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Richter's transformation

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https://www.readbyqxmd.com/read/29159711/treatment-of-richter-s-syndrome
#1
REVIEW
Adalgisa Condoluci, Davide Rossi
Based on the available literature, mostly derived from retrospective or non-randomized phase I or II studies, it is difficult to define an optimized treatment approach for patients developing Richter's syndrome (RS). Early recognition of chronic lymphocytic leukemia (CLL) patients presenting clinical features suspected for a transformation is useful to avoid exposing them to multiple lines of therapy that, being targeted to CLL progression, have poor efficacy against RS. Because of the low specificity (~ 50-60%) of clinical signs of RS (such as rapid and discordant bulky localized lymphadenopathies, elevated LDH levels, emergent physical deterioration, and/or fever in the absence of infection), a (18)FDG PET/CT and a biopsy are recommended to confirm RS...
November 21, 2017: Current Treatment Options in Oncology
https://www.readbyqxmd.com/read/29146406/central-nervous-involvement-by-chronic-lymphocytic-leukaemia
#2
Dariusz Szczepanek, Ewa Wąsik-Szczepanek, Agnieszka Szymczyk, Małgorzata Wach, Maria Cioch, Monika Podhorecka, Ewelina Grywalska, Marek Hus
Inclusion of the central nervous system (CNS) in the course of chronic lymphocytic leukaemia (CLL) is rare. At the moment no risk factors or proven treatment methods are known. The disease is described both in its early phase and during its acceleration period, thus it has been suggested that there might be independent mechanisms influencing the development of this condition. As there are no unified diagnostic procedure algorithms each patient needs to be assessed individually. CLL can manifest mostly in elderly people, for whom a possibility of development of neurological disorders with their aetiology different from leukaemia, should also be taken into consideration...
November 1, 2017: Neurologia i Neurochirurgia Polska
https://www.readbyqxmd.com/read/29118007/pd-1-expression-and-clinical-pd-1-blockade-in-b-cell-lymphomas
#3
Zijun Y Xu-Monette, Jianfeng Zhou, Ken H Young
PD-1 blockade targeting the PD-1 immune checkpoint has demonstrated unprecedented clinical efficacy in the treatment of advanced cancers including hematologic malignancies. This article reviews the landscape of PD-1/PD-L1 expression and current PD-1 blockade immunotherapy trials in B-cell lymphomas. Most notably, in relapsed/refractory classical Hodgkin lymphoma, which frequently has increased PD-1(+) tumor-infiltrating T cells, 9p24 genetic alteration and high PD-L1 expression, anti-PD-1 monotherapy has demonstrated remarkable objective response rates (ORR) of 65-87% and durable disease control in phase I/II clinical trials...
November 8, 2017: Blood
https://www.readbyqxmd.com/read/29063177/approach-to-richter-transformation-of-chronic-lymphocytic-leukemia-in-the-era-of-novel-therapies
#4
REVIEW
Maliha Khan, Rabbia Siddiqi, Philip A Thompson
Chronic lymphocytic leukemia (CLL) has a highly variable clinical course. About 2-10% of CLL patients develop aggressive histological transformation, most commonly to diffuse large B cell lymphoma (DLBCL), historically called Richter transformation (RT). Clinical features suggestive of RT include elevated LDH and non-specific symptoms such as fever, weight loss, and lymphadenopathy. 18-fluorodeoxyglucose (18-FDG) uptake is increased on PET scan (standardized uptake value max most commonly ≥ 10). PET/CT study can identify optimal site for excisional biopsy, which is the gold standard for RT diagnosis, as well as aid in disease staging and prognostication...
October 23, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/29051895/chronic-lymphocytic-leukemia-a-rare-cause-of-pathological-fracture-of-the-femur
#5
Parita Soni, Nidhi Aggarwal, Anand Rai, Vivek Kumar, Kamholz Stephan, Yoon Taek, Kupfer Yizhak
The incidence rate of chronic lymphocytic leukemia (CLL) in the United States is approximately 0.005%; men are at slightly higher risk than women. Bony involvement or pathological fracture rarely occurs in CLL, and it may be the initial presentation. An 85-year-old woman presented with acute respiratory failure secondary to pneumonia. Symptomatology included dyspnea. She was found to have pathological fracture of the femur caused by CLL. The diagnosis of CLL had been made 6 years previously, but the patient had refused therapy...
October 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/29031279/rotationally-inelastic-collisions-of-excited-nak-and-nacs-molecules-with-noble-gas-and-alkali-atom-perturbers
#6
J Jones, K Richter, T J Price, A J Ross, P Crozet, C Faust, R F Malenda, S Carlus, A P Hickman, J Huennekens
We report measurements of rate coefficients at T ≈ 600 K for rotationally inelastic collisions of NaK molecules in the 2(A)(1)Σ(+) electronic state with helium, argon, and potassium atom perturbers. Several initial rotational levels J between 14 and 44 were investigated. Collisions involving molecules in low-lying vibrational levels (v = 0, 1, and 2) of the 2(A)(1)Σ(+) state were studied using Fourier-transform spectroscopy. Collisions involving molecules in a higher vibrational level, v = 16, were studied using pump/probe, optical-optical double resonance spectroscopy...
October 14, 2017: Journal of Chemical Physics
https://www.readbyqxmd.com/read/29028194/temporal-scalability-of-dynamic-volume-data-using-mesh-compensated-wavelet-lifting
#7
Wolfgang Schnurrer, Niklas Pallast, Thomas Richter, Andre Kaup
Due to their high resolution, dynamic medical 2D+t and 3D+t volumes from computed tomography (CT) and magnetic resonance tomography (MR) reach a size which makes them very unhandy for teleradiologic applications. A lossless scalable representation offers the advantage of a down-scaled version which can be used for orientation or previewing, while the remaining information for reconstructing the full resolution is transmitted on demand. The wavelet transform offers the desired scalability. A very high quality of the lowpass sub-band is crucial in order to use it as a down-scaled representation...
October 12, 2017: IEEE Transactions on Image Processing: a Publication of the IEEE Signal Processing Society
https://www.readbyqxmd.com/read/29024083/stimulated-transitions-of-directed-nonequilibrium-self-assemblies
#8
Alexander A Steinschulte, Andrea Scotti, Khosrow Rahimi, Oleksii Nevskyi, Alex Oppermann, Sabine Schneider, Steffen Bochenek, Marie F Schulte, Karen Geisel, Felicitas Jansen, Andre Jung, Sabrina Mallmann, Roland Winter, Walter Richtering, Dominik Wöll, Ralf Schweins, Nicholas J Warren, Felix A Plamper
Near-equilibrium stimulus-responsive polymers have been used extensively to introduce morphological variations in dependence of adaptable conditions. Far-less-well studied are triggered transformations at constant conditions. These require the involvement of metastable states, which are either able to approach the equilibrium state after deviation from metastability or can be frozen on returning from nonequilibrium to equilibrium. Such functional nonequilibrium macromolecular systems hold great promise for on-demand transformations, which result in substantial changes in their material properties, as seen for triggered gelations...
October 10, 2017: Advanced Materials
https://www.readbyqxmd.com/read/28994094/novel-prognostic-molecular-factors-a-quantum-leap-in-the-field-of-chronic-lymphocytic-leukemia
#9
Ewelina Zakrzewska, Marta Pirog, Joanna Purkot, Krzysztof Giannopoulos
Cytogenetic lesions do not completely explain clinical heterogeneity of chronic lymphocytic leukemia (CLL). The 2016 revision of the World Health Organization classification 2008 introduced that molecular lesions of TP53, NOTCH1, SF3B1 and BIRC3 have potential clinical relevance and could be integrated into an updated risk profile. The negative clinical implications of TP53 disruptions are well constituted and patients with these mutations should be considered for novel, small molecule signal transduction inhibitors therapies...
October 10, 2017: Folia Histochemica et Cytobiologica
https://www.readbyqxmd.com/read/28975027/second-lung-malignancy-and-richter-syndrome-in-chronic-lymphocytic-leukemia-case-report-and-literature-review
#10
Ghassen Soussi, Selsabil Daboussi, Samira Mhamdi, Zied Moatemri, Hela Ghedira, Chiraz Aichaouia, Mohsen Khadhraoui, Faouzi El Mezni, Rezaik Cheikh
BACKGROUND: Chronic lymphocytic leukemia (CLL) is the most frequent lymphoproliferative disease. Transformation into Richter disease and occurrence of second malignancies involving the lungs are rare complications. The hallmarks of any thoracic involvement are still unknown. CASE PRESENTATION: We report a case of a 56-year-old male patient, with history of tobacco smoking, who presented with recurrent hemoptysis, fatigue and weight loss. Physical examination was normal except a slightly enlarged supraclavicular lymph node...
2017: Multidisciplinary Respiratory Medicine
https://www.readbyqxmd.com/read/28970470/nfat2-is-a-critical-regulator-of-the-anergic-phenotype-in-chronic-lymphocytic-leukaemia
#11
Melanie Märklin, Jonas S Heitmann, Alexander R Fuchs, Felicia M Truckenmüller, Michael Gutknecht, Stefanie Bugl, Sebastian J Saur, Juliane Lazarus, Ursula Kohlhofer, Leticia Quintanilla-Martinez, Hans-Georg Rammensee, Helmut R Salih, Hans-Georg Kopp, Michael Haap, Andreas Kirschniak, Lothar Kanz, Anjana Rao, Stefan Wirths, Martin R Müller
Chronic lymphocytic leukaemia (CLL) is a clonal disorder of mature B cells. Most patients are characterised by an indolent disease course and an anergic phenotype of their leukaemia cells, which refers to a state of unresponsiveness to B cell receptor stimulation. Up to 10% of CLL patients transform from an indolent subtype to an aggressive form of B cell lymphoma over time (Richter´s syndrome) and show a significantly worse treatment outcome. Here we show that B cell-specific ablation of Nfat2 leads to the loss of the anergic phenotype culminating in a significantly compromised life expectancy and transformation to aggressive disease...
October 2, 2017: Nature Communications
https://www.readbyqxmd.com/read/28949401/kronisk-myeloisk-leukemi-f%C3%A3-rebild-f%C3%A3-r-m%C3%A3-lstyrd-terapi-revolutionerande-%C3%A3-verlevnadsvinster-med-definitiv-bot-i-sikte-och-kraftigt-f%C3%A3-rb%C3%A3-ttrad-h%C3%A3-lsoekonomi
#12
Johan Richter, Leif Stenke
Chronic myeloid leukemia - a model disease for targeted therapy Chronic myeloid leukemia (CML) pioneered as the first human malignancy linked to a specific cytogenetic aberration (the Philadelphia chromosome), which led the way to specific targeted therapies with imatinib (Glivec) and later tyrosine kinase inhibitors (TKI). Continuous TKI administration, blocking the oncogenic fusion protein Bcr-Abl, has revolutionized the outcome of CML, transforming an almost uniformly deadly disease into a chronic disorder with a near to normal life expectancy for many patients...
September 21, 2017: Läkartidningen
https://www.readbyqxmd.com/read/28937430/cutaneous-richter-syndrome-mimicking-primary-cutaneous-cd4-positive-small-medium-t-cell-lymphoma-case-report-and-review-of-the-literature
#13
Miguel Rito, José Cabeçadas, Joaninha Costa Rosa, Mariana Cravo, Alistair Robson
Richter syndrome represents the transformation of chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma into an aggressive large B-cell lymphoma. Skin involvement is exceptionally rare, with <20 cases reported and its presence as the first presentation of CLL/small lymphocytic lymphoma, as an isolated skin lesion has never been described. Primary cutaneous CD4-positive small/medium T-cell lymphoma (CD4 PCSM-TCL) characteristically presents with a dense dermal infiltrate consisting primarily of small-/medium-sized pleomorphic T-cells and less than 30% large pleomorphic cells...
September 12, 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28929419/high-resolution-mr-imaging-for-dental-impressions-a-feasibility-study
#14
Julian Boldt, Kurt Rottner, Marc Schmitter, Andreas Hopfgartner, Peter Jakob, Ernst-Jürgen Richter, Olga Tymofiyeva
OBJECTIVES: Magnetic resonance imaging is an emerging technology in dental medicine. While low-resolution MRI has especially provided means to examine the temporomandibular joint due to its anatomic inaccessibility, it was the goal of this study to assess whether high-resolution MRI is capable of delivering a dataset sufficiently precise enough to serve as digital impression of human teeth. MATERIALS AND METHODS: An informed and consenting patient in need of dental restoration with fixed partial dentures was chosen as subject...
September 19, 2017: Clinical Oral Investigations
https://www.readbyqxmd.com/read/28878900/intravascular-large-b-cell-lymphoma-presenting-as-richter-s-syndrome-with-cerebral-involvement-in-a-patient-with-chronic-lymphocytic-leukemia
#15
Robert Puckrin, Paula Pop, Zeina Ghorab, Julia Keith, Lisa Chodirker, Yulia Lin, Jeannie Callum
Intravascular large B-cell lymphoma (IVLBCL) is an aggressive non-Hodgkin's lymphoma which can present with B symptoms, rash, and neurological deterioration. Up to 10% of cases of IVLBCL are associated with other hematological neoplasms, including this extremely rare presentation of IVLBCL as Richter's transformation in chronic lymphocytic leukemia.
September 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28837797/ocular-adnexal-lymphoma-dissimilar-to-systemic-lymphoma
#16
Tal J Rubinstein, Hassan A Aziz, Claudine Bellerive, Bryan S Sires, Andrew W Hing, Gabriel Habermehl, Eric Hsi, Arun D Singh
Ocular adnexal lymphoma (OAL) and intraocular lymphoma, whether occurring simultaneously or sequentially, are often similar to associated systemic lymphoma. We describe 4 cases of OAL or intraocular lymphoma with dissimilar systemic lymphoma. Two of the cases represent Richter transformation of chronic lymphocytic leukemia/small cell lymphoma (CLL/SLL) into diffuse large B-cell lymphoma (DLBCL). The third patient developed conjunctival extranodal marginal zone lymphoma (EMZL) following treatment for Hodgkin lymphoma...
August 21, 2017: Survey of Ophthalmology
https://www.readbyqxmd.com/read/28803824/the-clinical-spectrum-of-hepatic-manifestations-in-chronic-lymphocytic-leukemia
#17
REVIEW
Natalia Kreiniz, Ofrat Beyar Katz, Aaron Polliack, Tamar Tadmor
Chronic lymphocytic leukemia (CLL) is the most common leukemia in the Western world, characterized by the presence of long-lived circulating leukemic cells in the peripheral blood that may infiltrate all organs, particularly those of the reticulo-endothelial system. Liver enlargement and elevation of liver enzymes related to specific involvement by the underlying disease are well-recognized features in these patients. In CLL, the differential diagnosis of liver disorders is broad and includes liver infiltration by leukemic cells, immunologic manifestations associated with CLL, primary and secondary hepatic malignancies, drug-induced hepatotoxicity, infections, and Richter transformation...
July 23, 2017: Clinical Lymphoma, Myeloma & Leukemia
https://www.readbyqxmd.com/read/28776024/a-laboratory-nanoseismological-study-on-deep-focus-earthquake-micromechanics
#18
Yanbin Wang, Lupei Zhu, Feng Shi, Alexandre Schubnel, Nadege Hilairet, Tony Yu, Mark Rivers, Julien Gasc, Ahmed Addad, Damien Deldicque, Ziyu Li, Fabrice Brunet
Global earthquake occurring rate displays an exponential decay down to ~300 km and then peaks around 550 to 600 km before terminating abruptly near 700 km. How fractures initiate, nucleate, and propagate at these depths remains one of the greatest puzzles in earth science, as increasing pressure inhibits fracture propagation. We report nanoseismological analysis on high-resolution acoustic emission (AE) records obtained during ruptures triggered by partial transformation from olivine to spinel in Mg2GeO4, an analog to the dominant mineral (Mg,Fe)2SiO4 olivine in the upper mantle, using state-of-the-art seismological techniques, in the laboratory...
July 2017: Science Advances
https://www.readbyqxmd.com/read/28756015/mechanical-thrombectomy-in-acute-stroke-five-years-of-experience-in-poland
#19
Agnieszka Słowik, Marcin Wnuk, Paweł Brzegowy, Joanna Chrzanowska-Waśko, Aleksandra Golenia, Bartłomiej Łasocha, Dorota Włoch-Kopeć, Antoni Ferens, Wojciech Serednicki, Piotr Jarocki, Halina Bartosik-Psujek, Rafał Kaczorowski, Edward Filip, Monika Grzegorzak, Jarosław Homa, Janusz Darocha, Daniel Dudek, Wiesław Guz, Konrad Rejdak, Piotr Luchowski, Joanna Wojczal, Michał Sojka, Michał Górnik, Sylwia Stachowicz, Jacek Jaworski, Kinga Buraczyńska, Remigiusz Ficek, Anna Szczepańska-Szerej, Tomasz Jargiełło, Małgorzata Szczerbo-Trojanowska, Anetta Lasek-Bal, Przemysław Puz, Aldona Warsz-Wianecka, Arkadiusz Stęposz, Krzysztof Ziaja, Wacław Kuczmik, Tomasz Urbanek, Damian Ziaja, Witold Tomalski, Adam Kobayashi, Przemysław Richter, Andrzej Płoński, Marcin Kotkowski, Wojciech Czepiel, Iwona Kurkowska-Jastrzębska, Halina Sienkiewicz-Jarosz, Anna Członkowska, Beata BłażejewskaHyżorek, Danuta Ryglewicz, Magdalena Konopko, Edyta Brelak, Jacek Antecki, Igor Szydłowski, Maciej Włosek, Adam Stępień, Krzysztof Brzozowski, Jacek Staszewski, Piotr Piasecki, Piotr Zięcina, Izabela Wołoszyńska, Norbert Kolmaga, Jerzy Narloch, Tomasz Hasiec, Jacek Gawłowicz, Monika Pędracka, Jacek Porębiak, Bogumił Grzechnik, Vadym Matsibora, Michał Frąszczak, Marcin Leus, Maciej Mazgaj, Violetta Palacz-Duda, Grzegorz Meder, Wojciech Skura, Piotr Płeszka, Milena Świtońska, Krzysztof Słomiński, Józef Kościelniak, Paulina Sobieszak-Skura, Magdalena Konieczna-Brazis, Olgierd Rowiński, Andrzej Opuchlik, Anatol Mickielewicz, Beata Szyluk, Piotr Szczudlik, Anna Kostera-Pruszczyk, Maciej Jaworski, Rafał Maciąg, Jarosław Żyłkowski, Bożena Adamkiewicz, Wojciech Szubert, Jarosław Chrząstek, Marek Raźniewski, Agnieszka Pawelec, Paweł Wilimborek, Ryszard Wagner, Paweł Pilarski, Paweł Gierach, Jan Baron, Wojciech Gruszka, Stanisław Ochudło, Agnieszka Krzak-Kubica, Monika Rudzińska-Bar, Miłosz Zbroszczyk, Kamila Smulska, Michał Arkuszewski, Dorota Różański, Dariusz Koziorowski, Izabela Meisner-Kramarz, Stanisław Szlufik, Artur Zaczyński, Krzysztof Kądziołka, Kazimierz Kordecki, Michał Zawadzki, Mirosław Ząbek, Bartosz Karaszewski, Dariusz Gąsecki, Paweł Łowiec, Waldemar Dorniak, Tomasz Gorycki, Edyta Szurowska, Ewa Wierzchowska-Cioch, Tomasz Smyk, Beata Szajnoga, Marcin Bachta, Kajetan Mazurek, Magdalena Piwowarska, Wojciech Kociemba, Artur Drużdż, Adrian Dąbrowski, Michał Glonek, Małgorzata Wawrzyniak, Radosław Kaźmierski, Robert Juszkat, Witold Tomalski, Adam Heliosz, Adam Ryszczyk, Jacek Zwiernik, Grzegorz Wasilewski, Andrzej Tutaj, Grzegorz Dałek, Krzysztof Nosek, Sławomir Bereza, Katarzyna Lubkowska, Jerzy Kamienowski, Piotr Sobolewski, Adam Bielecki, Maciej Miś, Marcin Miś, Maria Krużewska-Orłowska, Jan Kochanowicz, Zenon Mariak, Marta Jakoniuk, Grzegorz Turek, Urszula Łebkowska, Andrzej Lewszuk, Kazimierz Kordecki, Tomasz Dziedzic, Tadeusz Popiela
OBJECTIVES: Mechanical thrombectomy (MT) is not reimbursed by the Polish public health system. We present a description of 5 years of experience with MT in acute stroke in Comprehensive Stroke Centers (CSCs) in Poland. METHODS AND RESULTS: We retrospectively analyzed the results of a structured questionnaire from 23 out of 25 identified CSCs and 22 data sets that include 61 clinical, radiological and outcome measures. RESULTS: Most of the CSCs (74%) were founded at University Hospitals and most (65...
September 2017: Neurologia i Neurochirurgia Polska
https://www.readbyqxmd.com/read/28754115/in-vivo-plug-and-play-a-modular-multi-enzyme-single-cell-catalyst-for-the-asymmetric-amination-of-ketoacids-and-ketones
#20
Judith E Farnberger, Elisabeth Lorenz, Nina Richter, Volker F Wendisch, Wolfgang Kroutil
BACKGROUND: Transaminases have become a key tool in biocatalysis to introduce the amine functionality into a range of molecules like prochiral α-ketoacids and ketones. However, due to the necessity of shifting the equilibrium towards the product side (depending on the amine donor) an efficient amination system may require three enzymes. So far, this well-established transformation has mainly been performed in vitro by assembling all biocatalysts individually, which comes along with elaborate and costly preparation steps...
July 28, 2017: Microbial Cell Factories
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