Read by QxMD icon Read

Lymphoproliferative diseases

S Bobillo, P Abrisqueta, B Sánchez-González, E Giné, S Romero, M Alcoceba, E González-Barca, S González de Villambrosía, J M Sancho, P Gómez, L Bento, J Montoro, S Montes, A López, F Bosch
Burkitt's monomorphic posttransplant lymphoproliferative disorder (B-PTLD) is an uncommon subtype of PTLD. Owing to the paucity of this complication, clinical characteristics and outcome has not been fully described. Clinical characteristics and outcomes of 20 patients diagnosed with B-PTLD from 10 transplant centers belonging to the GEL/TAMO group were reviewed. Median time from transplant to B-PTLD was 7.2 years. All the cases fulfill the morphologic and genetic criteria of B-PTLD, whereas Epstein-Barr virus (EBV) was detected in 70% of cases...
August 16, 2018: Annals of Hematology
Céline Habre, Paola M Soccal, Frédéric Triponez, John-David Aubert, Thorsten Krueger, Steve P Martin, Joanna Gariani, Jean-Claude Pache, Frédéric Lador, Xavier Montet, Anne-Lise Hachulla
Complications following lung transplantation may impede allograft function and threaten patient survival. The five main complications after lung transplantation are primary graft dysfunction, post-surgical complications, alloimmune responses, infections, and malignancy. Primary graft dysfunction, a transient ischemic/reperfusion injury, appears as a pulmonary edema in almost every patient during the first three days post-surgery. Post-surgical dysfunction could be depicted on computed tomography (CT), such as bronchial anastomosis dehiscence, bronchial stenosis and bronchomalacia, pulmonary artery stenosis, and size mismatch...
August 15, 2018: Insights Into Imaging
Samantha Correia, Ray Bridges, Fanny Wegner, Cristina Venturini, Anne Palser, Jaap M Middeldorp, Jeffrey I Cohen, Mario A Lorenzetti, Irene Bassano, Robert E White, Paul Kellam, Judith Breuer, Paul J Farrell
138 new Epstein-Barr virus (EBV) genome sequences have been determined. 125 of these and 116 from previous reports were combined to produce a multiple sequence alignment of 241 EBV genomes, which we have used to analyze variation within the viral genome. The type 1/type2 classification of EBV remains the major form of variation and is defined mostly by EBNA2 and EBNA3, but the type 2 SNPs at the EBNA3 locus extend into the adjacent gp350 and gp42 genes, whose products mediate infection of B cells by EBV. A small insertion within the BART miRNA region of the genome was present in 21 EBV strains...
August 15, 2018: Journal of Virology
Yue-Yang Li, Dong-Zhi Hu, Chen Tian
The 2016 world health organization (WHO) classification of B cell chronic lymphoproliferative disease (B-CLPD) includes chronic lymphocytic leukemia (CLL), B prolymphocytic leukemia, (B-PLL), hairy cell leukemia (HCL), marginal zone lymphoma (MZL), follicular lymphoma (FL), mantle cell lymphoma (MCL), Waldenstrom macroglobulinemia (LPL/WM). All the above-mentioned diseases are partially similar in cell morphology, immunophenotype and molecular genetics, but significantly different in treatment and prognosis...
August 2018: Zhongguo Shi Yan Xue Ye Xue za Zhi
Satoshi Kimura, Hiroshi Ohkawara, Kazuei Ogawa, Mizuko Tanaka, Takahiro Sano, Kayo Harada-Shirado, Hiroshi Takahashi, Koki Ueda, Akiko Shichishima-Nakamura, Hayato Matsumoto, Kazuhiko Ikeda, Junichiro James Kazama, Yuko Hashimoto, Takayuki Ikezoe
Light chain deposition disease (LCDD) is a rare systemic disorder caused by the deposition of light chain immunoglobulins, which often results in renal impairment associated with either nephrotic syndrome or asymptomatic proteinuria. B-cell neoplasms, such as multiple myeloma and lymphoproliferative disorders, are well-known underlying diseases in LCDD. Some chemotherapy regimens have been reported, but both evidence-based treatment and management for LCDD have yet to be established. We herein report three cases of LCDD treated with lenalidomide-based therapy, resulting in hematologic responses accompanied by a significant reduction in proteinuria and improvement in the renal function...
August 10, 2018: Internal Medicine
İsmail Harmankaya, Nevzat Serdar Ugras, Tamer Sekmenli, Fatih Demir, Yavuz Köksal
Simultaneous calcified fibrous pseudotumor (CFT) and Castleman disease (CD) is an extremely rare association. CD is an uncommon lymphoproliferative disease that can arise in various sites of the body, while CFT is a rare type of benign fibrous lesion that frequently affects children and young adults, occurring as solitary or multiple lesions throughout the human body. Both entities are rare and exhibit typical and diverse histomorphological features. We report the case of a 15-year-old female patient, who, at the age of 13 had a biopsy performed at an external medical center; however, after 4 months the lesion had regrown...
July 2018: Autopsy & Case Reports
Ziyi Li, Chuan Zhou, Ge Chen, Yuhai Bao
BACKGROUND: Rosai-Dorfman disease (RDD), also called sinus histiocytosis with massive lymphadenopathy (SHML), is an idiopathic, non-neoplastic, lymphoproliferative disorder. Histologically, the disease is characterized by emperipolesis and S-100 immunoreactivity. RDD usually presents with massive painless cervical lymphadenopathy, only 5% affecting the central nervous system (CNS). CASE DESCRIPTION: We here report on a 46-year-old female of isolated RDD of the left cavernous sinus with left facial numbness and weakness of left masticatory muscle...
August 9, 2018: World Neurosurgery
Maria Cosenza, Samantha Pozzi
Histone deacetylases (HDACs) are master regulators of chromatin remodeling, acting as epigenetic regulators of gene expression. In the last decade, inhibition of HDACs has become a target for specific epigenetic modifications related to cancer development. Overexpression of HDAC has been observed in several hematologic malignancies. Therefore, the observation that HDACs might play a role in various hematologic malignancies has brought to the development of HDAC inhibitors as potential antitumor agents. Recently, the class IIb, HDAC6, has emerged as one potential selective HDACi...
August 9, 2018: International Journal of Molecular Sciences
Bruno Roque Lima, Caroline Correa de Tulio Augusto Roque, Maria Dirlei Begnami, Patricia Peresi, Eduardo Nobrega Pereira Lima, Celso Abdon Lopes de Mello, Felipe José Fernandez Coimbra, Rubens Chojniak, Tiago Goss Santos
Liver metastasis is the major cause of death for patients with colorectal cancer. Despite treatment with surgery and chemotherapy, patient outcomes are quite unfavorable. Thus, there is an urgent need to develop new treatment strategies with the associated establishment of good animal models. Metastatic disease can be modeled using patient-derived orthotopic xenografts, which accurately replicate intra-tumoral heterogeneity, so that various chemotherapeutic agents can be tested on individual tumors to aid in clinical decision-making...
August 8, 2018: Journal of Drug Targeting
Carole Le Coz, Brian E Nolan, Melissa Trofa, Alicia M Kamsheh, Mustafa K Khokha, Saquib A Lakhani, Antonio Novelli, Elaine H Zackai, Kathleen E Sullivan, Silvana Briuglia, Tricia R Bhatti, Neil Romberg
Located contiguously on the long arm of the second chromosome are gene paralogs encoding the immunoglobulin-family co-activation receptors CD28 and cytotoxic T-lymphocyte-associated protein 4 (CTLA4). CD28 and CTLA4 share the same B7 ligands yet each provides opposing proliferative signals to T cells. Herein, we describe for the first time two unrelated subjects with coexisting CD28 and CTLA4 haploinsufficiency due to heterozygous microdeletions of chromosome 2q. Although their clinical phenotype, multi-organ inflammatory disease, is superficially similar to that of CTLA4 haploinsufficient autoimmune lymphoproliferative syndrome type V (ALPS5) patients, we demonstrate our subjects' underlying immunopathology to be distinct...
2018: Frontiers in Immunology
Yasodha Natkunam, Dita Gratzinger, Amy Chadburn, John R Goodlad, John K C Chan, Jonathan Said, Elaine S Jaffe, Daphne de Jong
Immunodeficiency-associated lymphoproliferative disorders (IA-LPDs) are pathologically and clinically heterogeneous. In many instances, similar features are shared by a spectrum of IA-LPDs in clinically diverse settings. The World Health Organization (WHO), however, classifies IA-LPDs by their immunodeficiency setting largely according to the paradigm of post-transplant lymphoproliferative disorders (PTLD), but with inconsistent terminology and disease definitions. The field currently lacks standardization and would greatly benefit from thinking across immunodeficiency categories by adopting a common working vocabulary to better understand these disorders and guide clinical management...
August 6, 2018: Blood
Natalia Pin Chuen Zing, Thais Fischer, Jasmine Zain, Massimo Federico, Steven T Rosen
Peripheral T-cell lymphomas (PTCLs) represent a heterogeneous group of diseases, with low incidence and unique epidemiology and pathobiology; they are usually clinically aggressive, with poor outcomes. There have been significant advances in our understanding of the molecular and signaling alterations seen in these malignancies. These observations have led to novel therapeutic strategies that have had a meaningful impact on outcomes. This two-part series highlights the most important aspects of PTCLs and describes current treatment options and investigative opportunities...
July 15, 2018: Oncology (Williston Park, NY)
Hidemi Toyoda, Takao Deguchi, Shotaro Iwamoto, Kentaro Kihira, Hiroki Hori, Yoshihiro Komada, Masahiro Hirayama
Recently, a new disease of lymphocyte homeostasis caused by somatic mosaicism for the RAS mutation has been discovered (known as RALD, RAS-associated leukoproliferative disorder). Since few cases have been reported in literature, the prognosis and standard treatment for autoimmune diseases associated with RALD remain poorly understood. Standard rituximab therapy (375 mg/m for 4 wk) is effective in patients with autoimmune diseases, but early recurrences are common. We highlight the potential for monthly administration of rituximab in a patient with autoimmune thrombocytopenia and hemolytic anemia associated with RALD...
August 4, 2018: Journal of Pediatric Hematology/oncology
Heather M Holahan, Ronda S Farah, Sara Fitz, Sarah L Mott, Nkanyezi N Ferguson, Julie McKillip, Brian Link, Vincent Liu
BACKGROUND: Little is currently known about health-related quality of life (HRQoL) of patients with cutaneous T-cell lymphoma (CTCL), a condition characterized by chronic, pruritic, visible lesions, features which may be uniquely influential. OBJECTIVE: The aim of this study was to establish baseline HRQoL data for patients with CTCL and identify its influencing factors. METHODS: Prospective, nonblinded survey design utilizing questionnaires including panels of QoL indices obtained from 105 patients with mycosis fungoides, Sezary syndrome, and CD30+ lymphoproliferative disorder...
August 3, 2018: International Journal of Dermatology
Matteo Sbattella, Andrea Zanichelli, Paolo Ghia, Valter Gattei, Chiara Suffritti, Thomas Teatini, Marco Cicardi, Roberto Castelli
Angioedema due to acquired deficiency of the inhibitor of the first component of complement (C1-INH) is a rare disease known as acquired angioedema (AAE). About 70% of patients with AEE display autoantibodies to C1-INH, the remaining patients have no antibodies to C1-INH. The clinical features of C1-INH deficiency include recurrent, self-limiting local swellings involving the skin, the gastrointestinal tract, and the upper respiratory tract. Swelling is due to accumulation of bradykinin released from high molecular weight kininogen...
August 2, 2018: Medical Oncology
Dario Roccatello, David Saadoun, Manuel Ramos-Casals, Athanasios G Tzioufas, Fernando C Fervenza, Patrice Cacoub, Anna Linda Zignego, Clodoveo Ferri
Cryoglobulinaemia refers to the serum presence of cryoglobulins, which are defined as immunoglobulins that precipitate at temperatures <37 °C. Type I cryoglobulinaemia consists of only one isotype or subclass of monoclonal immunoglobulin, whereas type II and type III are classified as mixed cryoglobulinaemia because they include immunoglobulin G (IgG) and IgM. Many lymphoproliferative, infectious and autoimmune disorders have been associated with mixed cryoglobulinaemia; however, hepatitis C virus (HCV) is the aetiologic agent in most patients...
August 2, 2018: Nature Reviews. Disease Primers
Margaret E Flanagan, Christopher Dirk Keene, David N Louis, Gordana Juric-Sekhar
Crystal-storing histiocytosis (CSH) is an uncommon histiocytic proliferation reported to involve diverse organs and tissues, but involvement of the central nervous system (CNS) is rare. In most cases CSH is identified in association with underlying lymphoproliferative, plasma cell diseases or rarely with various inflammatory or infectious conditions. CSH is characterized by the cytoplasmic accumulation of crystalline material in histiocytes, most commonly of kappa immunoglobulin light chain. We report a unique case of localized CSH involving the left cerebellum and caudal brain stem in a young man with a history of gout but without known lymphoproliferative or plasma cell disorders...
July 31, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
Brian Odom, Iyad Khourdaji, Victoria Golas, Richard Zekman, Bradley Rosenberg
Background: Acquired von Willebrand disease (AvWD) is a rare and often underdiagnosed disease that typically is associated with lymphoproliferative, cardiovascular disease, and myeloproliferative disease. It is challenging to diagnose as it requires a hemostatic challenge to present itself. Case Presentation: This is a 46-year-old male with a history of multiple sclerosis complicated by neurogenic bladder who presented with intermittent gross painless hematuria. He underwent a gross hematuria workup. Cystoscopy demonstrated active bleeding from the right ureteral orifice...
2018: Journal of Endourology Case Reports
O Mazanowska, P Donizy, J Kuźmińska, K Roczek, A Zimny, M Banasik, D Kaminska, K Hap, M Klinger, A Halon
INTRODUCTION: Acute central nervous system (CNS) damage in a patient who has received organ transplant is an extremely difficult and complex clinical issue that spans a wide spectrum of differential diagnoses with ischemia, post-transplant lymphoproliferative disorders (PTLDs), infections, lymphomas, and progressive multifocal leukoencephalopathy (PML). PTLDs are a clinically and histopathologically heterogeneous group of diseases that most often occur in heavily immunocompromised populations after solid organ transplantation (SOT), probably related to the infection or reactivation of Epstein-Barr virus (EBV) infection, whereas PML is an infectious disease caused by the John Cunningham virus (JCV)...
July 2018: Transplantation Proceedings
Lucia Martinez Cuesta, Maria Victoria Nieto Farias, Pamela Anahi Lendez, Lucas Barone, Sandra Elizabeth Pérez, Guillermina Laura Dolcini, Maria Carolina Ceriani
Bovine leukemia virus (BLV) is a retrovirus that affects cattle causing a lymphoproliferative disease. BLV infection has been associated with misbalance of the immune response causing a higher incidence of other infections. Mastitis is one of the most important conditions that affect milk production in cattle. The aim of this study was to stably infect a bovine mammary epithelial cell line (MAC-T). MAC-T cell line was successfully infected with BLV and the infection was confirmed by nested PCR, qPCR, immunocytochemistry, western blot and transmission electron microscopy...
July 25, 2018: Virus Research
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"