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T-prolymphocytic leukemia

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https://www.readbyqxmd.com/read/29483097/patient-customized-drug-combination-prediction-and-testing-for-t-cell-prolymphocytic-leukemia-patients
#1
Liye He, Jing Tang, Emma I Andersson, Sanna Timonen, Steffen Koschmieder, Krister Wennerberg, Satu Mustjoki, Tero Aittokallio
The molecular pathways that drive cancer progression and treatment resistance are highly redundant and variable between individual patients with the same cancer type. To tackle this complex rewiring of pathway cross-talk, personalized combination treatments targeting multiple cancer growth and survival pathways are required. Here we implemented a computational-experimental drug combination prediction and testing (DCPT) platform for efficient in silico prioritization and ex vivo testing in patient-derived samples to identify customized synergistic combinations for individual cancer patients...
May 1, 2018: Cancer Research
https://www.readbyqxmd.com/read/29449683/adult-t-cell-leukemia-lymphoma-can-be-indistinguishable-from-other-more-common-t-cell-lymphomas-the-university-of-miami-experience-with-a-large-cohort-of-cases
#2
Mahsa Khanlari, Juan Carlos Ramos, Sandra Patricia Sanchez, Jeong Hee Cho-Vega, Alexandra Amador, German Campuzano-Zuluaga, Francisco Vega, Jennifer R Chapman
Adult T-cell leukemia/lymphoma, an aggressive T-cell neoplasm, is causally linked to human T-cell lymphotropic virus type 1 and based on this association has a distinct geographic distribution. In our United States-based practice, whose population is enriched for immigrants from human T-cell lymphotropic virus type 1 endemic areas, we have identified that a subset of adult T-cell leukemia/lymphoma, in the absence of human T-cell lymphotropic virus type 1 identification, are indistinguishable from other more common T-cell neoplasms...
February 15, 2018: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/29449575/actionable-perturbations-of-damage-responses-by-tcl1-atm-and-epigenetic-lesions-form-the-basis-of-t-pll
#3
A Schrader, G Crispatzu, S Oberbeck, P Mayer, S Pützer, J von Jan, E Vasyutina, K Warner, N Weit, N Pflug, T Braun, E I Andersson, B Yadav, A Riabinska, B Maurer, M S Ventura Ferreira, F Beier, J Altmüller, M Lanasa, C D Herling, T Haferlach, S Stilgenbauer, G Hopfinger, M Peifer, T H Brümmendorf, P Nürnberg, K S J Elenitoba-Johnson, S Zha, M Hallek, R Moriggl, H C Reinhardt, M-H Stern, S Mustjoki, S Newrzela, P Frommolt, M Herling
T-cell prolymphocytic leukemia (T-PLL) is a rare and poor-prognostic mature T-cell malignancy. Here we integrated large-scale profiling data of alterations in gene expression, allelic copy number (CN), and nucleotide sequences in 111 well-characterized patients. Besides prominent signatures of T-cell activation and prevalent clonal variants, we also identify novel hot-spots for CN variability, fusion molecules, alternative transcripts, and progression-associated dynamics. The overall lesional spectrum of T-PLL is mainly annotated to axes of DNA damage responses, T-cell receptor/cytokine signaling, and histone modulation...
February 15, 2018: Nature Communications
https://www.readbyqxmd.com/read/29415977/composite-lymphoma-as-co-occurrence-of-advanced-chronic-lymphocytic-leukemia-small-lymphocytic-lymphoma-carrying-trisomy-12-and-t-14-18-and-peripheral-t-cell-lymphoma
#4
Yumi Aoyama, Taiichi Kodaka, Yuriko Zushi, Yuta Goto, Hiroko Tsunemine, Tomoo Itoh, Takayuki Takahashi
Composite lymphoma is defined as the co-occurrence of two types of lymphoma, comprising 1-4% of lymphomas, and the association of B-cell-type chronic lymphocytic leukemia (B-CLL)/small lymphocytic lymphoma and peripheral T-cell lymphoma (PTCL) is rare. Here, we report a case (77-year-old woman) of advanced B-CLL complicated by newly appearing PTCL. Two years after the onset of B-CLL, CLL cells acquired CD38 antigen expression and the disease entity became CLL/prolymphocytic leukemia. Trisomy 12 and t(14;18) karyotypes were observed...
March 16, 2018: Journal of Clinical and Experimental Hematopathology: JCEH
https://www.readbyqxmd.com/read/29407180/allogeneic-hematopoietic-cell-transplantation-in-t-cell-prolymphocytic-leukemia-a-single-center-experience
#5
Bhagirathbhai R Dholaria, Ernesto Ayala, Lubomir Sokol, Taiga Nishihori, Julio C Chavez, Mohammad Hussaini, Ambuj Kumar, Mohamed A Kharfan-Dabaja
BACKGROUND: T- cell prolymphocytic leukemia (T- PLL) is a rare aggressive hematological malignancy. Alemtuzumab, an anti-CD52 humanized monoclonal antibody, is the treatment of choice for remission induction. Allogeneic hematopoietic cell transplantation (allo-HCT) has been described to induce durable remissions and improve survival, but data is limited. PATIENTS AND METHODS: We evaluated clinical outcomes of 11 patients, median age of 56 (range, 43-71) years who underwent allo-HCT for T-PLL...
April 2018: Leukemia Research
https://www.readbyqxmd.com/read/29373342/choriocapillaris-flow-voids-in-cryptococcal-choroiditis-using-optical-coherence-tomography-angiography
#6
Daniel M Vu, Akshay S Thomas, Avni P Finn, Dilraj S Grewal
PURPOSE: To report an unusual case of cryptococcal choroiditis and highlight the multimodal imaging findings, particularly the choriocapillaris flow voids using optical coherence tomography angiography. METHODS: Retrospective review of the clinical course of a single patient with cryptococcal choroiditis. RESULTS: A 69-year-old man undergoing chemotherapy for T-cell prolymphocytic leukemia developed disseminated crytococcal neoformans infection...
January 25, 2018: Retinal Cases & Brief Reports
https://www.readbyqxmd.com/read/29352181/samhd1-is-recurrently-mutated-in-t-cell-prolymphocytic-leukemia
#7
Patricia Johansson, Ludger Klein-Hitpass, Axel Choidas, Peter Habenberger, Bijan Mahboubi, Baek Kim, Anke Bergmann, René Scholtysik, Martina Brauser, Anna Lollies, Reiner Siebert, Thorsten Zenz, Ulrich Dührsen, Ralf Küppers, Jan Dürig
T-cell prolymphocytic leukemia (T-PLL) is an aggressive malignancy with a median survival of the patients of less than two years. Besides characteristic chromosomal translocations, frequent mutations affect the ATM gene, JAK/STAT pathway members, and epigenetic regulators. We here performed a targeted mutation analysis for 40 genes selected from a RNA sequencing of 10 T-PLL in a collection of 28 T-PLL, and an exome analysis of five further cases. Nonsynonymous mutations were identified in 30 of the 40 genes, 18 being recurrently mutated...
January 19, 2018: Blood Cancer Journal
https://www.readbyqxmd.com/read/29296924/t-cell-prolymphocytic-leukemia-in-an-adolescent-with-ataxia-telangiectasia-novel-approach-with-a-jak3-inhibitor-tofacitinib
#8
Geling Li, Emily Waite, Julie Wolfson
A 19-year-old ataxia-telangiectasia patient with T-cell prolymphocytic leukemia harbored 2 JAK3 -activating hotspot mutations.The patient suffered toxicities with chemotherapy, but demonstrated a clinical response to novel use of a JAK3 inhibitor (tofacitinib).
December 26, 2017: Blood Advances
https://www.readbyqxmd.com/read/29112013/cancer-therapy-associated-lymphoproliferative-disorders-an-under-recognized-type-of-immunodeficiency-associated-lymphoproliferative-disorder
#9
Sergio Pina-Oviedo, Roberto N Miranda, L Jeffrey Medeiros
We describe the clinicopathologic features of 17 patients who had a hematologic malignancy of various types, were treated, and subsequently developed a lymphoproliferative disorder (LPD). There were 10 men and 7 women with a median age of 59 years (range, 36 to 83 y). The primary hematologic neoplasms included: 5 chronic lymphocytic leukemia/small lymphocytic lymphoma, 3 plasma cell myeloma, 2 acute monoblastic leukemia, and 1 case each of mixed-phenotype acute leukemia, chronic myeloid leukemia, splenic marginal zone lymphoma, follicular lymphoma, mantle cell lymphoma, T-cell prolymphocytic leukemia, and peripheral T-cell lymphoma...
January 2018: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28972014/first-in-human-response-of-bcl-2-inhibitor-venetoclax-in-t-cell-prolymphocytic-leukemia
#10
Bernd Boidol, Christoph Kornauth, Emiel van der Kouwe, Nicole Prutsch, Lukas Kazianka, Sinan Gültekin, Gregor Hoermann, Marius E Mayerhoefer, Georg Hopfinger, Alexander Hauswirth, Michael Panny, Marie-Bernadette Aretin, Bernadette Hilgarth, Wolfgang R Sperr, Peter Valent, Ingrid Simonitsch-Klupp, Richard Moriggl, Olaf Merkel, Lukas Kenner, Ulrich Jäger, Stefan Kubicek, Philipp B Staber
T-cell prolymphocytic leukemia (T-PLL) is a rare and aggressive T-lymphoid malignancy usually refractory to current treatment strategies and associated with short overall survival. By applying next-generation functional testing of primary patient-derived lymphoma cells using a library of 106 US Food and Drug Administration (FDA)-approved anticancer drugs or compounds currently in clinical development, we set out to identify novel effective treatments for T-PLL patients. We found that the B-cell lymphoma 2 (BCL-2) inhibitor venetoclax (ABT-199) demonstrated the strongest T-PLL-specific response when comparing individual ex vivo drug response in 86 patients with refractory hematologic malignancies...
December 7, 2017: Blood
https://www.readbyqxmd.com/read/28878921/t-cell-prolymphocytic-leukemia-and-tuberculosis-a-puzzling-association
#11
Pascale Cervera, Amélie Gilhot, Christophe Marzac, Frédéric Féger, Ruoping Tang, Nabaz Jaff, Paul Coppo
T-cell prolymphocytic leukemia can result in severe immune T-cell deficiency. Clinicians should be aware of this complication in this rare lymphoid malignancy, and opportunistic infections should be ruled out before the use of usual immunosuppressive procedures such as alemtuzumab and hematopoietic stem cell transplantation.
September 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28819574/sequential-kinase-inhibition-idelalisib-ibrutinib-induces-clinical-remission-in-b-cell-prolymphocytic-leukemia-harboring-a-17p-deletion
#12
H Coelho, M Badior, T Melo
B-cell prolymphocytic leukemia (B-PLL) is a rare lymphoid neoplasm with an aggressive clinical course. Treatment strategies for B-PLL remain to be established, and, until recently, alemtuzumab was the only effective therapeutic option in patients harboring 17p deletions. Herein, we describe, for the first time, a case of B-cell prolymphocytic leukemia harboring a 17p deletion in a 48-year-old man that was successfully treated sequentially with idelalisib-rituximab/ibrutinib followed by allogeneic hematopoietic stem cell transplant (allo-HSCT)...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28804127/discovery-of-novel-drug-sensitivities-in-t-pll-by-high-throughput-ex-vivo-drug-testing-and-mutation-profiling
#13
E I Andersson, S Pützer, B Yadav, O Dufva, S Khan, L He, L Sellner, A Schrader, G Crispatzu, M Oleś, H Zhang, S Adnan-Awad, S Lagström, D Bellanger, J P Mpindi, S Eldfors, T Pemovska, P Pietarinen, A Lauhio, K Tomska, C Cuesta-Mateos, E Faber, S Koschmieder, T H Brümmendorf, S Kytölä, E-R Savolainen, T Siitonen, P Ellonen, O Kallioniemi, K Wennerberg, W Ding, M-H Stern, W Huber, S Anders, J Tang, T Aittokallio, T Zenz, M Herling, S Mustjoki
T-cell prolymphocytic leukemia (T-PLL) is a rare and aggressive neoplasm of mature T-cells with an urgent need for rationally designed therapies to address its notoriously chemo-refractory behavior. The median survival of T-PLL patients is <2 years and clinical trials are difficult to execute. Here we systematically explored the diversity of drug responses in T-PLL patient samples using an ex vivo drug sensitivity and resistance testing platform and correlated the findings with somatic mutations and gene expression profiles...
March 2018: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/28775161/broken-heart-by-t-prolymphocytic-leukemia
#14
Christian Arranto, Alexandar Tzankov
No abstract text is available yet for this article.
August 3, 2017: Blood
https://www.readbyqxmd.com/read/28435655/a-80-year-old-woman-with-b-cell-prolymphocytic-leukemia
#15
Alparslan Merdin, Jale Yıldız, Sinan Dal Mehmet, Merih Kızıl Çakar, Hikmetullah Batgi, Emre Tekgündüz, Aykut Onursever, Fevzi Altuntaş
Prolymhocytic leukemia (PLL) is a rare subtype of lymphocytic leukemias and its cells are immature lymphocytes. It is divided into 2 subgroups: T-PLL and B-PLL according to the lymphocytic origin of the cells. Discriminating B-PLL from other diseases with clinically-similar features is important because of the different treatment approaches and follow-up programs. Hereby, we report a 80-year-old woman presenting with fatigue, leucocytosis and mild anemia. Her peripheral blood smear evaluation revealed 85% prolymphocytes with moderately condensed nuclear chromatin, prominent nucleoli, and a faintly basophilic cytoplasm...
February 23, 2017: Hematology Reports
https://www.readbyqxmd.com/read/28379307/characteristics-outcomes-prognostic-factors-and-treatment-of-patients-with-t-cell-prolymphocytic-leukemia-t-pll
#16
P Jain, E Aoki, M Keating, W G Wierda, S O'Brien, G N Gonzalez, A Ferrajoli, N Jain, P A Thompson, E Jabbour, R Kanagal-Shamanna, S Pierce, A Alousi, C Hosing, I Khouri, Z Estrov, J Cortes, H Kantarjian, F Ravandi, T M Kadia
Background: T-cell prolymphocytic leukemia (T-PLL) is a rare and aggressive disease. In this study, we report our experience from 119 patients with T-PLL. Patients and methods: We reviewed the clinico-pathologic records of 119 consecutive patients with T-PLL, who presented to our institution between 1990 and 2016. Results: One hundred and nineteen patients with T-PLL were analysed. Complex karyotype and aberrations in chromosome 14 were seen in 65% and 52% patients, respectively...
July 1, 2017: Annals of Oncology: Official Journal of the European Society for Medical Oncology
https://www.readbyqxmd.com/read/28377595/gvl-effects-in-t-prolymphocytic-leukemia-evidence-from-mrd-kinetics-and-tcr-repertoire-analyses
#17
L Sellner, M Brüggemann, M Schlitt, H Knecht, D Herrmann, T Reigl, A Krejci, V Bystry, N Darzentas, M Rieger, S Dietrich, T Luft, A D Ho, M Kneba, P Dreger
No abstract text is available yet for this article.
April 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28340878/t-cell-prolymphocytic-leukemia
#18
REVIEW
Amit Sud, Claire Dearden
T-cell prolymphocytic leukemia (T-PLL) is a rare and aggressive T-cell malignancy. T-PLL can be distinguished from other lymphoid diseases by the evaluation and integration of clinical features, morphology, immunophenotyping, cytogenetics, and molecular features. The current therapeutic approach relies on immunotherapy followed by a hematopoietic stem cell transplant in selected cases. Clinical outcomes are generally poor, although insights from genomic and molecular studies may increase our understanding of this disease, with the promise of additional effective therapeutic options...
April 2017: Hematology/oncology Clinics of North America
https://www.readbyqxmd.com/read/28324286/prolymphocytic-leukemia-new-insights-in-diagnosis-and-in-treatment
#19
REVIEW
Aude Collignon, Anne Wanquet, Elsa Maitre, Edouard Cornet, Xavier Troussard, Thérèse Aurran-Schleinitz
PURPOSE OF REVIEW: We aimed to produce a comprehensive update on clinical and biological data regarding two rare lymphoid neoplasms, B and T prolymphocytic leukemias, and assess therapeutic management in the light of new molecular insights and the advent of targeted therapies. RECENT FINDINGS: B cell prolymphocytic leukemia (B-PLL) diagnosis remains challenging in the absence of clear immunophenotypic or cytogenetic signature and overlap with mantle cell lymphoma...
April 2017: Current Oncology Reports
https://www.readbyqxmd.com/read/28245368/-clinical-and-immunophenotypic-properties-of-small-cell-variant-of-t-cell-prolymphocytic-leukemia
#20
Ya-Ping Yu, Li-Ping Wang, Ping Song, Jian-Gang Mei, Zhi-Ming An, Xiao-Gang Zhou, Feng Li, Yu-Mei Tang, Yong-Ping Zhai
OBJECTIVE: To investigate the clinical, morphologic and immunophenotypic properties of the patients with small cell variant of T-cell prolymphocytic leukaemia(T-PLL). METHODS: Peripheral blood and bone marrow cytomorphologic and immunophenotypic examination, and T-cell receptor(TCR) gene rearrangement detection were used to verify the diagnosis for 2 patients with lymphocytosis. Two patients were treated with combined chemotherapeutic protocol based on fludarabine...
February 2017: Zhongguo Shi Yan Xue Ye Xue za Zhi
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