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https://www.readbyqxmd.com/read/27848183/an-atypical-case-of-late-onset-systemic-lupus-erythematosus-with-systemic-lymphadenopathy-and-severe-autoimmune-thrombocytopenia-neutropenia-mimicking-malignant-lymphoma
#1
Keita Tamaki, Satoko Morishima, Sawako Nakachi, Sakiko Kitamura, Sachie Uchibori, Shouhei Tomori, Taeko Hanashiro, Natsuki Shimabukuro, Iori Tedokon, Kazuho Morichika, Yukiko Nishi, Takeaki Tomoyose, Kennosuke Karube, Takuya Fukushima, Hiroaki Masuzaki
Here, we report a rare case of systemic lupus erythematosus (SLE) with conspicuous manifestation of hematological abnormalities. At onset, the 52-year-old male patient showed systemic lymphadenopathy and splenomegaly, severe autoimmune thrombocytopenia, and autoimmune neutropenia. Bone marrow examination and lymph node biopsy excluded the possibility of malignant lymphoma. Based on laboratory findings, he was finally diagnosed with combined autoimmune cytopenia coupled with SLE. Atypical clinical manifestations of SLE prompted us to explore the possibility of autoimmune lymphoproliferative syndrome (ALPS)...
November 15, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27833448/a-possible-link-between-the-epstein-barr-virus-infection-and-autoimmune-thyroid-disorders
#2
REVIEW
Anna Dittfeld, Katarzyna Gwizdek, Marek Michalski, Romuald Wojnicz
The Epstein-Barr virus (EBV), also known as human herpesvirus 4, is a member of the Herpesviridae virus family. EBV infection can cause infectious mononucleosis (IM) in the lytic phase of EBV's life cycle. Past EBV infection is associated with lymphomas, and may also result in certain allergic and autoimmune diseases. Although potential mechanisms of autoimmune diseases have not been clearly elucidated, both genetic and environmental factors, such as infectious agents, are considered to be responsible for their development...
2016: Central-European Journal of Immunology
https://www.readbyqxmd.com/read/27818202/the-expression-of-bcl-6-in-circulating-follicular-helper-like-t-cells-positively-correlates-with-the-disease-activity-in-systemic-lupus-erythematosus
#3
Xin Huang, Haijing Wu, Hong Qiu, Huilan Yang, Yaxiong Deng, Ming Zhao, Hairong Luo, Xiang Zhou, Yubin Xie, Vera Chan, Chak-Sing Lau, Qianjin Lu
Increased circulating follicular helper-like T cells (cTfh) are reported in systemic lupus erythematosus (SLE) patients. However, whether B-cell lymphoma 6 (Bcl-6) is expressed in cTfh cells remains to be clarified. In this study, we found that the frequencies of CD4(+)CXCR5(hi)PD-1(hi)cTfh, CD4(+)CXCR5(hi)PD-1(hi)ICOS(hi), and CD4(+)CXCR5(hi)PD-1(hi)Bcl-6(+) populations were significantly increased in SLE patients (n=70) when compared with healthy controls (n=48). Surprisingly, only CD4(+)CXCR5(hi)PD-1(hi)Bcl-6(+) cTfh cells, rather than CD4(+)CXCR5(hi)PD-1(hi) population, were positively correlated with SLEDAI and anti-dsDNA antibodies...
November 3, 2016: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/27807018/atypical-lymphocytic-lobular-panniculitis-an-overlap-condition-with-features-of-subcutaneous-panniculitis-like-t-cell-lymphoma-and-lupus-profundus
#4
Alice He, Shawn G Kwatra, Najiyah Kazi, Ronald J Sweren
No abstract text is available yet for this article.
November 2, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27766674/cutaneous-metastasis-of-inflammatory-breast-carcinoma-mimicking-an-erythema-annulare-centrifugum-a-sign-of-locally-recurrent-cancer
#5
V Sabater, F Ferrando, A Morera, L Palomar
Erythema annulare centrifugum (EAC) is a clinical reaction pattern that includes lupus erythematosus, spongiotic dermatitis (particularly pityriasis rosea), pseudolymphoma and cutaneous B-cell lymphoma. However, it can be the result of cutaneous metastasis by an internal carcinoma. We present the case of a 38-year-old woman with bilateral inflammatory breast cancer following multimodal therapy. After chemotherapy, the patient developed EAC on her back, clinically suspect of subacute cutaneous lupus erythematosus...
October 20, 2016: Clinical and Experimental Dermatology
https://www.readbyqxmd.com/read/27759698/occlusive-nonvasculitic-vasculopathy-a-review
#6
Mar Llamas-Velasco, Victoria Alegría, Ángel Santos-Briz, Lorenzo Cerroni, Heinz Kutzner, Luis Requena
We review the most characteristic clinical and histopathologic findings of the cutaneous manifestations of the occlusive nonvasculitic vasculopathic disorders. Clinically, most of these conditions are characterized by retiform purpura. Histopathologic findings consist of occlusion of the vessel lumina with no vasculitis. Different disorders may produce nonvasculitic occlusive vasculopathy in cutaneous blood and lymphatic vessels, including embolization due to cholesterol and oxalate emboli, cutaneous intravascular metastasis from visceral malignancies, atrial myxomas, intravascular angiosarcoma, intralymphatic histiocytosis, intravascular lymphomas, endocarditis, crystal globulin vasculopathy, hypereosinophilic syndrome, and foreign material...
October 18, 2016: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/27730785/a-young-woman-with-panniculitis-and-cytopenia-who-later-developed-coagulopathy
#7
Sanat Phatak, Latika Gupta, Amita Aggarwal
A young woman presented with panniculitis, fever and blood cytopenias, later going on to develop hemophagocytic lymphohistiocytosis. Further evaluation revealed the presence of subcutaneous panniculitis like T-cell lymphoma (SPTCL). With this case we present an approach to panniculitis, a commonly encountered skin manifestation with a wide range of differentials. We discuss the close similarity between lupus erythematous panniculitis (LEP) and lymphoma.
February 2016: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/27564390/comorbidities-in-patients-with-primary-sj%C3%A3-gren-s-syndrome-and-systemic-lupus-erythematosus-a-comparative-registries-based-study
#8
Iñigo Rúa-Figueroa, Mónica Fernández Castro, José L Andreu, Carlos Sanchez-Piedra, Víctor Martínez-Taboada, Alejandro Olivé, Javier López-Longo, José Rosas, María Galindo, Jaime Calvo-Alén, Antonio Fernández-Nebro, Fernando Alonso, Beatriz Rodríguez-Lozano, Jesús Alberto García Vadillo, Raúl Menor, Francisco Javier Narváez, Celia Erausquin, Ángel García-Aparicio, Eva Tomero, Sara Manrique-Arija, Loreto Horcada, Esther Uriarte, Susana Gil, Ricardo Blanco, Ruth López-González, Alina Boteanu, Mercedes Freire, Carlos Galisteo, Manuel Rodríguez-Gómez, Elvira Díez-Álvarez, José M Pego-Reigosa
OBJECTIVE: To compare the prevalence of the main comorbidities in 2 large cohorts of patients with primary Sjögren's syndrome (SS) and systemic lupus erythematosus (SLE), with a focus on cardiovascular (CV) diseases. METHODS: This was a cross-sectional multicenter study where the prevalence of more relevant comorbidities in 2 cohorts was compared. Patients under followup from SJOGRENSER (Spanish Rheumatology Society Registry of Primary SS) and RELESSER (Spanish Rheumatology Society Registry of SLE), and who fulfilled the 2002 American-European Consensus Group and 1997 American College of Rheumatology classification criteria, respectively, were included...
August 26, 2016: Arthritis Care & Research
https://www.readbyqxmd.com/read/27537853/18f-fdg-pet-ct-for-identifying-the-potential-causes-and-extent-of-secondary-hemophagocytic-lymphohistiocytosis
#9
Leilei Yuan, Ying Kan, Jacqui K Meeks, Daqing Ma, Jigang Yang
PURPOSE: We aimed to evaluate the value of 18F-FDG positron emission tomography/computed tomography (PET/CT) for identifying the possible causes of secondary hemophagocytic lymphohistiocytosis (HLH). METHODS: Forty-five cases (17 female, 28 male; age, 17-79 years) with secondary HLH were included. The standard of reference for diagnosis in all patients was a combination of histology, clinical results (medical history, physical examination, and laboratory test results), and follow-up imaging for at least 12 months...
September 2016: Diagnostic and Interventional Radiology: Official Journal of the Turkish Society of Radiology
https://www.readbyqxmd.com/read/27520362/cytoprotective-pathways-in-the-vascular-endothelium-do-they-represent-a-viable-therapeutic-target
#10
Justin C Mason
The vascular endothelium is a critical interface, which separates the organs from the blood and its contents. The endothelium has a wide variety of functions and maintenance of endothelial homeostasis is a multi-dimensional active process, disruption of which has potentially deleterious consequences if not reversed. Vascular injury predisposes to endothelial apoptosis, dysfunction and development of atherosclerosis. Endothelial dysfunction is an end-point, a central feature of which is increased ROS generation, a reduction in endothelial nitric oxide synthase and increased nitric oxide consumption...
August 9, 2016: Vascular Pharmacology
https://www.readbyqxmd.com/read/27484255/hemophagocytic-lymphohistiocytosis-with-leukoencephalopathy-in-a-patient-with-dermatomyositis-accompanied-with-peripheral-t-cell-lymphoma-a-case-report
#11
Satoru Teshigawara, Yoshinori Katada, Yuichi Maeda, Maiko Yoshimura, Eriko Kudo-Tanaka, Soichiro Tsuji, Yoshinori Harada, Masato Matsushita, Shiro Ohshima, Kotaro Watanabe, Takahiro Kumode, Yoshihiko Hoshida, Yukihiko Saeki
BACKGROUND: Hemophagocytic lymphohistiocytosis associated with autoimmune diseases is seen in patients with systemic juvenile idiopathic arthritis, adult-onset Still's disease, and systemic lupus erythematosus, whereas it is rarely seen in patients with dermatomyositis. In addition, central nervous system involvement with dermatomyositis is rare. To the best of our knowledge, this is the first case of hemophagocytic lymphohistiocytosis complicated by leukoencephalopathy in a patient with dermatomyositis accompanied with peripheral T-cell lymphoma...
2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27476075/spleen-tyrosine-kinase-a-crucial-player-and-potential-therapeutic-target-in-renal-disease
#12
Terry King-Wing Ma, Stephen P McAdoo, Frederick Wai-Keung Tam
Spleen tyrosine kinase (Syk), a 72 kDa cytoplasmic non-receptor protein-tyrosine kinase, plays an important role in signal transduction in a variety of cell types. Ever since its discovery in the early 1990s, there has been accumulating evidence to suggest a pathogenic role of Syk in various allergic disorders, autoimmune diseases and malignancies. Additionally, there is emerging data from both pre-clinical and clinical studies that Syk is implicated in the pathogenesis of proliferative glomerulonephritis (GN), including anti-glomerular basement membrane disease, anti-neutrophil cytoplasmic antibody-associated GN, lupus nephritis and immunoglobulin A nephropathy (IgAN)...
2016: Nephron
https://www.readbyqxmd.com/read/27436361/rock2-signaling-is-required-to-induce-a-subset-of-t-follicular-helper-cells-through-opposing-effects-on-stats-in-autoimmune-settings
#13
Jonathan M Weiss, Wei Chen, Melanie S Nyuydzefe, Alissa Trzeciak, Ryan Flynn, James R Tonra, Suzana Marusic, Bruce R Blazar, Samuel D Waksal, Alexandra Zanin-Zhorov
Rho-associated kinase 2 (ROCK2) determines the balance between human T helper 17 (TH17) cells and regulatory T (Treg) cells. We investigated its role in the generation of T follicular helper (TFH) cells, which help to generate antibody-producing B cells under normal and autoimmune conditions. Inhibiting ROCK2 in normal human T cells or peripheral blood mononuclear cells from patients with active systemic lupus erythematosus (SLE) decreased the number and function of TFH cells induced by activation ex vivo. Moreover, inhibition of ROCK2 activity decreased the abundance of the transcriptional regulator Bcl6 (B cell lymphoma 6) and increased that of Blimp1 by reducing the binding of signal transducer and activator of transcription 3 (STAT3) and increasing that of STAT5 to the promoters of the genes Bcl6 and PRDM1, respectively...
July 19, 2016: Science Signaling
https://www.readbyqxmd.com/read/27435953/a20-snp-rs77191406-may-be-related-to-secondary-cancer-for-rheumatoid-arthritis-and-systemic-lupus-erythematosus-patients
#14
Lihua Zhu, Lingling Zhou, Liang Wang, Zhaoxia Li, Shuai Lu, Lijian Yang, Shaohua Chen, Bo Li, Xiuli Wu, Yi Zhou, Yangqiu Li
AIM: An increased risk for malignancy for rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) patients may be related to factors that play a critical role in the regulation of T-cell activation. A20 is an important negative immunoregulatory factor that was found to be associated with lymphoma and the development of numerous solid tumors. Previous studies have implicated the A20 locus in RA susceptibility. In this study, we investigated polymorphisms in the A20 3' UTR and explored whether there was an association between these polymorphisms and malignancy risk in autoimmune diseases...
July 20, 2016: Asia-Pacific Journal of Clinical Oncology
https://www.readbyqxmd.com/read/27429984/autoimmune-inflammatory-arthritis-associated-lymphomas-who-is-at-risk
#15
REVIEW
Sujani Yadlapati, Petros Efthimiou
Specific autoimmune and inflammatory rheumatic diseases have been associated with an increased risk of malignant lymphomas. Conditions such as rheumatoid arthritis (RA), primary Sjögren's syndrome (pSS), systemic lupus erythematosus (SLE), dermatomyositis, and celiac disease have been consistently linked to malignant lymphomas. Isolated cases of lymphomas associated with spondyloarthropathies and autoinflammatory diseases have also been reported. Direct association between autoimmunity and lymphomagenesis has been reinforced by large epidemiological studies...
2016: BioMed Research International
https://www.readbyqxmd.com/read/27429760/recognizing-igg4-related-tubulointerstitial-nephritis
#16
REVIEW
Shawna Mann, Michael A Seidman, Sean J Barbour, Adeera Levin, Mollie Carruthers, Luke Y C Chen
PURPOSE OF THE REVIEW: Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory disorder affecting nearly all organs, including the kidney. Tubulointerstitial nephritis (IgG4-TIN) is the most common form of IgG4-related kidney disease (IgG4-RKD) and is the focus of this concise review. OBJECTIVE: The study aims to describe when IgG4-TIN should be suspected and to summarize the diagnosis, treatment, and natural history of the disease. SOURCES OF INFORMATION: Ovid MEDLINE, Google Scholar, and PubMed were searched for full-text English language articles up to January 2016...
2016: Canadian Journal of Kidney Health and Disease
https://www.readbyqxmd.com/read/27408688/case-report-kikuchi-fujimoto-disease-a-diagnostic-and-therapeutic-dilemma-following-pretransplant-nephrectomy-for-a-2-35-kg-kidney
#17
Arvind P Ganpule, Jaspreet Singh Chabra, Abhishek G Singh, Gopal R Tak, Shailesh Soni, Ravindra Sabnis, Mahesh Desai
Kikuchi-Fujimoto disease (KFD) is an extremely rare disease with a worldwide distribution and higher prevalence in Asians. It is a benign and self-limiting disorder, characterized by regional cervical lymphadenopathy accompanied with mild fever and night sweats. Lymph node histopathology is diagnostic and treating physicians should be aware of this entity as it may mimic other systemic diseases like systemic lupus erythematosus, tuberculosis, malignant lymphoma, and more rarely adenocarcinoma. Key features on lymph node biopsy are fragmentation, necrosis and karyorrhexis...
2016: F1000Research
https://www.readbyqxmd.com/read/27389558/characteristic-distribution-pattern-of-cd30-positive-cytotoxic-t-cells-aids-diagnosis-of-kikuchi-fujimoto-disease
#18
Tetsuya Tabata, Katsuyoshi Takata, Tomoko Miyata-Takata, Yasuharu Sato, Shin Ishizawa, Tomoyoshi Kunitomo, Keina Nagakita, Nobuhiko Ohnishi, Kohei Taniguchi, Mai Noujima-Harada, Yoshinobu Maeda, Mitsune Tanimoto, Tadashi Yoshino
INTRODUCTION: Histiocytic necrotizing lymphadenitis (or Kikuchi-Fujimoto disease) frequently occurs in Asian young adult females and typically presents as cervical lymphadenopathy with unknown etiology. Although large immunoblasts frequently appear in Kikuchi-Fujimoto disease, the diffuse infiltration of these cells can cause difficulty in establishing a differential diagnosis from lymphoma. In such cases, CD30 immunostaining may be used; however, the extent or distribution pattern of CD30-positive cells in Kikuchi-Fujimoto disease remains largely unknown...
July 6, 2016: Applied Immunohistochemistry & Molecular Morphology: AIMM
https://www.readbyqxmd.com/read/27380773/atypical-clinical-presentation-of-primary-and-secondary-cutaneous-follicle-center-lymphoma-fcl-on-the-head-characterized-by-macular-lesions
#19
Cesare Massone, Regina Fink-Puches, Lorenzo Cerroni
BACKGROUND: Primary cutaneous follicle center lymphoma (pcFCL) usually presents with reddish nodules, plaques, and tumors on the head and neck or the back. OBJECTIVE: We sought to describe a peculiar clinical presentation of pcFCL and secondary cutaneous follicle center lymphoma (FCL). METHODS: We report a series of 13 patients (7 male and 6 female; median age 58 years, mean age 58.2 years, age range 26-83 years) with either pcFCL (11 patients) or secondary cutaneous FCL (2 patients) presenting with lesions on the head deviating from the classic clinical manifestations characterized by plaques and tumors...
July 2, 2016: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/27365372/chronic-myelomonocytic-leukemia-mimicking-hematologic-systemic-lupus-erythematosus
#20
C A Moura, M Santiago, J Neto, V H Gomes de Souza, C Geraldo Moura
The relationship between primary hematologic disease and rheumatologic manifestations is well known, especially acute lymphocytic leukemia, lymphomas, plasma cell dyscrasias and myelodysplastic syndrome (MDS). Currently, more has been described about autoimmune manifestations in chronic myelomonocytic leukemia (CMML). Many different clinical scenarios may lead a patient with MDS/CMML initially to seek a rheumatological unit. Autoimmune features such as polymyalgia rheumatic symptoms, myositis, neutrophilic dermatosis, cutaneous vasculitis and positive antinuclear antibodies (ANA) are some examples of clinical presentation of MDS/CMML...
June 30, 2016: Lupus
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