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Lymphoproliferative syndromes

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https://www.readbyqxmd.com/read/28937520/evans-syndrome-as-first-manifestation-of-primary-immunodeficiency-in-clinical-practice
#1
Libny Martínez-Valdez, Angela Deyà-Martínez, María T Giner, Rubén Berrueco, Ana Esteve-Solé, Manel Juan, Ana M Plaza-Martín, Laia Alsina
BACKGROUND: Evans syndrome (ES) is a rare immune disorder in children, manifested by simultaneous or sequential autoimmune cytopenias (ACs) of unknown cause and having a chronic course with periods of exacerbation and remission. Some primary immunodeficiencies (PIDs) may present with autoimmune manifestations without infections, masking suspicion of them. The PIDs that can typically manifest as ES are autoimmune lymphoproliferative syndrome and common variable immunodeficiency (CVID)...
October 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28935848/clinical-relevance-of-silent-red-blood-cell-autoantibodies
#2
Francesca R Mauro, Fabio Trastulli, Cristiano Alessandri, Guido Valesini, Gianluca Giovannetti, Costantino Riemma, Marika Porrazzo, Sara Pepe, Gioia Colafigli, Maria D Caputo, Maria S De Propris, Anna R Guarini, Gabriella Girelli, Serelina Coluzzi, Robin Foà
To evaluate the clinical significance of RBC autoantibodies in the absence of clinical signs of autoimmune hemolytic anemia (AHIA), we retrospectively analyzed the clinical course of 60 subjects with silent RBC autoantibodies (IgG, 24; IgM, 35; IgG+IgM, 1) diagnosed and followed at our institute. Asymptomatic RBC autoantibodies were detected in 5 (8.3%) pregnant females, 34 (56.7%) healthy individuals (blood donors, 32; subjects with abnormal agglutination of blood samples, 2) and 21 (35%) patients screened prior to surgery (benign disorders, 16; tumors, 5)...
September 21, 2017: Haematologica
https://www.readbyqxmd.com/read/28906499/-incomplete-poems-syndrome-with-multicentric-castleman-s-disease
#3
P González de la Aleja, M García-Navarro, R Sánchez-Rodríguez, J M Ramos-Rincón
Castleman's disease (CD) is an atypical lymphoproliferative disorder of unknown cause, characterized by non-clonal nodal hyperplastic growth. Two forms of clinical presentation are currently recognized, one localized and the other multicentric, and four histopathologic variants. It is characterized by generalized lymphadenopathy, hepatosplenomegaly, fever and night sweats. CD may present severe pancytopenia, multi-organ failure, lymphoma evolution and it can sometimes be associated with paraneoplastic syndromes such as POEMS syndrome...
September 14, 2017: Anales del Sistema Sanitario de Navarra
https://www.readbyqxmd.com/read/28898770/immunoglobulin-replacement-therapy-in-primary-and-secondary-antibody-deficiency-the-correct-clinical-approach
#4
REVIEW
Antonio Pecoraro, Ludovica Crescenzi, Francescopaolo Granata, Arturo Genovese, Giuseppe Spadaro
Immunoglobulin therapy is the administration of human polyvalent IgG and represents the most effective treatment to prevent recurrent infections in antibody deficiency patients. Primary antibody deficiency represents the main indication of immunoglobulin replacement therapy and includes a wide range of disorders characterized by impaired antibody production in response to pathogens and recurrent infections. However, not all primary antibody deficiency patients require immunoglobulin replacement. Indeed, immunoglobulin preparations are expensive and, once prescribed, usually result in lifelong therapy...
September 9, 2017: International Immunopharmacology
https://www.readbyqxmd.com/read/28872191/cutaneous-t-cell-lymphoma-2017-update-on-diagnosis-risk-stratification-and-management
#5
Ryan A Wilcox
DISEASE OVERVIEW: Cutaneous T-cell lymphomas are a heterogenous group of T-cell lymphoproliferative disorders involving the skin, the majority of which may be classified as Mycosis Fungoides (MF) or Sézary Syndrome (SS). DIAGNOSIS: The diagnosis of MF or SS requires the integration of clinical and histopathologic data. RISK-ADAPTED THERAPY: TNMB (tumor, node, metastasis, blood) staging remains the most important prognostic factor in MF/SS and forms the basis for a "risk-adapted," multi-disciplinary approach to treatment...
October 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28871936/xanthoma-like-skin-changes-in-an-elderly-woman-with-a-normal-lipid-profile
#6
Piotr Nockowski, Zdzisław Woźniak, Adam Reich, Joanna Maj
Dear Editor, An 83-year-old woman developed yellow-brownish infiltrates, nodules, and tumors mimicking xanthomas, mostly involving the periorbital and chest area within three months (Figure 1). She had no abnormalities in serum cholesterol or triglycerides levels. A detailed laboratory analysis revealed the presence of mild monoclonal gammopathy with a presence of immunoglobulin G (IgG) kappa light chains; however, according to hematologist consultation, it did not require medical intervention. Imaging assessment and ultrasound examination did not show any specific involvement of internal organs...
July 2017: Acta Dermatovenerologica Croatica: ADC
https://www.readbyqxmd.com/read/28871434/cutaneous-and-mucosal-manifestations-of-sj%C3%A3-gren-s-syndrome
#7
REVIEW
Elena Generali, Antonio Costanzo, Carlo Mainetti, Carlo Selmi
Sjögren's syndrome is currently considered an "autoimmune epithelitis," as exocrine glands, especially salivary and lacrimal, are progressively destructed by an immune-mediated process associated with specific serum autoantibodies and local lymphocyte infiltrate. Xerostomia remains a key complain in patients with Sjögren's syndrome but should be evaluated also for other causes such as xerogenic medications, followed by radiation and chemotherapy for head and neck cancers, hormone disorders, infections, or other connective tissue diseases...
September 4, 2017: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/28868635/usefulness-of-the-lymphocyte-positional-parameters-in-the-sysmex-xn-haematology-analyser-in-lymphoproliferative-disorders-and-mononucleosis-syndrome
#8
J R Furundarena, A Uranga, M R Sainz, C González, N Uresandi, N Argoitia, M Araiz
INTRODUCTION: The lymphocyte positional parameters included in Sysmex XN have been suggested as useful means to differentiate lymphoproliferative disorders (LPD), mononucleosis syndrome (MNS) and other lymphocytoses. METHODS: We evaluated Sysmex XN analysers, which supply 6 lymphocyte positional parameters that can be measured in the WDF scattergram: LY-X, LY-Y, LY-Z, LY-WX, LY-WY and LY-WZ. RESULTS: We collected 301 samples from normal controls, polyclonal lymphocytosis, MNS and LPD...
September 4, 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28860939/infectious-mononucleosis-like-syndrome-with-high-lymphocytosis-and-positive-igm-ebv-and-cmv-antibodies-in-a-three-year-old-girl
#9
Joanna Alicja Krupka, Katarzyna Samelska, Anna Tomasik, Anna Stelmaszczyk-Emmel, Katarzyna Pawelec
Primary Epstein-Barr virus (EBV) and cytomegalovirus (CMV) infection usually affects preadolescent children or young adults, causing similar clinical presentation. Signs and symptoms are typically mild, and the majority of clinical and laboratory findings resolve spontaneously within one month after onset. In adulthood, the risk of fulminant EBV infection and severe complications is much higher, which may be related to increasing memory CD8+ T-cell population with age. It is still not clear what exactly triggers T-cell clonal proliferation...
2017: Central-European Journal of Immunology
https://www.readbyqxmd.com/read/28829215/monoclonal-antibodies-against-cutaneous-t-cell-lymphomas
#10
Mauro Alaibac
Cutaneous T-cell lymphomas (CTCLs) comprise of a group of rare and heterogeneous skin lymphoproliferative disorders derived from skin resident T cells. Treatment of CTCLs is based on skin-directed approaches and/or systemic therapies. Advanced CTCLs are difficult to treat with the currently available treatments as they generally fail to obtain prolonged clinical remission. Recent studies concerning the pathogenetic mechanisms that are operative in CTCL have provided additional potential therapeutic targets for the treatment of these disorders...
August 28, 2017: Expert Opinion on Biological Therapy
https://www.readbyqxmd.com/read/28816794/x-linked-lymphoproliferative-disease-type-1-in-a-patient-with-the-p-gly93asp-sh2d1a-gene-mutation-and-hemophagocytic-lymphohistiocytosis
#11
Raquel de la Varga-Martínez, Francisco Mora-López, Daniel García-Cuesta, M Paz Garrastazul-Sánchez, Sebastián Quintero, Carmen Rodríguez, Almudena Sampalo
Hemophagocytic lymphohistiocytosis is characterized by uncontrolled activation of the immune system that leads to systemic hyperinflammation. Lymphoproliferative syndrome linked to the X chromosome is a hereditary immunodeficiency characterized by an inability to mount an adequate immune response to an Epstein-Barr virus infection. Hemophagocytic lymphohistiocytosis is one of the main clinical features of X-linked lymphoproliferative syndrome. We report the case of a patient who presented with primary hemophagocytic lymphohistiocytosis associated with Epstein-Barr virus infection without a familial history of immunodeficiency...
August 14, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28814155/frequency-of-a-fas-ligand-gene-variant-associated-with-inherited-feline-autoimmune-lymphoproliferative-syndrome-in-british-shorthair-cats-in-new-zealand
#12
D Aberdein, J S Munday, K E Dittmer, R W Heathcott, L A Lyons
AIMS To determine the frequency of the FAS-ligand gene (FASLG) variant associated with feline autoimmune lymphoproliferative syndrome (FALPS) and the proportion of carriers of the variant in three British shorthair (BSH) breeding catteries in New Zealand. METHODS Buccal swabs were collected from all cats in two BSH breeding catteries from the South Island and one from the North Island of New Zealand. DNA was extracted and was tested for the presence of the FASLG variant using PCR. Cats with the FASLG variant were identified and the frequency of the FASLG variant allele calculated...
August 30, 2017: New Zealand Veterinary Journal
https://www.readbyqxmd.com/read/28769137/is-primary-sj%C3%A3-gren-s-syndrome-a-risk-factor-for-malignancies-different-from-lymphomas-what-does-the-literature-highlight-about-it
#13
REVIEW
Ciro Manzo, Melek Kechida
BACKGROUND: Primary Sjögren's syndrome (pSS) is a chronic systemic autoimmune disease with an elevated risk of developing lymphoproliferative malignancies (LM). Whether pSS is a risk factor or not for non-lymphoma malignancies (NLM) has been scarcely evaluated in the literature. Age is per se a risk factor for malignancies: patients over 70 years old have 4 times higher risk for cancers than adults. Even if the mean age of pSS onset usually is in the 4(th) and 5(th) decade, its onset in patients aged over 65 years (Elderly Onset pSS - EOpSS) is not uncommon...
2017: Reumatologia
https://www.readbyqxmd.com/read/28757383/indications-and-outcome-of-splenectomy-in-hematologic-disease
#14
S Bonnet, A Guédon, J-A Ribeil, F Suarez, J Tamburini, S Gaujoux
Splenectomy is part of the therapeutic arsenal for benign or malignant hematological disorders that constitute the main indication for elective splenectomy. With the development of minimally invasive approaches, and in particular, laparoscopy, as well as the advent of monoclonal antibody therapy, the indications and the outcomes of splenectomy for hematologic disease have changed in recent years. Nonetheless, splenectomy has its place in hemoglobinopathies and hemolytic diseases, improves thrombocytopenia in refractory immune thrombocytopenic purpura, can reverse sequelae linked to voluminous splenomegaly secondary to myelofibrosis, or can be used for diagnostic purposes or for splenomegaly in lymphoproliferative syndromes...
July 27, 2017: Journal of Visceral Surgery
https://www.readbyqxmd.com/read/28751992/prostatic-like-syndrome-in-a-woman-with-chronic-lymphocytic-leukemia-sequential-kinase-inhibitor-therapy
#15
Diego Velasco-Rodríguez, Miguel Piris-Villaespesa, Carmen Soteras, Ana Vallés, José Antonio García-Marco, José Antonio García-Vela
Chronic lymphocytic leukemia (CLL) is an incurable lymphoproliferative disorder with a heterogeneous genetic and clinical course. Two kinase inhibitors, ibrutinib and idelalisib, have demonstrated achievement of complete and durable remissions in relapse/refractory genetically unselected CLL patients. We present a case of relapsed CLL with extensive disease and hourglass deformity of urinary bladder as a result of the compression of two extraperitoneal paravesical soft tissue bulky masses, with excellent response to sequential kinase inhibitor therapy...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28736547/thyroid-involvement-in-hepatitis-c-virus-infected-patients-with-without-mixed-cryoglobulinemia
#16
REVIEW
Clodoveo Ferri, Michele Colaci, Poupak Fallahi, Silvia Martina Ferrari, Alessandro Antonelli, Dilia Giuggioli
Thyroid involvement is a common condition that can be recorded during the natural course of different systemic rheumatic diseases, including the mixed cryoglobulinemia (MC) syndrome or cryoglobulinemic vasculitis. MC is triggered by hepatitis C virus (HCV) chronic infection in the majority of cases; it represents the prototype of autoimmune-lymphoproliferative disorders complicating a significant proportion of patients with chronic HCV infection. HCV is both hepato- and lymphotropic virus responsible for a great number of autoimmune/lymphoproliferative and/or neoplastic disorders...
2017: Frontiers in Endocrinology
https://www.readbyqxmd.com/read/28718061/schnitzler-syndrome-a-review
#17
REVIEW
L Gusdorf, D Lipsker
PURPOSE OF REVIEW: We focus on recent advances in diagnosis and therapeutic strategies, as well as on pathogenesis of Schnitzler syndrome. RECENT FINDINGS: New diagnostic criteria were established, and their external validity was assessed in a retrospective cohort study. The cytokine interleukin-1 (IL-1) plays a crucial role in the pathogenesis of the Schnitzler syndrome, and this explains the spectacular efficiency of IL-1 blocking therapies. The Schnitzler syndrome is now considered as a late-onset acquired autoinflammatory syndrome in which the cytokine IL-1 plays a crucial role...
August 2017: Current Rheumatology Reports
https://www.readbyqxmd.com/read/28710302/a-rare-case-of-atypical-chronic-lymphocytic-leukaemia-presenting-as-nephrotic-syndrome
#18
Martina Soldarini, Lucia Farina, Augusto Genderini, Niccolo Bolli
Chronic lymphocytic leukaemia (CLL) is characterised by a lymphocytosis of mature-appearing clonal CD5+, CD23+ B lymphocytes. CLL cells arise from the bone marrow and infiltrate lymphoid tissues such as lymph nodes and spleen. Presentation is usually through discovery of lymphocytosis or lymphadenopathy. Unusual presentations, especially paraneoplastic syndromes are rare. Here, we describe a rare case presenting with severe nephrotic syndrome associated with the presence of a monoclonal protein in serum. Workup for suspected plasma cell dyscrasia led instead to the diagnosis of bone marrow infiltration by atypical CLL without lymphocytosis...
July 14, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28705776/short-and-medium-term-biological-variation-estimates-of-leukocytes-extended-to-differential-count-and-morphology-structural-parameters-cell-population-data-in-blood-samples-obtained-from-healthy-people
#19
Sabrina Buoro, Anna Carobene, Michela Seghezzi, Barbara Manenti, Aurelio Pacioni, Ferruccio Ceriotti, Cosimo Ottomano, Giuseppe Lippi
BACKGROUND: Recent studies showed that some cell population data (CPD) parameters of neutrophils may be useful for diagnosing myelodysplastic syndromes and sepsis, for the differential diagnosis of acute promyelocytic leukemia, and some CPD parameters of lymphocytes may be a valuable tool for preliminary screening of B cell lymphoproliferative disease. Notwithstanding the knowledge, no information has been made available about their analytical quality specification. This study was aimed to define short- and medium-term biological variation (BV) estimates and reference change value (RCV) of leukocyte count, extended leukocyte differential and CPD...
October 2017: Clinica Chimica Acta; International Journal of Clinical Chemistry
https://www.readbyqxmd.com/read/28702026/lessons-from-genetic-studies-of-primary-immunodeficiencies-in-a-highly-consanguineous-population
#20
REVIEW
Mohamed-Ridha Barbouche, Najla Mekki, Meriem Ben-Ali, Imen Ben-Mustapha
During the last decades, the study of primary immunodeficiencies (PIDs) has contributed tremendously to unravel novel pathways involved in a variety of immune responses. Many of these PIDs have an autosomal recessive (AR) mode of inheritance. Thus, the investigation of the molecular basis of PIDs is particularly relevant in consanguineous populations from Middle East and North Africa (MENA). Although significant efforts have been made in recent years to develop genetic testing across the MENA region, few comprehensive studies reporting molecular basis of PIDs in these settings are available...
2017: Frontiers in Immunology
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