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Indolent lymphoma

Mark D Bartholomä
While radioimmunotherapy (RIT) for the treatment of hematological malignancies such as indolent B-cell lymphoma has proven quite successful, clinical results of RIT in solid tumors have only been moderate in the past. The reasons were manifold and can be mostly attributed to the different biological properties of solid tumors vs. hematological cancers. Furthermore, the slow clearance of the radiolabelled antibody prevents the use of radiation doses necessary to achieve clinical responses. The long biological half-life of radioimmunoconjugates results in high background levels and is the main reason for radiation related toxicities...
March 9, 2018: Journal of Labelled Compounds & Radiopharmaceuticals
Aby Z Philip
Splenic marginal zone lymphoma (SMZL) is a rare indolent B-cell malignancy involving the spleen and bone marrow. Various cytogenetic abnormalities with prognostic value have been identified in SMZL. Complexity of karyotype, 14q aberrations, and TP53 deletions have been found to be poor prognostic indicators. We report an unusual case of SMZL with a complex karyotype including 17p deletion, primarily refractory to 2 chemoimmunotherapy regimens, that responded well to treatment with phosphatidylinositol-3-kinase delta (PI3Kδ) inhibitors idelalisib and rituximab...
March 2018: Journal of the National Comprehensive Cancer Network: JNCCN
Mohammed Benadallah, Oualid Talhi, Fatiha Nouali, Nourredine Choukchou-Braham, Khaldoun Bachari, Artur M S Silva
The present review deals with the progress in medicinal chemistry of spirocyclic compounds, a wider class of natural and synthetic organic molecules, defined as a hybrid of two molecular entities covalently linked via a unique tetrahedral carbon. This spiro central carbon confers to the molecules a tridimensional structurally oriented framework, which is found in many medicinally relevant compounds, a well-known example is the anti-hypertensive spironolactone. Various bioactive natural products possess the privileged spiro linkage and different chemo-types thereof become synthetically accessible since the 20th century...
March 9, 2018: Current Medicinal Chemistry
Madeliene Parrott, Simon Rule
Mantle cell lymphoma (MCL) is a rare but often aggressive B-cell non-Hodgkin lymphoma (NHL). Initial therapy can achieve high response rates but invariably patients relapse and die from their disease. Incorporating a maintenance phase into the treatment strategy may prolong remission duration and ultimately prolong survival. Areas covered: The current literature incorporating a maintenance phase into treatment strategies for newly diagnosed and pre-treated MCL patients has been summarized. A literature search was performed using search terms "mantle cell lymphoma", "indolent NHL", "maintenance", "interferon", "rituximab", "lenalidomide", "bortezomib" and "ibrutinib"...
March 9, 2018: Expert Review of Hematology
Xiaowen Ge, Na Zhu, Jiamei Yao, Haiying Zeng, Jieakesu Su, Zhengzeng Jiang, Yuan Ji, Yunshan Tan, Yingyong Hou
RATIONALE: Primary nodal CD4-positive T-cell lymophoproliferative disorder with a relatively indolent process is a rare kind of lymphoproliferative disease. Here we report the first case of a 49 year-old man developed indolent nodal CD4-positive T-cell lymophoproliferative disorder. To our knowledge, based on a careful search of PubMed, it is the first case of primary nodal CD4-positive T-cell lymophoproliferative disorder. PATIENT CONCERNS: A 49-year-old Chinese man presented to our hospital with fever, enlargement of multiple superficial lymphonodes more than 14 years and splenomegaly...
March 2018: Medicine (Baltimore)
Tammy T Hshieh, Wooram F Jung, Laura J Grande, Jiaying Chen, Richard M Stone, Robert J Soiffer, Jane A Driver, Gregory A Abel
Importance: As the population ages, cognitive impairment has promised to become increasingly common among patients with cancer. Little is known about how specific domains of cognitive impairment may be associated with survival among older patients with hematologic cancers. Objective: To determine the prevalence of domain-specific cognitive impairment and its association with overall survival among older patients with blood cancer. Design, Setting, and Participants: This prospective observational cohort study included all patients 75 years and older who presented for initial consultation in the leukemia, myeloma, or lymphoma clinics of a large tertiary hospital in Boston, Massachusetts, from February 1, 2015, to March 31, 2017...
March 1, 2018: JAMA Oncology
Sam Vander Meeren, Bert Heyrman, Wim Renmans, Marleen Bakkus, Brigitte Maes, Hendrik De Raeve, Rik Schots, Kristin Jochmans
High-count monoclonal B cell lymphocytosis (MBL) with a chronic lymphocytic leukemia (CLL) phenotype is a well-known entity, featuring 1-4% annual risk of progression towards CLL requiring treatment. Lymphoma-like MBL (L-MBL), on the other hand, remains poorly defined and data regarding outcome are lacking. We retrospectively evaluated 33 L-MBL cases within our hospital population and compared them to 95 subjects with CLL-like MBL (C-MBL). Diagnoses of L-MBL were based on asymptomatic B cell clones with Matutes score < 3, B cells < 5...
February 28, 2018: Annals of Hematology
Katja Maurus, Silke Appenzeller, Sabine Roth, Jochen Kuper, Simone Rost, Svenja Meierjohann, Panagiota Arampatzi, Matthias Goebeler, Andreas Rosenwald, Eva Geissinger, Marion Wobser
Primary cutaneous marginal zone lymphoma (PCMZL) represents an indolent subtype of Non-Hodgkin lymphomas being clinically characterized by slowly growing tumors of the skin, but a very low propensity for systemic dissemination. Up to now, the underlying genetic basis of PCMZL has not been comprehensively elucidated. With the aim to gain deeper insight into the molecular pathogenesis of PCMZL, we performed hybridization-based panel sequencing of 38 cases of well-characterized PCMZL. In 32 out of 38 cases we identified genetic alterations within 39 selected target genes...
February 23, 2018: Journal of Investigative Dermatology
James Isom, May R Arroyo, Deepa Reddy, Priti Joshi-Guske, Samer Z Al-Quran, Ying Li, Robert W Allan
NK-cell enteropathy is a recently described clinically indolent condition characterized by atypical NK-cell infiltrates in the gastrointestinal mucosa that mimics malignant lymphoma. We report the case of a 69-year-old female who clinically had long-standing abdominal pain and recurrent mucosal ulcerations associated with atypical NK-cell infiltrates. The clinical, morphologic, and immunophenotypic findings in this case were diagnostic of NK-cell enteropathy. Review of the patient's prior biopsies demonstrated that this persisted without clinical progression for ten years-confirming the clinical indolent course...
February 23, 2018: Histopathology
A Kreuter, I Pantelaki, A-L Michalowitz, U Wieland, L Cerroni, F Oellig, C Tigges
CD30-positive primary cutaneous anaplastic large cell lymphoma (C-ALCL) is an indolent type of cutaneous lymphoma with favourable clinical prognosis. Pseudocarcinomatous hyperplasia (PCH) is a rare benign epithelial condition that can resemble invasive squamous cell carcinoma both clinically and histopathologically. PCH predominantly occurs in CD30-positive lymphoproliferative disorders. We report a 75-year-old woman with PCH in a multifocal C-ALCL located on the scalp and right retroauricular area, which rapidly responded to treatment with psoralen ultraviolet A photochemotherapy...
February 23, 2018: Clinical and Experimental Dermatology
Riccardo Bomben, Simone Ferrero, Tiziana D'Agaro, Michele Dal Bo, Alessandro Re, Andrea Evangelista, Angelo Michele Carella, Alberto Zamò, Umberto Vitolo, Paola Omedè, Chiara Rusconi, Luca Arcaini, Luigi Rigacci, Stefano Luminari, Andrea Piccin, Delong Liu, Adrien Wiestner, Gianluca Gaidano, Sergio Cortelazzo, Marco Ladetto, Valter Gattei
Mantle cell lymphoma patients have variable clinical courses, ranging from indolent cases not requiring immediate treatment to aggressive, rapidly progressing diseases. Thus, diagnostic tools capable to stratify patients according to their risk of relapse and death are warranted. The study included 83 samples from the Fondazione Italiana Linfomi MCL-0208 clinical trial. Through gene expression profiling and quantitative real-time PCR we analyzed 46 peripheral blood and 43 formalin-fixed paraffin-embedded lymph node samples...
February 22, 2018: Haematologica
Caroline Stokke, Johan Blakkisrud, Ayca Løndalen, Jostein Dahle, Anne C T Martinsen, Harald Holte, Arne Kolstad
PURPOSE: 177 Lu-lilotomab satetraxetan is a novel anti-CD37 antibody radionuclide conjugate for the treatment of non-Hodgkin lymphoma (NHL). Four arms with different combinations of pre-dosing and pre-treatment have been investigated in a first-in-human phase 1/2a study for relapsed CD37+ indolent NHL. The aim of this work was to determine the tumor and normal tissue absorbed doses for all four arms, and investigate possible variations in the ratios of tumor to organs-at-risk absorbed doses...
February 22, 2018: European Journal of Nuclear Medicine and Molecular Imaging
Cesare Mazzaro, Luigino Dal Maso, Luca Quartuccio, Michela Ghersetti, Marco Lenzi, Endri Mauro, Milena Bond, Pietro Casarin, Valter Gattei, Ivo Maria Crosato, Salvatore De Vita, Gabriele Pozzato
OBJECTIVES: To investigate the long-term effects and safety of new direct anti-viral agents (DAAs) in patients with hepatitis C virus (HCV)-related mixed cryoglobulinaemia (MC) without renal involvement. METHODS: The study enrolled 22 consecutive patients, 19 received sofosbuvir-based regimen and three patients received other DAAs, individually tailored according to latest guidelines. As of December 2016, the median length of follow-up was 17 months (range 13-21)...
February 13, 2018: Clinical and Experimental Rheumatology
Rory McCulloch, Simon Rule
The last 20 years has seen considerable advances made in the management of younger patients with mantle cell lymphoma. The use of high dose cytarabine and rituximab in induction therapy, usually followed by autologous stem cell transplant consolidation, has become established practice and the median overall survival now exceeds 10 years. However, this high intensity upfront approach is not necessarily appropriate for all newly diagnosed patients. A minority exhibit disease that behaves in an indolent fashion with no proven benefit from early intervention, and at the opposite end of the spectrum a high-risk group exists who do poorly with conventional treatment...
March 2018: Best Practice & Research. Clinical Haematology
Christina Kalpadakis, Gerassimos A Pangalis, Maria K Angelopoulou, Sotirios Sachanas, Theodoros P Vassilakopoulos
SMZL is a relatively rare low grade B-cell lymphoma, characterized usually by an indolent clinical behavior. Since there is no prospective randomized trials to establish the best treatment approach, decision on therapeutic management should be based on the available retrospective series. Based on these data, rituximab and splenectomy appear to be the most effective. Splenectomy represented the standard treatment modality until early 2000s. More than 90% of the patients present quick amelioration of splenomegaly related symptoms along with improvement of cytopenias related to hypersplenism...
March 2018: Best Practice & Research. Clinical Haematology
Irene Defrancesco, Luca Arcaini
Extranodal marginal zone B-cell lymphomas (EMZLs) of the mucosa-associated lymphoid tissue (MALT) are indolent lymphomas which can present at any extranodal site. The most frequent localizations (other than stomach) are ocular adnexa, salivary gland, skin, lung and thyroid. Chronic inflammation and antigenic stimulation are a potential risk for the development of MALT lymphomas. While Helicobacter Pylori (HP) is known to be associated with gastric MALT lymphoma and antibiotic therapy is effective in the setting of HP-positive, other microorganisms (such as Chlamydophila Psittaci, Campylobacter Jejiuni, Borrelia Burgdoferi) have been implicated in the pathogenesis of non-gastric MALT lymphomas...
March 2018: Best Practice & Research. Clinical Haematology
Jayadev Manikkam Umakanthan, Mathew A Lunning
Follicular lymphoma is the most common indolent non-Hodgkin lymphoma. Advanced stage disease is common at diagnosis. The timing of treatment for follicular lymphoma is best approached by considering the combination of presence or absence of symptoms along with estimation of tumor burden. Upfront treatment strategies should take into initial presentation variables, pace of disease progression and goals of care after discussion with the patient. Treatment approaches remain diverse and patient discernment is paramount...
March 2018: Best Practice & Research. Clinical Haematology
Tracy Lackraj, Rashmi Goswami, Robert Kridel
Follicular lymphoma (FL) is presented as a germinal centre B cell lymphoma that is characterized by an indolent clinical course, but remains - paradoxically - largely incurable to date. The last years have seen significant progress in our understanding of FL lymphomagenesis, which is a multi-step process beginning in the bone marrow with the hallmark t(14;18)(q32;q21) translocation. The pathobiology of FL is complex and combines broad somatic changes at the level of both the genome and the epigenome, the latter evidenced by highly recurrent mutations in chromatin-modifying genes such as KMT2D and CREBBP...
March 2018: Best Practice & Research. Clinical Haematology
Brad Kahl
No abstract text is available yet for this article.
March 2018: Best Practice & Research. Clinical Haematology
Alessandro Pileri, Claudio Agostinelli, Elena Sabattini, Michela Tabanelli, Valeria Gaspari, Miriam Leuzzi, Annalisa Patrizi, Francesco Savoia
Primary cutaneous small/medium-sized pleomorphic T-cell lymphoproliferative disorder (PCSM-TCLPD) is a rare and indolent primary cutaneous T-cell lymphoma variant [1], usually presenting as a solitary nodule or plaque in the head and neck [2] or rarely as multiple lesions in anatomic areas apart from head and neck [2]. Histology reveals the presence of a dense dermal infiltrate of CD4+, CD8-, small/medium pleomorphic cells [2] intermingled with reactive cells. Surgical or radiotherapic approach are the preferred option, while oral doxycycline has been suggested in cases with multiple lesions [2-3]...
February 14, 2018: Journal of the European Academy of Dermatology and Venereology: JEADV
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