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Lymphoproliferative syndromes

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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
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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...
July 10, 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
#8
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
https://www.readbyqxmd.com/read/28686783/schnitzler-syndrome-with-delirium-and-vertigo-the-utility-of-neurologic-manifestations-in-diagnosis
#9
Stanislav N Tolkachjov, David A Wetter
<p>Schnitzler syndrome (SS) is an autoinflammatory dermatosis that often goes undiagnosed for 5-6 years. Patients typically carry a diagnosis of urticaria; however, their cutaneous symptoms fail to respond to typical urticaria therapies and lack symptoms such as pruritus. Additionally, patients with SS may see multiple providers for nonspecific complaints of fever, lymphadenopathy, arthralgias, and bone pain. A correct diagnosis is paramount, as close to 20% of patients may develop a lymphoproliferative disorder and appropriate treatment may ameliorate all symptoms...
June 1, 2017: Journal of Drugs in Dermatology: JDD
https://www.readbyqxmd.com/read/28684198/flow-cytometry-based-diagnosis-of-primary-immunodeficiency-diseases
#10
REVIEW
Hirokazu Kanegane, Akihiro Hoshino, Tsubasa Okano, Takahiro Yasumi, Taizo Wada, Hidetoshi Takada, Satoshi Okada, Motoi Yamashita, Tzu-Wen Yeh, Ryuta Nishikomori, Masatoshi Takagi, Kohsuke Imai, Hans D Ochs, Tomohiro Morio
Primary immunodeficiencies (PIDs) are a heterogeneous group of inherited diseases of the immune system. The definite diagnosis of PID is ascertained by genetic analysis; however, this takes time and is costly. Flow cytometry provides a rapid and highly sensitive tool for diagnosis of PIDs. Flow cytometry can evaluate specific cell populations and subpopulations, cell surface, intracellular and intranuclear proteins, biologic effects associated with specific immune defects, and certain functional immune characteristics, each being useful for the diagnosis and evaluation of PIDs...
July 3, 2017: Allergology International: Official Journal of the Japanese Society of Allergology
https://www.readbyqxmd.com/read/28680340/successful-treatment-of-pulmonary-candidiasis-and-aspergillosis-in-patient-with-refractory-hodgkin-lymphoma-using-micafungin-case-study-and-brief-literature-review
#11
Marta Barańska, Renata Kroll-Balcerzak, Lidia Gil, Joanna Rupa-Matysek, Mieczysław Komarnicki
The number of patients with hematological malignancies who develop invasive fungal disease (IFD) has increased dramatically in recent decades. This increase is attributed to impairment of the host immune system due to intensive cytotoxic chemotherapies, use of corticosteroids and profound immunosuppression after hematopoietic stem cell transplantation (HSCT). Additionally, the increasing prevalence of fungal infections caused by emerging and rare pathogens, IFD of mixed etiology or of atypical localization is observed...
2017: Central-European Journal of Immunology
https://www.readbyqxmd.com/read/28679991/acquired-immune-mediated-von-willebrand-syndrome-accompanied-by-antiphospholipid-syndrome
#12
Nobuhiko Kobayashi, Yoshiyuki Ogawa, Kunio Yanagisawa, Takuma Ishizaki, Hideki Uchiumi, Nobuaki Suzuki, Tadashi Matsushita, Akitada Ichinose, Hiroshi Handa
Acquired von Willebrand syndrome (AvWS) is a rare bleeding disorder with laboratory findings resembling those of congenital von Willebrand disease. AvWS usually occurs in association with a variety of underlying disorders, such as lymphoproliferative disease or cardiovascular disease, but autoimmune AvWS is very rare. We now describe the case of a 42-year-old woman with autoimmune AvWS with concurrent antiphospholipid syndrome (APS). The patient was suffering from epistaxis and menorrhagia from few years prior to referral...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28672090/efficacy-of-mycophenolate-on-lung-disease-and-autoimmunity-in-children-with-immunodeficiency
#13
Giorgia Bucciol, Angelamaria Petrone, Maria Caterina Putti
The autoimmune manifestations of primary immunodeficiencies, such as autoimmune lymphoproliferative syndrome (ALPS) and common variable immunodeficiency (CVID), often constitute a great therapeutic challenge and have a significant impact on patients' morbidity and mortality. The most common autoimmune presentations are autoimmune cytopenias, but organ-related autoimmunity is also frequently observed. From a pulmonology perspective, granulomatous/lymphocytic interstitial lung disease (GLILD) is a severe immunological complication which significantly worsens the clinical outcome of these patients and for which there are currently few guidelines or protocols for treatment...
July 3, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28668589/rare-splicing-defects-of-fas-underly-severe-recessive-autoimmune-lymphoproliferative-syndrome
#14
N Agrebi, I Ben-Mustapha, N Matoussi, N Dhouib, M Ben-Ali, N Mekki, M Ben-Ahmed, B Larguèche, S Ben Becher, M Béjaoui, M R Barbouche
Autoimmune lymphoproliferative syndrome (ALPS) is a prototypic disorder of impaired apoptosis characterized by autoimmune features and lymphoproliferation. Heterozygous germline or somatic FAS mutations associated with preserved protein expression have been described. Very rare cases of homozygous germline FAS mutations causing severe autosomal recessive form of ALPS with a complete defect of Fas expression have been reported. We report two unrelated patients from highly inbred North African population showing a severe ALPS phenotype and an undetectable Fas surface expression...
June 29, 2017: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/28668112/pearls-and-pitfalls-autoimmune-lymphoproliferative-syndrome-and-autoimmune-lymphoproliferative-syndrome-like-disease
#15
Anne K Bartels, Taylor A Banks, Jeannie L Bay
A case of autoimmune lymphoproliferative syndrome (ALPS) was presented, followed by a discussion of the clinical characteristics, pathophysiology, diagnosis, and management of this disease. Clinical pearls and pitfalls are emphasized for the use of the practicing allergist and the fellow in-training. The diagnosis of ALPS was guided by published criteria. A careful history and workup were needed to exclude other possible etiologies for the patient's symptoms and physical findings. ALPS often carries significant morbidity and is best managed through a multidisciplinary approach...
July 1, 2017: Allergy and Asthma Proceedings:
https://www.readbyqxmd.com/read/28648940/upregulation-of-inhibitory-signaling-receptor-programmed-death-marker-1-pd-1-in-disease-evolution-from-cutaneous-lymphoid-dyscrasias-to-mycosis-fungoides-and-sezary-s-syndrome
#16
Giang Huong Nguyen, Luke C Olson, Cynthia M Magro
BACKGROUND: Negative immunoregulatory checkpoints impede effective immune responses to tumor and reduce the action of anticancer agents. One such example is programmed death marker-1 (PD-1), an inhibitory signaling receptor expressed on activated and regulatory T-cells. PD-1 expression was reported in a few reports, but the expression profile of PD-1 and mycosis fungoides (MF) remains largely to be characterized. DESIGN: In this study, skin biopsies from 42 prelymphomatous T-cell dyscrasias (CLD), 9 Sezary's syndrome (SS), 103 MF, and 20 CD30+ lymphoproliferative diseases (LPD) were examined for PD-1 expression using immunohistochemistry...
June 2017: Annals of Diagnostic Pathology
https://www.readbyqxmd.com/read/28641577/a-case-report-of-septic-shock-syndrome-caused-by-s-pneumoniae-in-an-immunocompromised-patient-despite-of-vaccination
#17
Josef Singer, Christoph Testori, Peter Schellongowski, Ammon Handisurya, Catharina Müller, Eva-Maria Reitter, Wolfgang Graninger, Paul Knöbl, Thomas Staudinger, Stefan Winkler, Florian Thalhammer
BACKGROUND AND CASE PRESENTATION: We report a case of septic shock syndrome caused by Streptococcus pneumoniae in a patient who had undergone splenectomy due to an autoimmune lymphoproliferative syndrome (ALPS), which is characterized as a dysfunction of immunoregulation. Although the patient was vaccinated with a conjugated polysaccharide vaccine after the splenectomy, he was still susceptible to S. pneumoniae infection, because the isolated serovar (24F), a serovar long thought to be apathogenic, is not covered by any vaccine currently approved, neither a conjugated nor an unconjugated polysaccharide one...
June 22, 2017: BMC Infectious Diseases
https://www.readbyqxmd.com/read/28617889/erdheim-chester-disease-presenting-with-histiocytic-colitis-and-cytokine-storm
#18
George P Christophi, Yeshika Sharma, Quader Farhan, Umang Jain, Ted Walker, Gregory S Sayuc, Deborah C Rubin
BACKGROUND: Non-Langerhans histiocytosis is a group of inflammatory lymphoproliferative disorders originating from non-clonal expansion of hematopoietic stem cells into cytokine-secreting dendritic cells or macrophages. Erdheim-Chester Disease (ECD) is a rare type of non-Langerhans cell histiocytosis characterized by tissue inflammation and injury caused by macrophage infiltration and histologic findings of foamy histiocytes. Often ECD involves the skeleton, retroperitoneum and the orbits...
June 2017: Journal of Gastrointestinal and Liver Diseases: JGLD
https://www.readbyqxmd.com/read/28579554/stat3-gain-of-function-mutations-associated-with-autoimmune-lymphoproliferative-syndrome-like-disease-deregulate-lymphocyte-apoptosis-and-can-be-targeted-by-bh3-mimetic-compounds
#19
Schafiq Nabhani, Cyrill Schipp, Hagit Miskin, Carina Levin, Sergey Postovsky, Tal Dujovny, Ariel Koren, Dan Harlev, Anne-Marie Bis, Franziska Auer, Baerbel Keller, Klaus Warnatz, Michael Gombert, Sebastian Ginzel, Arndt Borkhardt, Polina Stepensky, Ute Fischer
Autoimmune lymphoproliferative syndrome (ALPS) is typically caused by mutations in genes of the extrinsic FAS mediated apoptotic pathway, but for about 30% of ALPS-like patients the genetic diagnosis is lacking. We analyzed 30 children with ALPS-like disease of unknown cause and identified two dominant gain-of-function mutations of the Signal Transducer And Activator Of Transcription 3 (STAT3, p.R278H, p.M394T) leading to increased transcriptional activity. Hyperactivity of STAT3, a known repressor of FAS, was associated with decreased FAS-mediated apoptosis, mimicking ALPS caused by FAS mutations...
June 1, 2017: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/28573819/use-of-eltrombopag-for-secondary-immune-thrombocytopenia-in-clinical-practice
#20
Tomás J González-López, María T Alvarez-Román, Cristina Pascual, Blanca Sánchez-González, Fernando Fernández-Fuentes, Gloria Pérez-Rus, José A Hernández-Rivas, Silvia Bernat, José M Bastida, María P Martínez-Badas, Violeta Martínez-Robles, Inmaculada Soto, Pavel Olivera, Estefanía Bolaños, Rafael Alonso, Laura Entrena, Marta Gómez-Nuñez, Arancha Alonso, María Yera Cobo, Isabel Caparrós, María Tenorio, Esther Arrieta-Cerdán, Elsa Lopez-Ansoar, Javier García-Frade, José R González-Porras
Eltrombopag is a second-line treatment in primary immune thrombocytopenia (ITP). However, its role in secondary ITP is unknown. We evaluated the efficacy and safety of eltrombopag in secondary ITP in daily clinical practice. Eighty-seven secondary ITP patients (46 with ITP secondary to autoimmune syndromes, 23 with ITP secondary to a neoplastic disease subtype: lymphoproliferative disorders [LPDs] and 18 with ITP secondary to viral infections) who had been treated with eltrombopag were retrospectively evaluated...
June 1, 2017: British Journal of Haematology
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