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Lymphoproliferative

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https://www.readbyqxmd.com/read/29345341/novel-insights-into-fas-defects-underlying-autoimmune-lymphoproliferative-syndrome-revealed-by-studies-in-consanguineous-patients
#1
REVIEW
Imen Ben-Mustapha, Nourhen Agrebi, Mohamed-Ridha Barbouche
Autoimmune lymphoproliferative syndrome (ALPS) is a primary immunodeficiency disease due to impaired Fas-Fas ligand apoptotic pathway. It is characterized by chronic nonmalignant, noninfectious lymphadenopathy and/or splenomegaly associated with autoimmune manifestations primarily directed against blood cells. Herein, we review the heterogeneous ALPS molecular bases and discuss recent findings revealed by the study of consanguineous patients. Indeed, this peculiar genetic background favored the identification of a novel form of AR ALPS-FAS associated with normal or residual protein expression, expanding the spectrum of ALPS types...
December 27, 2017: Journal of Leukocyte Biology
https://www.readbyqxmd.com/read/29344502/orbital-epstein-barr-virus-positive-polymorphic-b-cell-lymphoproliferative-disorder-in-an-apparently-immunocompetent-woman
#2
Michael D Abendroth, Michael G Bayerl, Michael J Wilkinson, David F Claxton, Charles S Specht
We report a rare case of Epstein-Barr virus (EBV)-positive polymorphic B-cell lymphoproliferative disorder (LPD) involving the lacrimal gland of a 28-year-old, apparently immunocompetent woman. She presented with a chief complaint of orbital swelling and tenderness and was found to have a lesion involving the right lacrimal gland and distal superior and lateral rectus muscles. Histology of the lesion revealed histiocytes with pleomorphic nuclei, reactive lymphocytes, and scattered cells that resembled the Reed-Sternberg (R-S) cells of classical Hodgkin lymphoma...
December 2017: Ocular Oncology and Pathology
https://www.readbyqxmd.com/read/29342998/-x-linked-immunodeficiency-with-magnesium-defect-epstein-barr-virus-infection-and-neoplasia-report-of-a-family-and-literature-review
#3
T Y He, Y Xia, C G Li, C R Li, Z X Qi, J Yang
Objective: To investigate the clinical features and genetic characteristics of cases with X-linked immunodeficiency with magnesium defect, Epstein-Barr virus (EBV) infection, and neoplasia (XMEN). Methods: Characteristics of clinical material, immunological data and gene mutation of two cases with XMEN in the same family in China were retrospectively analyzed. The related reports literature were searched by using search terms'MAGT1 gene'or'XMEN'. Results: The proband, a 2-year-eight-month old boy, was admitted due to 'Urine with deepened color for two days and yellow stained skin for one day'...
January 2, 2018: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29342279/incidence-and-predictors-of-post-transplant-lymphoproliferative-disease-after-kidney-transplantation-during-adulthood-and-childhood-a-registry-study
#4
Anna Francis, David W Johnson, Armando Teixeira-Pinto, Jonathan C Craig, Germaine Wong
Background: Differences in the epidemiology of post-transplant lymphoproliferative disease (PTLD) between adult and paediatric kidney transplant recipients remain unclear. Methods: Using the Australian and New Zealand Dialysis and Transplant Registry (1963-2015), the cumulative incidences of PTLD in children (age <20 years) and adults were calculated using a competing risk of death model and compared with age-matched population-based data using standardized incidence ratios (SIRs)...
January 12, 2018: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/29341892/premature-aging-in-behavior-and-immune-functions-in-tyrosine-hydroxylase-haploinsufficient-female-mice-a-longitudinal-study
#5
A Garrido, J Cruces, N Ceprián, C Hernández-Sánchez, M De la Fuente
Aging is accompanied by impairment in the nervous, immune, and endocrine systems as well as in neuroimmunoendocrine communication. In this context, there is an age-related alteration of the physiological response to acute stress, which is modulated by catecholamine (CA), final products of the sympathetic-adreno-medullary axis. The involvement of CA in essential functions of the nervous system is consistent with the neuropsychological deficits found in mice with haploinsufficiency (hemizygous; HZ) of tyrosine hydroxylase (TH) enzyme (TH-HZ)...
January 13, 2018: Brain, Behavior, and Immunity
https://www.readbyqxmd.com/read/29341283/mogamulizumab-induced-phosensitivity-in-patients-with-mycosis-fungoides-and-other-t-cell-neoplasms
#6
Y Masuda, K Tatsuno, S Kitano, H Miyazawa, J Ishibe, M Aoshima, T Shimauchi, T Fujiyama, T Ito, Y Tokura
BACKGROUND: Mogamulizumab (Mog) is a defucosylated, therapeutic monoclonal antibody, targeting CCR4, and was first approved in Japan for the treatment of adult T cell leukaemia/lymphoma (ATLL), followed by cutaneous T cell lymphoma and peripheral T cell lymphoma. OBJECTIVE: To retrospectively investigate development of photosensitivity in patients with mycosis fungoides and other T cell neoplasms after treatment with Mog. METHODS: We treated 7 cutaneous lymphoma patients with Mog...
January 16, 2018: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/29340266/18f-fdg-pet-ct-in-a-patient-with-methotrexate-associated-lymphoproliferative-disorder
#7
Hayato Shimizu, Hiroaki Nishioka
18F-FDG PET-CT clearly demonstrated the disease activity of MTX-LPD.
January 2018: Journal of General and Family Medicine
https://www.readbyqxmd.com/read/29336635/lymphomatoid-granulomatosis-in-a-14-year-old-boy-with-trisomy-21-and-history-of-b-lymphoblastic-leukemia-lymphoma
#8
Anna Paulina Matynia, Sherrie L Perkins, David Li
BACKGROUND: Lymphomatoid granulomatosis is a EBV-driven lymphoproliferative disorder that has been reported in association with immunodeficiency, but only exceptionally in patients with hematopoietic malignancy. CASE REPORT: A 14-year-old boy with trisomy-21 and a history of B-lymphoblastic leukemia/lymphoma (B-ALL) diagnosed 1.5 years prior, on maintenance chemotherapy, presented with fever and respiratory symptoms. Chest X-ray revealed right-lower-lobe consolidation...
January 16, 2018: Fetal and Pediatric Pathology
https://www.readbyqxmd.com/read/29333414/differential-diagnosis-of-primary-cutaneous-cd4-small-medium-t-cell-lymphoproliferative-lesions-a-report-of-three-cases
#9
Hyun-Jung Kim, Jae-Ho Han, Soo Kee Min
No abstract text is available yet for this article.
December 2017: Blood Research
https://www.readbyqxmd.com/read/29330756/-rheumatoid-symptoms-in-patients-with-hematologic-neoplasms
#10
REVIEW
S Kleinert, P La Rosée, K Krüger
Paraneoplastic syndromes in lymphatic or myeloid neoplasms can present with musculoskeletal symptoms, vasculitis-like or febrile symptoms. Hematologic diseases are also associated with rheumatic diseases whereas inflammatory rheumatic diseases are often associated with an increased risk for lymphoproliferative disease. Atypical disease characteristics, lack of disease-specific antibodies or therapeutic response are red flags for diagnosing paraneoplastic or coexistent malignant diseases. New onset of systemic symptoms, worsening of general condition, night sweats or weight loss need to be considered during follow-up and differential diagnostics...
October 2017: Zeitschrift Für Rheumatologie
https://www.readbyqxmd.com/read/29330115/pediatric-onset-evans-syndrome-heterogeneous-presentation-and-high-frequency-of-monogenic-disorders-including-lrba-and-ctla4-mutations
#11
Caroline Besnard, Eva Levy, Nathalie Aladjidi, Marie-Claude Stolzenberg, Aude Magerus-Chatinet, Olivier Alibeu, Patrick Nitschke, Stéphane Blanche, Olivier Hermine, Eric Jeziorski, Judith Landman-Parker, Guy Leverger, Nizar Mahlaoui, Gérard Michel, Isabelle Pellier, Felipe Suarez, Isabelle Thuret, Geneviève de Saint-Basile, Capucine Picard, Alain Fischer, Bénédicte Neven, Frédéric Rieux-Laucat, Pierre Quartier
Evans syndrome (ES) is defined by the combination of autoimmune hemolytic anemia and immune thrombocytopenia. Clinical presentation includes manifestations of immune dysregulation, found in primary immune deficiencies, autoimmune lymphoproliferative syndrome with FAS (ALPS-FAS), Cytotoxic T Lymphocyte Antigen-4 (CTLA-4) and Lipopolysaccharide-Responsive vesicle trafficking Beige-like and Anchor protein (LRBA) defects. We report the clinical history and genetic results of 18 children with ES after excluding ALPS-FAS...
January 9, 2018: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/29327533/post-transplant-lymphoproliferative-disorder
#12
G S Chowdhary, Malav Jhala
Post-transplant lymphoproliferative disorders (PTLDs) are life-threatening complications of solid-organ transplantation and bone marrow transplantation leading to a high mortality. PTLD represents a heterogeneous group of lymphoproliferative diseases. They become clinically relevant because of the expansion of transplantation medicine together with the development of potent immunosuppressive drugs associated now with long survival. The risk of PTLD is highest in the early post-transplant period, but the cumulative risk increases with time...
December 2017: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/29326801/waldenstrom-s-macroglobulinemia-an-update
#13
REVIEW
Maddalena Mazzucchelli, Anna Maria Frustaci, Marina Deodato, Roberto Cairoli, Alessandra Tedeschi
Waldenstrom Macroglobulinemia is a rare lymphoproliferative disorder with distinctive clinical features. Diagnostic and prognostic characterisation in WM significantly changed with the discovery of two molecular markers: MYD88 and CXCR4. Mutational status of these latter influences both clinical presentation and prognosis and demonstrated therapeutic implications. Treatment choice in Waldenstrom disease is strictly guided by patients age and characteristics, specific goals of therapy, the necessity for rapid disease control, the risk of treatment-related neuropathy, disease features, the risk of immunosuppression or secondary malignancies and potential for future autologous stem cell transplantation...
2018: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/29325246/-diagnostic-significance-of-lymph-node-core-needle-biopsy-for-lymphoproliferative-disease-a-clinicopathologic-study-of-1-013-cases
#14
R F Huang, W Y Zhang, W P Liu, S Zhao, Y X Ye, H Sun, L M Gao, J C Wang, Q P Yang
Objective: To study the clinicopathologic features of lymphoproliferative disease by lymph node core needle biopsy(CNB)and to evaluate the diagnostic significance of CNB for lymphoproliferative disease. Methods: The annual distribution, entity constitute, clinical finding, gross feature, morphologic change, affiliate study and repeat biopsy diagnosis of 1 013 cases of lymph node CNB diagnosed at West China Hospital of Sichuan University from January 2009 to December 2015 were investigated. Results: (1) Proportion of lymph node CNB in total amount of biopsy specimens increased from 0...
January 8, 2018: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/29324572/epstein-barr-virus-ebv-induced-b-cell-lymphoproliferative-disorder-mimicking-the-recurrence-of-ebv-associated-hemophagocytic-lymphohistiocytosis
#15
Yuki Yatsushiro, Takuro Nishikawa, Aki Saito, Yozo Nakazawa, Ken-Ichi Imadome, Shunsuke Nakagawa, Yuichi Kodama, Yasuhiro Okamoto, Hirokazu Kanegane, Yoshifumi Kawano
We describe the case of a 23-month-old male infant with Epstein-Barr virus (EBV)-associated lymphoproliferative disorder, which mimicked the recurrence of EBV-associated hemophagocytic lymphohistiocytosis. Chemotherapy with dexamethasone, etoposide, and cyclosporine resolved fever, hepatosplenomegaly, and pancytopenia. However, on day 81 of illness, the patient developed similar symptoms. Plasma EBV-DNA levels markedly increased again, but no T-cell clonality was observed. B cells were identified to be infected with EBV...
January 10, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29324208/solid-organ-transplantation-in-patients-with-a-history-of-lymphoma
#16
Philip J Bierman
There is an increasing number of long-term survivors of Hodgkin and non-Hodgkin lymphoma. These people may have a need for subsequent solid organ transplantation, often as a result of late effects of their lymphoma treatment. There is abundant literature demonstrating that patients with a history of lymphoma are appropriate candidates for solid organ transplantation. Long-term survival without relapse and with a functioning graft is possible. Patients with a history of post-transplantation lymphoproliferative disorders and patients who have received a prior hematopoietic stem-cell transplantation may also be candidates...
January 2018: Journal of Oncology Practice
https://www.readbyqxmd.com/read/29322833/primary-genitourinary-lymphoma-on-fdg-pet
#17
Nikita Naik, Michael Lin, Peter Lin
Primary extra nodal lymphomatous involvement of the genitourinary tract is rare and secondary extra-nodal involvement in disseminated disease occurs more frequently. Imaging of metabolic activity with 2-[fluorine-18] fluoro-2-deoxy-d-glucose (FDG) used in positron emission tomography (PET) facilitates the identification of these extra-nodal sites of disease, particularly in the absence of structural lesions on conventional imaging modalities. Primary extra-nodal lymphoma affecting the genitourinary system is often caused by high-grade Non-Hodgkin's Lymphoma (NHL) with the most common subtype being Diffuse Large B-Cell Lymphoma (DLBCL)...
January 11, 2018: British Journal of Radiology
https://www.readbyqxmd.com/read/29318585/dermoscopic-characterization-of-cutaneous-lymphomas-a-pilot-survey
#18
Grant K Ghahramani, Kirsten E Goetz, Vincent Liu
BACKGROUND: While substantial dermoscopic analysis of melanocytic lesions has been performed, dermoscopic characterization of cutaneous lymphoid proliferations has been limited. Cutaneous lymphoma, particularly early mycosis fungoides (MF) and its variants, is often challenging to clinically and pathologically distinguish from inflammatory processes of the skin. This study aimed to survey the dermoscopic findings of cutaneous lymphomas and to discern whether any patterns might potentially serve as specific signatures...
January 10, 2018: International Journal of Dermatology
https://www.readbyqxmd.com/read/29317997/experience-with-ibrutinib-for-first-line-use-in-patients-with-chronic-lymphocytic-leukemia
#19
REVIEW
Gilad Itchaki, Jennifer R Brown
Ibrutinib is the first in-class, orally administered, Bruton's tyrosine kinase (BTK) inhibitor that abrogates the critical signaling downstream of the B-cell receptor (BCR). This signaling is required for B-cell survival, proliferation and interaction with the microenvironment. Ibrutinib proved active in preclinical models of lymphoproliferative diseases and achieved impressive response rates in heavily pretreated relapsed and refractory (R/R) patients with chronic lymphocytic leukemia (CLL). Ibrutinib prolonged survival compared to standard therapy and mitigated the effect of most poor prognostic factors in CLL, thus becoming the main therapeutic option in high-risk populations...
January 2018: Therapeutic Advances in Hematology
https://www.readbyqxmd.com/read/29316199/usefulness-of-the-cllflow-score
#20
Marc Sorigue, Mireia Franch-Sarto, Edurne Sarrate, Jordi Junca
BACKGROUND: The CLLflow score was recently suggested as an improvement over the Moreau score for the diagnosis and classification of B-cell lymphoproliferative disorders (B-LPD). METHODS: We determined the CLLflow score in peripheral blood or bone marrow of a series of cases with an inconclusive immunophenotype, including samples with a Moreau score of 3 (n=52) and CD5-positive with a score of 2 (n=38). As controls, B-LPD with a Moreau score of 0-1 (n=95), CD5-negative score 2 (n=24) and score 4-5 (i...
January 6, 2018: Cytometry. Part B, Clinical Cytometry
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