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Lymphoproliferative syndromes

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https://www.readbyqxmd.com/read/29779211/cutaneous-sarcoidosis-a-new-subset-in-the-spectrum-of-paraneoplastic-dermatoses
#1
M El-Khalawany, A Mosbeh, S Aboeldahab, S Ali
BACKGROUND: Sarcoidosis is a well-described disease that can be associated with various malignancies; however, this correlation is still not fully clarified. AIM: To determine the clinical and histological features, demographic characteristics, and prognosis of patients diagnosed with paraneoplastic sarcoidosis (PS). METHODS: This observational cohort prospective study included all patients diagnosed as cutaneous sarcoidosis. All patients were monitored for therapeutic response, prognosis, and any associated diseases or malignancies...
May 20, 2018: Clinical and Experimental Dermatology
https://www.readbyqxmd.com/read/29777376/comprehensive-molecular-diagnosis-of-epstein-barr-virus-associated-lymphoproliferative-diseases-using-next-generation-sequencing
#2
Shintaro Ono, Manabu Nakayama, Hirokazu Kanegane, Akihiro Hoshino, Saeko Shimodera, Hirofumi Shibata, Hisanori Fujino, Takahiro Fujino, Yuta Yunomae, Tsubasa Okano, Motoi Yamashita, Takahiro Yasumi, Kazushi Izawa, Masatoshi Takagi, Kohsuke Imai, Kejian Zhang, Rebecca Marsh, Capucine Picard, Sylvain Latour, Osamu Ohara, Tomohiro Morio
Epstein-Barr virus (EBV) is associated with several life-threatening diseases, such as lymphoproliferative disease (LPD), particularly in immunocompromised hosts. Some categories of primary immunodeficiency diseases (PIDs) including X-linked lymphoproliferative syndrome (XLP), are characterized by susceptibility and vulnerability to EBV infection. The number of genetically defined PIDs is rapidly increasing, and clinical genetic testing plays an important role in establishing a definitive diagnosis. Whole-exome sequencing is performed for diagnosing rare genetic diseases, but is both expensive and time-consuming...
May 18, 2018: International Journal of Hematology
https://www.readbyqxmd.com/read/29770706/intestinal-transplants-review-of-normal-imaging-appearance-and-complications
#3
Bashir Hakim, Daniel T Myers, Todd R Williams, Shunji Nagai, John Bonnett
Intestinal transplant (IT) is one of the least common forms of organ transplant but is increasing both in volume of cases and number of centers performing intestinal transplants, with the busiest centers in North America and Europe. IT can be performed in isolation or as part of a multivisceral transplant (MVT). Intestinal failure either in the form of short gut syndrome or functional bowel problems is the primary indication for IT. The normal post-surgical anatomy can be variable due to both recipient anatomy in regard to amount of residual bowel and status of native vasculature as well as whether the transplant is isolated or part of an multivisceral transplant...
May 17, 2018: British Journal of Radiology
https://www.readbyqxmd.com/read/29766733/-hemophagocytic-lymfohistiocytosis-in-adults-review-and-case-report
#4
Jiří Šrámek, Thomas Karvunidis, Daniel Lysák, Martin Harazim, Michal Karas, Pavel Jindra
Hemophagocytic lymfohistiocytosis (HLH) is rare, life-threatening condition, characterized by excessive activation of immune system with subsequent proinflammatory state resulting in multiorgan failure. Most frequently, it appears in infancy as a primary disorder caused by mutation of immune-regulatory genes. Increasingly, HLH is being diagnosed as a secondary - adult - form, which occurs as a result of aberrant immune response. Viral or bacterial systemic infections, malignancy with a predominance of lymphoproliferative disorders and autoimmune diseases are the most common triggers...
2018: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/29761072/sezary-syndrome-manifesting-as-posttransplant-lymphoproliferative-disorder
#5
Thanh-Phuong Afiat, Xiaohui Zhang, Hailing Zhang, Ernesto Ayala, Ling Zhang, Lubomir Sokol
Posttransplant lymphoproliferative disorders (PTLDs) of T-cell orgin are rare biologically heterogeneous diseases of mature lymphoid cells manifesting in immunosuppressed patients. Only a few cases of mycosis fungoides diagnosed post allogeneic hematopoietic cell transplant (alloHSCT) have been described so far. We present a patient with myelodysplastic syndrome (MDS) post matched unrelated donor alloHSCT who was on long-term immunosuppressive therapy due to graft versus host disease. Three years after an alloHSCT, she developed generalized erythroderma and peripheral blood lymphocytosis...
2018: Leukemia Research Reports
https://www.readbyqxmd.com/read/29755712/lymphoproliferative-syndromes-associated-with-human-herpesvirus-6a-and-human-herpesvirus-6b
#6
REVIEW
Eva Eliassen, Gerhard Krueger, Mario Luppi, Dharam Ablashi
Human herpesvirus 6A and 6B (HHV-6A and HHV-6B) have been noted since their discovery for their T-lymphotropism. Although it has proven difficult to determine the extent to which HHV-6A and HHV-6B are involved in the pathogenesis of many diseases, evidence suggests that primary infection and reactivation of both viruses may induce or contribute to the progression of several lymphoproliferative disorders, ranging from benign to malignant and including infectious mononucleosis-like illness, drug induced hypersensitivity syndrome/drug reaction with eosinophilia and systemic symptoms (DIHS/DRESS), and nodular sclerosis Hodgkin's lymphoma...
2018: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/29743402/-durable-remission-attained-with-rituximab-therapy-in-a-patient-with-acquired-von-willebrand-syndrome-associated-with-cd20-positive-lymphoproliferative-disorder
#7
Honami Kurahashi, Yoshinari Kawabata, Yoshihiro Michishita, Atsushi Kitabayashi, Takahiro Kobayashi, Akihiro Kitadate, Naoto Takahashi
A 61-year-old female with no history of bleeding was admitted to our hospital owing to persistent bleeding after the left knee joint injection and activated partial thromboplastin time prolongation. Subsequent coagulation tests revealed a critically declined level of the von Willebrand factor (VWF) antigen (<10%) and activity (<10%) measurement besides a significantly declined factor VIII activity (4%). Despite diagnosing her with acquired von Willebrand syndrome (AvWS) and managing her bleeding with desmopressin acetate hydrate (DDAVP), we could not precisely make a definitive diagnosis the underlying disorder...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/29732477/effectiveness-of-rituximab-containing-treatment-regimens-in-idiopathic-multicentric-castleman-disease
#8
Yujun Dong, Lu Zhang, Lin Nong, Lihong Wang, Zeyin Liang, Daobin Zhou, David C Fajgenbaum, Hanyun Ren, Jian Li
Human herpes virus type 8 (HHV-8)-negative, idiopathic multicentric Castleman disease (iMCD) is a rare lymphoproliferative disease often involving constitutional symptoms, cytopenias, and multiple organ system dysfunction. In China, the majority of MCD cases are HHV-8 negative. Given that siltuximab, the only FDA-approved treatment for iMCD is not available in China; rituximab- and cyclophosphamide-containing regimens are often used in the treatment of Chinese iMCD patients. To evaluate the efficacy of rituximab in this rare and heterogeneous disease, clinical and pathological data from 27 cases of iMCD were retrospectively analyzed from two large medical centers in China...
May 7, 2018: Annals of Hematology
https://www.readbyqxmd.com/read/29728131/multicentric-castleman-s-disease-in-human-immunodeficiency-virus-infection-two-case-reports
#9
Amanda Caroline Ribeiro Sales, Valter Romão de Souza Junior, Marta Iglis de Oliveira, Claudia Azevedo Braga Albuquerque, Evônio de Barros Campelo Júnior, Paulo Sérgio Ramos de Araújo
BACKGROUND: Castleman's Disease is a rare B-cell lymphoproliferative disease. It is mostly benign and is characterized by non-neoplastic lymph node hypertrophy, associated with infection by human herpesvirus-8 in people with the human immunodeficiency virus/acquired immunodeficiency syndrome. Although the unicentric or localized form presents as benign, the multifocal form can manifest severe systemic symptoms. We report two unusual cases of men presenting cervical enlarged lymph nodes that were believed to be infectious...
May 5, 2018: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/29707401/a-rare-but-fascinating-disorder-case-collection-of-patients-with-schnitzler-syndrome
#10
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/29692937/ebv-associated-lymphoproliferative-disorder-and-hemophagocytic-lymphohistiocytosis-in-a-patient-with-severe-celiac-disease
#11
John Jacob Kinross-Wright, Kalyan Chakravarthy Potu, Brandy Pownell, Randall Lamfers, Jonathan S Bleeker
Background: Epstein-Barr virus- (EBV-) associated lymphoproliferative disease (LPD) is a rare condition, usually occurring in immunocompromised patients. We report a case of EBV-associated LPD in a patient with severe celiac disease, the first report to describe this syndrome in a patient with this diagnosis. Case Summary: A 69-year-old Caucasian woman with recent diagnosis of celiac sprue presented to our hospital with persistent diarrhea, abdominal pain, weight loss, and fatigue despite adherence to gluten-free diet for a number of weeks prior to presentation...
2018: Case Reports in Hematology
https://www.readbyqxmd.com/read/29686686/autoimmune-lymphoproliferative-syndrome-fas-patients-have-an-abnormal-regulatory-t-cell-treg-phenotype-but-display-normal-natural-treg-suppressive-function-on-t-cell-proliferation
#12
Fabienne Mazerolles, Marie-Claude Stolzenberg, Olivier Pelle, Capucine Picard, Benedicte Neven, Alain Fischer, Aude Magerus-Chatinet, Frederic Rieux-Laucat
Objective: Autoimmune lymphoproliferative syndrome (ALPS) with FAS mutation (ALPS-FAS) is a nonmalignant, noninfectious, lymphoproliferative disease with autoimmunity. Given the central role of natural regulatory T cells (nTregs) in the control of lymphoproliferation and autoimmunity, we assessed nTreg-suppressive function in 16 patients with ALPS-FAS. Results: The proportion of CD25high CD127low Tregs was lower in ALPS-FAS patients than in healthy controls. This subset was correlated with a reduced CD25 expression in CD3+ CD4+ T cells from ALPS patients and thus an abnormally low proportion of CD25high FOXP3+ Helios+ T cells...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29682404/possible-association-of-multicentric-castleman-s-disease-with-autoimmune-lymphoproliferative-syndrome
#13
REVIEW
Hiroyuki Minemura, Yoshinori Tanino, Kazuhiko Ikeda
Multicentric Castleman's disease (MCD) is lymphoproliferative disorder characterized by systemic inflammatory symptoms such as fever and weight loss. Human herpes virus-8 (HHV-8) is thought to be a causable pathogen in all HIV-positive and some HIV-negative MCD patients. Furthermore, the term idiopathic MCD (iMCD) was recently proposed to represent a group of HIV-negative and HHV-8-negative patients with unknown etiologies. Although the international diagnostic criteria for iMCD require exclusion of infection-related disorders, autoimmune/autoinflammatory diseases and malignant/lymphoproliferative disorders to make an iMCD diagnosis, the relationships and differences between these disorders and MCD have not yet been clarified...
2018: BioResearch Open Access
https://www.readbyqxmd.com/read/29680323/the-first-year-of-the-aevd-primary-cutaneous-lymphoma-registry
#14
Y Peñate, O Servitje, S Machan, R Fernández-de-Misa, M T Estrach, E Acebo, J Mitxelena, M D Ramón, A Flórez, M Blanes, M Morillo, S Medina, J Bassas, A Zayas, P Espinosa, A Pérez, N Gónzalez-Romero, J D Domínguez, C Muniesa, J López Robles, A Combalia, I Yanguas, H Suh, I Polo-Rodríguez, I Bielsa, A Mateu, B Ferrer, M A Descalzo, I García-Doval, P L Ortiz-Romero
BACKGROUND AND OBJECTIVE: Primary cutaneous lymphomas are uncommon. This article describes the Primary Cutaneous Lymphoma Registry of the Spanish Academy of Dermatology and Venereology (AEDV) and reports on the results from the first year. PATIENTS AND METHODS: Disease registry for patients with primary cutaneous lymphoma. The participating hospitals prospectively recorded data on diagnosis, treatment, tests, and disease stage for all patients with primary cutaneous lymphoma...
April 18, 2018: Actas Dermo-sifiliográficas
https://www.readbyqxmd.com/read/29670631/x-linked-lymphoproliferative-disease-type-1-a-clinical-and-molecular-perspective
#15
REVIEW
Neelam Panchal, Claire Booth, Jennifer L Cannons, Pamela L Schwartzberg
X-linked lymphoproliferative disease (XLP) was first described in the 1970s as a fatal lymphoproliferative syndrome associated with infection with Epstein-Barr virus (EBV). Features include hemophagocytic lymphohistiocytosis (HLH), lymphomas, and dysgammaglobulinemias. Molecular cloning of the causative gene, SH2D1A , has provided insight into the nature of disease, as well as helped characterize multiple features of normal immune cell function. Although XLP type 1 (XLP1) provides an example of a primary immunodeficiency in which patients have problems clearing primarily one infectious agent, it is clear that XLP1 is also a disease of severe immune dysregulation, even independent of EBV infection...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29658924/an-efficient-and-simple-method-to-establish-nk-and-t-cell-lines-from-patients-with-chronic-active-epstein-barr-virus-infection
#16
Chongfeng Xu, Junhong Ai, Qingxun Zhang, Ting Li, Xiaorong Wu, Zhengde Xie, Ziyuan Duan
A number of methods have been described to establish NK/T cell lines from patients with lymphoma or lymphoproliferative syndrome. These methods employed feeder cells, purified NK or T cells with as much as 10 mL of blood, or a high-dose of IL-2. This study presents a new method with a powerful and simple strategy to establish NK and T cell lines by culturing the peripheral blood mononuclear cells (PBMC) with the addition of recombinant human IL-2 (rhIL-2), and uses as little as 2 mL of whole blood. The cells can proliferate quickly in two weeks and be maintained for more than 3 months...
March 30, 2018: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/29652819/the-when-what-how-of-measuring-vitamin-d-metabolism-in-clinical-medicine
#17
REVIEW
Niek F Dirks, Mariëtte T Ackermans, Paul Lips, Renate T de Jongh, Marc G Vervloet, Robert de Jonge, Annemieke C Heijboer
We now have the ability to measure a number of different vitamin D metabolites with very accurate methods. The most abundant vitamin D metabolite, 25-hydroxyvitamin D, is currently the best marker for overall vitamin D status and is therefore most commonly measured in clinical medicine. The added value of measuring metabolites beyond 25-hydroxyvitamin D, like 1,25-, and 24,25-dihydroxyvitamin D is not broadly appreciated. Yet, in some more complicated cases, these metabolites may provide just the information needed for a legitimate diagnosis...
April 13, 2018: Nutrients
https://www.readbyqxmd.com/read/29649976/identification-of-a-novel-nonsense-mutation-in-sh2d1a-in-a-patient-with-x-linked-lymphoproliferative-syndrome-type-1-a-case-report
#18
Xiaodong Lyu, Zhen Guo, Yangwei Li, Ruihua Fan, Yongping Song
BACKGROUND: X-linked lymphoproliferative syndrome type 1 (XLP1) is an X-linked recessive genetic disorder with a strong resemblance to hemophagocytic lymphohistiocytosis (HLH). Causative mutations for XLP1 have been identified in SH2D1A, located on chromosome Xq25. CASE PRESENTATION: We report a case of an 18-month-old male with a novel nonsense mutation in SH2D1A. The patient presented the typical phenotype of HLH, including splenomegaly and hemophagocytosis in the bone marrow...
April 12, 2018: BMC Medical Genetics
https://www.readbyqxmd.com/read/29605220/lymphocytic-focus-score-is-positively-related-to-airway-and-interstitial-lung-diseases-in-primary-sj%C3%A3-gren-s-syndrome
#19
Tomoyuki Kakugawa, Noriho Sakamoto, Hiroshi Ishimoto, Toshimasa Shimizu, Hideki Nakamura, Aya Nawata, Chiyo Ito, Shuntaro Sato, Tetsuya Hanaka, Keishi Oda, Takashi Kido, Takuto Miyamura, Shota Nakashima, Takatoshi Aoki, Seiko Nakamichi, Yasushi Obase, Kazuyoshi Saito, Kazuhiro Yatera, Yuji Ishimatsu, Toshiyuki Nakayama, Yukunori Korogi, Atsushi Kawakami, Yoshiya Tanaka, Hiroshi Mukae
OBJECTIVE: Although high-resolution computed tomography (HRCT) is useful for the characterization of minute morphological changes in the lungs, no study has investigated risk factors for lung involvement detected by HRCT in patients with Sjögren's syndrome with or without respiratory symptoms. The aim of the current study was to investigate risk factors for lung involvement in patients with primary Sjögren's syndrome detected by HRCT, with a particular focus on airway and interstitial lung diseases...
April 2018: Respiratory Medicine
https://www.readbyqxmd.com/read/29599784/disease-evolution-and-response-to-rapamycin-in-activated-phosphoinositide-3-kinase-%C3%AE-syndrome-the-european-society-for-immunodeficiencies-activated-phosphoinositide-3-kinase-%C3%AE-syndrome-registry
#20
Maria Elena Maccari, Hassan Abolhassani, Asghar Aghamohammadi, Alessandro Aiuti, Olga Aleinikova, Catherine Bangs, Safa Baris, Federica Barzaghi, Helen Baxendale, Matthew Buckland, Siobhan O Burns, Caterina Cancrini, Andrew Cant, Pascal Cathébras, Marina Cavazzana, Anita Chandra, Francesca Conti, Tanya Coulter, Lisa A Devlin, J David M Edgar, Saul Faust, Alain Fischer, Marina Garcia Prat, Lennart Hammarström, Maximilian Heeg, Stephen Jolles, Elif Karakoc-Aydiner, Gerhard Kindle, Ayca Kiykim, Dinakantha Kumararatne, Bodo Grimbacher, Hilary Longhurst, Nizar Mahlaoui, Tomas Milota, Fernando Moreira, Despina Moshous, Anna Mukhina, Olaf Neth, Benedicte Neven, Alexandra Nieters, Peter Olbrich, Ahmet Ozen, Jana Pachlopnik Schmid, Capucine Picard, Seraina Prader, William Rae, Janine Reichenbach, Stephan Rusch, Sinisa Savic, Alessia Scarselli, Raphael Scheible, Anna Sediva, Svetlana O Sharapova, Anna Shcherbina, Mary Slatter, Pere Soler-Palacin, Aurelie Stanislas, Felipe Suarez, Francesca Tucci, Annette Uhlmann, Joris van Montfrans, Klaus Warnatz, Anthony Peter Williams, Phil Wood, Sven Kracker, Alison Mary Condliffe, Stephan Ehl
Activated phosphoinositide 3-kinase (PI3K) δ Syndrome (APDS), caused by autosomal dominant mutations in PIK3CD (APDS1) or PIK3R1 (APDS2), is a heterogeneous primary immunodeficiency. While initial cohort-descriptions summarized the spectrum of clinical and immunological manifestations, questions about long-term disease evolution and response to therapy remain. The prospective European Society for Immunodeficiencies (ESID)-APDS registry aims to characterize the disease course, identify outcome predictors, and evaluate treatment responses...
2018: Frontiers in Immunology
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