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Lymphoproliferative syndromes

Mercedes G Vázquez, Pablo N Affranchino, Andrea Schon, Eduardo Otero, Pablo Young, Patricia Breyter
Goldbloom syndrome is a rare clinical entity, of unknown etiology that happens almost exclusively in pediatric population. It is a prolonged febrile syndrome with periosteal hyperostosis and dysproteinemia, and often simulates an hematooncology or lymphoproliferative disease. The diagnosis is to rule out the different causes of bone pain associated with hypergammaglobulinemia, hypoalbuminemia, high erythrocyte sedimentation rate and periostitis at the radiographies. Symptomatology, radiology and laboratory parameters refer in a variable time, usually from 3 to 12 months...
April 1, 2018: Archivos Argentinos de Pediatría
Alison M Condliffe, Anita Chandra
The activated phosphoinositide 3-kinase δ syndrome (APDS), also known as p110δ-activating mutation causing senescent T cells, lymphadenopathy, and immunodeficiency (PASLI), is a combined immunodeficiency syndrome caused by gain-of-function mutations in the phosphoinositide 3-kinase (PI3K) genes PIK3CD (encoding p110δ: APDS1 or PASLI-CD) and PIK3R1 (encoding p85α: APDS2 or PASLI-R1). While the disease is clinically heterogeneous, respiratory symptoms and complications are near universal and often severe...
2018: Frontiers in Immunology
Kimi Okada, Shoji Asakura, Tomofumi Yano, Takumi Kishimoto
Primary effusion lymphoma (PEL) is a rare type of extranodal lymphoma, typically of a B-cell origin, which presents as lymphomatous effusion with no nodal enlargement or tumor masses. The development PEL is universally associated with human herpes virus-8 (HHV-8) infection. Cases of HHV-8-negative primary lymphomatous effusion have recently been reported and referred to as HHV-8-unrelated PEL-like lymphoma. Some cases of this disease have been reported in iatrogenic immunocompromised patients. The mechanisms responsible for the inhibitory effects of the discontinuation of immunosuppressants other than methotrexate (MTX) against the disease, which have been demonstrated for MTX-associated lymphoproliferative disorders, have not yet been elucidated...
March 7, 2018: International Journal of Hematology
Fernando Gallardo, Joan Bertran, Erika López-Arribillaga, Jéssica González, Silvia Menéndez, Ignacio Sánchez, Luis Colomo, Mar Iglesias, Marta Garrido, Luis Francisco Santamaría-Babí, Ferran Torres, Ramon M Pujol, Anna Bigas, Lluís Espinosa
Cutaneous T-cell lymphomas (CTCLs) represent different subtypes of lymphoproliferative disorders with no curative therapies for the advanced forms of the disease (namely mycosis fungoides and the leukemic variant, Sézary syndrome). Molecular events leading to CTCL progression are heterogeneous, however recent DNA and RNA sequencing studies highlighted the importance of NF-κB and β-catenin pathways. We here show that the kinase TAK1, known as essential in B-cell lymphoma, is constitutively activated in CTCL cells, but tempered by the MYPT1/PP1 phosphatase complex...
February 22, 2018: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
Agostino Nocerino, Erica Valencic, Claudia Loganes, Giorgio Pelos, Alberto Tommasini
No abstract text is available yet for this article.
February 26, 2018: Pediatrics International: Official Journal of the Japan Pediatric Society
Weerapat Owattanapanich, Wikanda Pholmoo, Tawatchai Pongpruttipan, Noppadol Siritanaratkul
Castleman's disease (CD) is a rare lymphoproliferative disorder, and its prevalence in Thailand is not known. This 10-year period study investigated the prevalence of CD in Thailand, and the clinical characteristics and outcomes of Thai CD patients, with special focus on the existence and prevalence of TAFRO syndrome. TAFRO syndrome is defined as CD with thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly. Thirty-three CD patients diagnosed and treated at Siriraj Hospital during January 2007 to December 2016 were included...
February 20, 2018: Annals of Hematology
Kentaro Sakashita, Kengo Murata, Mikio Takamori
Multicentric Castleman's disease (MCD), a distinct subtype of Castleman's disease, is a rare, nonneoplastic, lymphoproliferative disorder. Patients with MCD present with systemic symptoms and multiple lymphadenopathy. Lymph node biopsy is necessary for the diagnosis of various histological MCD patterns including hyaline vascular, plasma cell, and mixed types. Human herpesvirus 8 (HHV8) infection was identified as an important etiology of MCD among immunocompromised patients such as those positive for human immunodeficiency virus...
2018: Journal of Blood Medicine
Wan-Chen Hsieh, Tzu-Sheng Hsu, Ya-Jen Chang, Ming-Zong Lai
X-linked lymphoproliferative syndrome type-2 (XLP-2) is a primary immunodeficiency disease attributed to XIAP mutation and is triggered by infection. Here, we show that mouse Xiap-/- regulatory T (Treg) cells and human XIAP-deficient Treg cells are defective in suppressive function. The Xiap-/- Treg cell defect is linked partly to decreased SOCS1 expression. XIAP binds SOCS1 and promotes SOCS1 stabilization. Foxp3 stability is reduced in Xiap-/- Treg cells. In addition, Xiap-/- Treg cells are prone to IFN-γ secretion...
January 31, 2018: Nature Communications
A Flores-Chávez, J A Carrion, X Forns, M Ramos-Casals
Chronic hepatitis C virus (HCV) infection has been associated with both organ-specific and systemic autoimmune diseases, with cryoglobulinemia being the most frequent associated disease. Experimental, virologic, and clinical evidence have demon-strated a close association between HCV infection and some systemic autoimmune diseases, especially Sjögren's syndrome, but also rheumatoid arthritis and lupus. A higher prevalence of hematological processes has also been described in patients with HCV infection, including cytopenias and lymphoproliferative disorders (B-cell lymphoma)...
December 2017: Revista Española de Sanidad Penitenciaria
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
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
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 10, 2018: Clinical Immunology: the Official Journal of the Clinical Immunology Society
Anna Hayden, Molly Lin, Sujin Park, Morris Pudek, Marion Schneider, Michael B Jordan, Andre Mattman, Luke Y C Chen
Serum soluble interleukin-2 receptor (sIL-2r) is an important disease marker in hemophagocytic lymphohistiocytosis (HLH), but there are no published data on its diagnostic value in adults. We conducted a single-center retrospective study of 78 consecutive adults who had sIL-2r measured for suspected HLH. Serum sIL-2r levels were measured by enzyme-linked immunosorbent assay (adult reference range, 241-846 U/mL). There were 38 patients with HLH and 40 with a non-HLH diagnosis (such as sepsis, liver disease, histiocyte disorders, autoimmune disease, leukemia, or lymphoma)...
December 12, 2017: Blood Advances
Stefano Vavassori, Jacob D Galson, Johannes Trück, Anke van den Berg, Rienk Y J Tamminga, Aude Magerus-Chatinet, Olivier Pellé, Ulrike Camenisch Gross, Ewerton Marques Maggio, Seraina Prader, Lennart Opitz, Ursina Nüesch, Andrea Mauracher, Benjamin Volkmer, Oliver Speer, Luzia Suda, Benno Röthlisberger, Dieter Robert Zimmermann, Rouven Müller, Arjan Diepstra, Lydia Visser, Eugenia Haralambieva, Bénédicte Neven, Frédéric Rieux-Laucat, Jana Pachlopnik Schmid
FAS-dependent apoptosis in Vδ1 T cells makes the latter possible culprits for the lymphadenopathy observed in patients with FAS mutations.Rapamycin and methylprednisolone resistance should prompt clinicians to look for Vδ1 T cell proliferation in ALPS-FAS patients.
June 27, 2017: Blood Advances
Xiaoxie Xu, Jun Huang, Mei Zhao, Huanpeng Chen, Jinhua Mo, Xiaoqing Zhou, Qiao Su, Bolan Yu, Zhaofeng Huang
Autoimmune lymphoproliferative syndrome (ALPS) is an incurable disease mainly caused by the defect of Fas-mediated apoptosis and characterized by nonmalignant autoimmune lymphoproliferation. Stabilized β-catenin could not only potentiate Fas-mediated T cell apoptosis via upregulating the expression of Fas on activated T cells, but also potentiate T cell apoptosis via intrinsic apoptotic pathway. In the present study, we introduced β-catTg into lpr/lpr mice and aimed to explore the potential role of stabilized β-catenin (β-catTg) in the development of ALPS-like phenotypes of lpr/lpr mice...
2017: Journal of Immunology Research
Patricia María O'Farrill-Romanillos, Diana Andrea Herrera-Sánchez, Cecilia Hernández-Fernández, Eunice Giselle López-Rocha
Primary immunodeficiencies (PIDs) are low-incidence diseases caused by defects in genes involved in the development, maintenance, and regulation of the immune system. Common variable immunodeficiency (CVID) is the most common symptomatic immunodeficiency of adulthood. It has an approximate prevalence of 1 in 25 000-50 000 in the general population, with a delay in diagnosis between 6-7 years. The clinical manifestations of CVID constitute six main categories: infections, pulmonary complications, granulomatous or polyclonal lymphocytic disease, autoimmunity, gastrointestinal diseases and malignancy Most patients must have at least one of the following clinical manifestations (infection, autoimmunity, lymphoproliferation)...
October 2017: Revista Alergia Mexico: Organo Oficial de la Sociedad Mexicana de Alergia e Inmunología, A.C
Keisuke Kawamoto, Hiroaki Miyoshi, Takaharu Suzuki, Yasuji Kozai, Koji Kato, Masaharu Miyahara, Toshiaki Yujiri, Naoki Oishi, Ilseung Choi, Katsumichi Fujimaki, Tsuyoshi Muta, Masaaki Kume, Sayaka Moriguchi, Shinobu Tamura, Takeharu Kato, Hiroyuki Tagawa, Junya Makiyama, Yuji Kanisawa, Yuya Sasaki, Daisuke Kurita, Kyohei Yamada, Joji Shimono, Hirohito Sone, Jun Takizawa, Masao Seto, Hiroshi Kimura, Koichi Ohshima
The characteristics of adult patients with chronic active Epstein-Barr virus infection (adult-onset CAEBV) are poorly recognized, hindering early diagnosis and an improved prognosis. Adult-onset CAEBV (n = 54) diagnosed between 2005 and 2015 were conducted. Adult-onset was defined as an estimated age of onset ≥15 years. To characterize the clinical features of adult-onset CAEBV, we compared them to those of pediatric-onset (estimated age of onset <15 years) patients (n = 75). We compared the prognosis of adult-onset CAEBV with that for patients with nasal-type (n = 37) and non-nasal-type (n = 45) extranodal NK/T-cell lymphoma (ENKTL)...
December 14, 2017: Haematologica
Stephen Opat, Anna C Hearps, Kevin Thia, Agnes Yuen, Ben Rogers, Mkunde Chachage, Gregory Moore, Jake Shortt, Georgina Ryland, Piers Blombery, Anthony P Schwarer, Tahereh Noori, Joseph A Trapani, Anthony Jaworowski, Ilia Voskoboinik
No abstract text is available yet for this article.
February 8, 2018: Blood
Alejandro A Gru, Dennis P O'Malley
This article will provide a discussion of some common autoimmune disorders that could affect the lymph nodes and potentially mimic B and T-cell lymphomas. Some of these disorders are more characteristic of individuals in the pediatric age group (autoimmune lymphoproliferative syndrome, Kawasaki disease), while others present in older individuals (rheumatoid arthritis, lupus erythematosus, sarcoidosis). A common finding that groups all of these disorders together is the overall relative preservation of the architecture, a feature that can be particularly helpful to distinguish them from many B and T-cell lymphomas...
January 2018: Seminars in Diagnostic Pathology
Wei-Fu Lai, Yen-Po Chin, Chih-Wei Liu, Chang-Youh Tsai
A 62-year-old woman with rheumatoid arthritis and secondary Sjögren's syndrome took methotrexate (MTX) 5 mg three times a week regularly but gradually developed an intermittent fever, oral ulcers and productive cough with mucopurulent sputum for about 2 weeks. Image study found multiple nodular lesions and lymphadenopathies in bilateral lungs. Empirical antibiotics for 1 week failed to alleviate the fever. A transbronchial biopsy in the right fourth bronchus showed infiltration of abnormally enlarged lymphoid cells with a surface marker of CD20, some of which also stained positively in situ with Epstein-Barr virus-encoded small RNA and some CD3(+) cells...
December 1, 2017: BMJ Case Reports
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