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Lymphoproliferative diseases

Edson Gonçalves Ferreira Junior, Philippos Apolinario Costa, Larissa Melo Freire Golveia Silveira, Rafael Valois Vieira, Hugo Alessi Lima Martins Soares, Bruna Menon Loureiro, Nayane Carolina Pertile Salvioni, Jose Roberto Coelho Ferreira Rocha
INTRODUCTION: Castleman disease (CD) is a rare polyclonal lymphoproliferative disorder of unknown etiology, which usually develops in the mediastinum. It can also occur in the cervical, retroperitoneal and axillary regions. Localized pancreatic CD is quite rare [1]. PRESENTATION OF CASE: The authors herein present a case of a 34 years old female that was diagnosed during a symptomatic cholelithiasis evaluation. During the evaluation, an abdominal ultrasonography revealed a tumor at the head of the pancreas, which went on to generate a dilatation of the extrahepatic bile ducts...
November 20, 2018: International Journal of Surgery Case Reports
Nelson Leung, Frank Bridoux, Vecihi Batuman, Aristeidis Chaidos, Paul Cockwell, Vivette D D'Agati, Angela Dispenzieri, Fernando C Fervenza, Jean-Paul Fermand, Simon Gibbs, Julian D Gillmore, Guillermo A Herrera, Arnaud Jaccard, Dragan Jevremovic, Efstathios Kastritis, Vishal Kukreti, Robert A Kyle, Helen J Lachmann, Christopher P Larsen, Heinz Ludwig, Glen S Markowitz, Giampaolo Merlini, Peter Mollee, Maria M Picken, Vincent S Rajkumar, Virginie Royal, Paul W Sanders, Sanjeev Sethi, Christopher P Venner, Peter M Voorhees, Ashutosh D Wechalekar, Brendan M Weiss, Samih H Nasr
The term monoclonal gammopathy of renal significance (MGRS) was introduced by the International Kidney and Monoclonal Gammopathy Research Group (IKMG) in 2012. The IKMG met in April 2017 to refine the definition of MGRS and to update the diagnostic criteria for MGRS-related diseases. Accordingly, in this Expert Consensus Document, the IKMG redefines MGRS as a clonal proliferative disorder that produces a nephrotoxic monoclonal immunoglobulin and does not meet previously defined haematological criteria for treatment of a specific malignancy...
December 3, 2018: Nature Reviews. Nephrology
Akihiro Yachie
Rapid, precise diagnosis of Epstein-Barr virus-associated T lymphocyte or natural killer cell lymphoproliferative diseases is clinically important to prevent disease progression and avoid fatal outcomes for patients. In addition to detecting increased copy numbers of Epstein-Barr virus, identification of the lymphocyte subpopulation targeted by the virus infection is crucial to reaching the final diagnosis. However, these procedures are laborious and require large amounts of sample. In contrast, flowcytometric analysis may provide crucial information for initial screening of diseases using only small amounts of sample and involves little labor...
2018: Frontiers in Pediatrics
Hao Gu, Run-Hui Wu
Immune-mediated hemocytopenia is a common cytopenic diseases without bone marrow hematopoietic abnormalities, the patient's quality of life is significantly reduced when first-line treatments are ineffective. Rapamycin, possesses a clear mechanism of targeting mTOR protein, can upregulate regulatory T cells and induces apoptosis of specific cells, by regulating the lymphocyte subsets, so as to treat various types of immune-mediated hemocytopenia with a certain therapeutic effect. In this reviews, the action mechanism and clinical application of rapamycin in immune thrombocytopenia(ITP), autoimmune hemolytic anemia(AIHA), acquired aplastic anemia and autoimmune lymphoproliferative syndrome(ALPS) etc...
December 2018: Zhongguo Shi Yan Xue Ye Xue za Zhi
Cynthia Chen, Yuhan D Gu, Larisa J Geskin
Primary cutaneous CD30+ lymphoproliferative diseases (LPDs) comprise a range of diseases (LyP, pcALCL, and borderline lesions) with broad histologic and phenotypical characteristics, although they all share the common feature of a favorable prognosis notwithstanding histology suggestive of a high-grade lymphoma. Given their cytomorphologic similarities, accurate diagnosis and workup are needed to differentiate these distinct entities in order to best use novel biologic therapies and avoid aggressive overtreatment...
February 2019: Hematology/oncology Clinics of North America
Andrew Iskandar, Andrew Hwang, Constantin A Dasanu
Castleman disease is a rare B-cell lymphoproliferative disorder characterized by lymph node enlargement with or without constitutional signs. Herein, we describe a unique patient with multicentric Castleman disease and retroviral infection who presented with a sudden onset of constitutional signs and was found to have severe warm-antibody autoimmune hemolytic anemia. Rituximab monotherapy yielded an excellent clinical response. We aim to inform the medical community of this rare paraneoplastic phenomenon in patients with Castleman disease and its effective management...
November 28, 2018: Journal of Oncology Pharmacy Practice
Aki Hoji, Susie Xu, Holly Bilben, David T Rowe
The latent state is a critical component of all herpesvirus infections, and its regulation remains one of the most active areas of Epstein-Barr Virus (EBV) research. In particular, identifying environmental factors that trigger EBV reactivation into a virus-productive state has become a central goal in EBV latency research. Recently, a category of chemicals known as inducers of the endoplasmic reticulum unfolded protein response (UPR) have been shown to trigger EBV lytic reactivation in various established EBV-associated lymphoma cell lines...
November 2018: Heliyon
Javier Pagan, Marco Ladino, David Roth
The identification of hepatitis C virus (HCV) occurred in 1989, and soon thereafter, it was recognized that there was a higher prevalence of anti-HCV seropositivity in patients with end-stage renal disease (ESRD) when compared to the general population. Multiple extrahepatic manifestations have been associated with HCV infection in patients with ESRD; these include an increased prevalence and risk of cardiovascular complications, insulin resistance, diabetes mellitus, and lymphoproliferative disorders. Infection with HCV has also been associated with an increased relative risk of mortality in the ESRD patient when contrasted to those patients without infection...
November 26, 2018: Seminars in Dialysis
Hany I Sakr, Kaila Buckley, Robert Baiocchi, Weiqiang John Zhao, Jessica A Hemminger
BACKGROUND: Erdheim Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis characterized by widespread tissue infiltration by CD68-positive, CD1a-negative foamy histiocytes. ECD can be difficult to identify, and diagnosis relies on the presence of histiocytes with certain histologic and immunophenotypic features in an appropriate clinical and radiologic setting. Clinical signs and symptoms are variable depending on which organ systems are involved. Most patients have at least skeletal involvement with bone pain as well as fatigue...
November 24, 2018: Diagnostic Pathology
Ana P Rossi, Christina L Klein
Posttransplant malignancy is a leading cause of death after solid organ transplantation (SOT). Recipients of SOT are at significantly higher risk of multiple cancers compared with the general population, most notably nonmelanoma skin cancer and posttransplant lymphoproliferative disorders. Risk factors for posttransplant malignancy include history of malignancy, immunosuppression, oncogenic viral infections, sun exposure, and disease-specific associations. Early detection and treatment of malignancies can improve survival...
February 2019: Surgical Clinics of North America
Akihisa Sawada, Masami Inoue
Chronic active Epstein-Barr virus infection (CAEBV) is a prototype of EBV-associated T- and/or NK-cell (EBV+ T/NK-cell) lymphoproliferative disorders. Most subtypes of these are lethal. We established a unified treatment strategy composed of step 1 (immunochemotherapy: steroids, cyclosporine A, and etoposide), step 2 (multi-drug block chemotherapy), and step 3 (allogeneic hematopoietic stem cell transplantation; HSCT) for CAEBV and its related diseases. Allogeneic HSCT is the only cure for CAEBV with few exceptions...
2018: Frontiers in Pediatrics
Yoshitaka Sato
No abstract text is available yet for this article.
2018: Frontiers in Pediatrics
Akihiro Tamura, Suguru Uemura, Nobuyuki Yamamoto, Atsuro Saito, Aiko Kozaki, Kenji Kishimoto, Toshiaki Ishida, Daiichiro Hasegawa, Haruka Hiroki, Tsubasa Okano, Kohsuke Imai, Tomohiro Morio, Hirokazu Kanegane, Yoshiyuki Kosaka
Background: X-linked lymphoproliferative disease type 1 (XLP1) is a rare primary immune deficiency, which is caused by SH2D1A gene mutations. XLP1 is commonly associated with Epstein-Barr virus (EBV)-associated hemophagocytic lymphohistiocytosis, hypogammaglobulinemia, and/or lymphoma. The only curative treatment for XLP1 is allogeneic hematopoietic cell transplantation. However, published data detailing the clinical course of, and indications for, allogeneic hematopoietic cell transplantation in asymptomatic patients with XLP1 is lacking...
2018: Allergy, Asthma, and Clinical Immunology
Stella Bernardi, Andrea Michelli, Deborah Bonazza, Veronica Calabrò, Fabrizio Zanconati, Gabriele Pozzato, Bruno Fabris
BACKGROUND: Thyroid lymphomas are an exceptional finding in patients with thyroid nodules. Burkitt's lymphoma is one of the rarest and most aggressive forms of thyroid lymphomas, and its prognosis depends on the earliness of medical treatment. Given the rarity of this disease, making a prompt diagnosis can be challenging. For instance, fine-needle aspiration (FNA) cytology, which is the first-line diagnostic test that is performed in patients with thyroid nodules, is often not diagnostic in cases of thyroid lymphomas, with subsequent delay of the start of therapy...
November 19, 2018: BMC Endocrine Disorders
Ryan G Short, Tina D Tailor
History A 31-year-old woman with a history of bilateral orthotopic lung transplantation performed 10 months earlier for cystic fibrosis presented for a routine follow-up appointment, with her chief symptom being a cough. The cough started approximately 1 month prior to this appointment and was minimally productive of clear to yellow phlegm. In addition to her cough, she reported increased sinus congestion and a sensation of "something in her upper chest." She denied shortness of breath, wheezing, hemoptysis, or cigarette smoking...
December 2018: Radiology
Morgane Cheminant, Julie Bruneau, Georgia Malamut, David Sibon, Nicolas Guegan, Tom van Gils, Sascha Cording, Amélie Trinquand, Virginie Verkarre, Ludovic Lhermitte, Nicole Brousse, Anne-Sophie Jannot, Sherine Khater, Laurent Frenzel, Richard Delarue, Felipe Suarez, Ambroise Marçais, Chris Jj Mulder, Elizabeth Macintyre, Vahid Asnafi, Laurent Pouyet, Cécile Bonnafous, Florence Lhospice, Thierry Jo Molina, Bertrand Meresse, Christophe Cellier, Nadine Cerf-Bensussan, Olivier Hermine
OBJECTIVES: Primary GI T-cell lymphoproliferative diseases (T-LPD) are heterogeneous entities, which raise difficult diagnosis and therapeutic challenges. We have recently provided evidences that lymphomas complicating coeliac disease (CD) arise from innate-like lymphocytes, which may carry NK receptors (NKRs). DESIGN: NKRs expression was compared by flow cytometry in intraepithelial lymphocytes (IEL) from CD, type I or type II refractory CD (RCD). NKp46 was next assessed by immunohistochemistry in paraffin-embedded biopsies from 204 patients with CD, RCDI, RCDII or GI T-cell lymphomas and from a validation cohort of 61 patients...
November 17, 2018: Gut
(no author information available yet)
The interaction between pituitary hormones, GH - PRL, and Growth Factors, GF, plays a fundamental role in the physiological and neoplastic mechanisms of growth, the latter using these factors to a much greater extent compared to the former, with a direct dose-dependent effect on the speed of local or metastatic expansion. In hormone-dependent tumours, the respective male and female sex hormones interact with GH - PRL - GF to sustain the expansion of the tumour. We carried out a review of the literature on the relationship between the expression of GH and GHR in tumour tissues compared to healthy tissues, and on the correlation between this expression and tumour aggressiveness...
September 15, 2018: Neuro Endocrinology Letters
Sandra Kojić Katović, Ankica Vasilj, Goran Rinčić
T-cell large granular lymphocytic leukemia (T-LGLL) is an uncommon but probably underdiagnosed disease caused by clonal proliferation of large granular lymphocytes. Diagnosis is typically based on the high number of morphologically characteristic lymphoid cells and finding of an abnormal immunophenotype by flow cytometry. Because of its relatively indolent clinical behavior, observation is often an appropriate therapy. Here we present a case of a 53-year-old male admitted to the hospital because of abdominal pain...
June 2018: Acta Clinica Croatica
Macartney Welborn, Madeleine Duvic
Cutaneous T-cell lymphomas (CTCLs) are a group of non-Hodgkin's lymphomas that present in the skin. In early-stage disease, the course is generally chronic and indolent; however, in advanced stages of disease, therapies rarely provide long-lasting responses, and the only potential curative therapy is allogeneic hematopoietic stem-cell transplantation. This has led to the search for novel targeted therapies to better treat more advanced stages of CTCLs that cannot be controlled by typical treatment regimens...
November 15, 2018: American Journal of Clinical Dermatology
Salah Ali, Sami AlThubaiti, Samuele Renzi, Joerg Krueger, K Y Chiang, Ahmed Naqvi, Tal Schechter, Angela Punnett, Muhammad Ali
EBV-related PTLD developing after HSCT is a potentially life-threatening disease. HLH is uncommon after allogeneic HSCT. Data on outcome of patients with PTLD and concomitant HLH after allogeneic HSCT are limited. In this retrospective study, we collected demographic, clinical, laboratory, and outcome data for 408 patients who underwent allogeneic HSCT from 2006 to 2015. Graft source included CB (n = 135; 33.1%), PBSCs (n = 34; 8.3%), and BM (n = 239; 58.6%). Eight out of 408 patients (2%) developed EBV-PTLD with a median age at HSCT of 5...
November 11, 2018: Pediatric Transplantation
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