-report
#18
Kamil Abed, Zygmunt Stopa, Marta Siewert-Gutowska
RATIONALE: Primary cutaneous anaplastic large-cell lymphoma (C-ALCL) is a rare cancer belonging to the group of primary T-cell lymphoproliferative diseases. C-ALCL is characterized by the presence of single or multiple ulcerated lesions on the skin's surface. PATIENT CONCERNS: This is the case of a 73-year-old man who reported to the Clinic of Cranio-Maxillofacial and Oral Surgery and Implantology, Medical University of Warsaw, owing to a skin tumor in the right parotideomasseteric region, initially diagnosed as discoid lupus erythematosus...
January 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29364334/extrahepatic-manifestations-associated-with-chronic-hepatitis-c-virus-infection
#19
A Flores-Chávez, J A Carrion, X Forns, M Ramos-Casals
Chronic hepatitis C virus (HCV) infection has been associated with both organ-specific and systemic autoimmune diseases, with cryoglobulinemia being the most frequent associated disease. Experimental, virologic, and clinical evidence have demon-strated a close association between HCV infection and some systemic autoimmune diseases, especially Sjögren's syndrome, but also rheumatoid arthritis and lupus. A higher prevalence of hematological processes has also been described in patients with HCV infection, including cytopenias and lymphoproliferative disorders (B-cell lymphoma)...
December 2017: Revista Española de Sanidad Penitenciaria
https://www.readbyqxmd.com/read/29333561/pharmacokinetics-of-the-b-cell-lymphoma-2-bcl-2-inhibitor-venetoclax-in-female-subjects-with-systemic-lupus-erythematosus
#20
Mukul Minocha, Jiewei Zeng, Jeroen K Medema, Ahmed A Othman
BACKGROUND AND OBJECTIVE: Venetoclax is an oral selective Bcl-2 inhibitor approved for the treatment of patients with chronic lymphocytic leukemia with 17p deletion. Mechanistic and preclinical evidence warranted evaluation of venetoclax for the treatment of systemic lupus erythematosus (SLE). This work characterized the pharmacokinetics of venetoclax in female subjects with SLE. METHODS: Single (10-500 mg) and multiple (30-600 mg) escalating doses of venetoclax or matching placebo were evaluated using randomized, double-blind, placebo-controlled designs (6 active and 2 placebo per dose with 73 unique SLE patients enrolled, 25 of whom enrolled twice)...
January 15, 2018: Clinical Pharmacokinetics
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