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Carmelo Gurnari, Paola Panetta, Emiliano Fabiani, Anna Maria Nardone, Diana Postorivo, Giulia Falconi, Luca Franceschini, Manuela Rizzo, Vito Mario Rapisarda, Eleonora De Bellis, Francesco Lo-Coco, Maria Teresa Voso
The World Health Organization classifies atypical chronic myeloid leukemia (aCML) as a myeloproliferative/myelodisplastic hematological disorder. The primary manifestations are leukocytosis with disgranulopoiesis, absence of basophilia and/or monocytosis, splenomegaly and absence of Philadelphia chromosome or BCR/ABL fusion. Overall 50-65% of patients demonstrate karyotypic abnormalities, although no specific cytogenetic alterations have been associated with this disease. X chromosome alterations have been rarely reported in myeloid malignancies...
March 2018: Molecular and Clinical Oncology
Victor B Pastor, Sushree S Sahoo, Jessica Boklan, Georg C Schwabe, Ebru Saribeyoglu, Brigitte Strahm, Dirk Lebrecht, Matthias Voss, Yenan T Bryceson, Miriam Erlacher, Gerhard Ehninger, Marena Niewisch, Brigitte Schlegelberger, Irith Baumann, John C Achermann, Akiko Shimamura, Jochen Hochrein, Ulf Tedgård, Lars Nilsson, Henrik Hasle, Melanie Boerries, Hauke Busch, Charlotte M Niemeyer, Marcin W Wlodarski
Familial myelodysplastic syndromes arise from haploinsufficiency of genes involved in hematopoiesis and are primarily associated with early-onset disease. Here we describe a familial syndrome in seven patients from four unrelated pedigrees presenting with myelodysplastic syndrome and loss of chromosome 7/7q. Their median age at diagnosis was 2.1 years (range, 1-42). All patients presented with thrombocytopenia with or without additional cytopenias and a hypocellular marrow without an increase of blasts. Genomic studies identified constitutional mutations (p...
March 2018: Haematologica
Giacomo Pata, Michele Bartoli, Enrico Damiani, Stefano Solari, Antonella Anastasia, Chiara Pagani, Alessandra Tucci
AIM: Assessment of hematologic improvement, survival and peri-operative morbidity after first-line splenectomy for splenic marginal zone lymphoma (SMZL). METHODS: Forty-three patients undergoing open splenectomy were prospectively analyzed. Perioperative clinical course, overall and progression-free survival (OS-PFS) were evaluated. Risk factors analyzed were gender, age, ASA-grade, ECOG performance status, presence of B-symptoms, body mass index, steroidal treatment, serum albumin concentration, IIL-score, operative time, spleen size and weight...
May 2017: International Journal of Surgery
Andrea Ferrario, Michele Merli, Claudia Basilico, Margherita Maffioli, Francesco Passamonti
The role of interleukin-6 (IL-6) in tumorigenesis and in particular in haematological malignancies is crucial. On the basis of the favourable results obtained in the subset of multicentric Castleman disease (MCD), Siltuximab, a chimeric, human-murine, immunoglobulin (Ig) Gk monoclonal antibody directed against human IL-6 has been evaluated in haematological malignancies such as multiple myeloma, myelodisplastic syndromes and non Hodgkin lymphomas. Areas covered: This review discusses available data related to the role of IL-6 as a therapeutic target, the characteristics of Siltuximab in term pharmacokinetics and pharmacodynamics properties and a detailed analysis of the studies involving haematological malignancies with a peculiar focus on non Hodgkin lymphoma...
March 2017: Expert Opinion on Investigational Drugs
Piera Calamita, Annarita Miluzio, Arianna Russo, Elisa Pesce, Sara Ricciardi, Farhat Khanim, Cristina Cheroni, Roberta Alfieri, Marilena Mancino, Chiara Gorrini, Grazisa Rossetti, Ivana Peluso, Massimiliano Pagani, Diego L Medina, Johanna Rommens, Stefano Biffo
Ribosomopathies are a family of inherited disorders caused by mutations in genes necessary for ribosomal function. Shwachman-Diamond Bodian Syndrome (SDS) is an autosomal recessive disease caused, in most patients, by mutations of the SBDS gene. SBDS is a protein required for the maturation of 60S ribosomes. SDS patients present exocrine pancreatic insufficiency, neutropenia, chronic infections, and skeletal abnormalities. Later in life, patients are prone to myelodisplastic syndrome and acute myeloid leukemia (AML)...
January 2017: PLoS Genetics
Marcio Miguel Andrade-Campos, Paola Liévano, Natalia Espinosa-Lara, Gloria Soro-Alcubierre, José María Grasa-Ulrich, Luis López-Gómez, Teresa Baringo, Pilar Giraldo
BACKGROUND: Non-Hodgkin lymphoma patients have a 25% increased risk of secondary primary neoplasms (SPNs). Regarding the controversy about the increased risk of SPN in patients exposed to radioimmunotherapy (RIT), we have analyzed this issue in a cohort of follicular lymphoma (FL) patients treated with/without RIT. PATIENTS AND METHODS: A retrospective study including all consecutive FL patients diagnosed since 2001 was performed. Demographic, clinical data including the incidence of any kind of neoplasm (excluding basocellular skin carcinoma) were recorded...
December 2016: European Journal of Haematology
Valentina Salsi, Sebastian Fantini, Vincenzo Zappavigna
NUP98 is a recurrent partner gene in translocations causing acute myeloid leukemias and myelodisplastic syndrome. The expression of NUP98 fusion oncoproteins has been shown to induce mitotic spindle defects and chromosome missegregation, which correlate with the capability of NUP98 fusions to cause mitotic checkpoint attenuation. We show that NUP98 oncoproteins physically interact with the APC/C(Cdc20) in the absence of the NUP98 partner protein RAE1, and prevent the binding of the mitotic checkpoint complex to the APC/C(Cdc20)...
September 2016: Cell Cycle
Antonio Gidaro, Giorgio Lambertenghi Deliliers, Paolo Gallipoli, Massimo Arquati, Maddalena Alessandra Wu, Roberto Castelli
Myelodisplastic syndromes (MDS) are heterogeneous myeloid disorders characterized by peripheral cytopenias and increased risk of transformation into acute myelogenous leukemia (AML). MDS are generally suspected in the presence of cytopenia on routine analysis and the evaluation of bone marrow cells morphology and cellularity leads to correct diagnosis of MDS. The incidence of MDS is approximately five cases per 100,000 people per year in the general population, but it increases up to 50 cases per 100,000 people per year after 60 years of age...
September 1, 2016: Clinical Chemistry and Laboratory Medicine: CCLM
Enrique M Ocio, Pilar Herrera, María-Teresa Olave, Nerea Castro, José A Pérez-Simón, Salut Brunet, Albert Oriol, Marta Mateo, Miguel-Ángel Sanz, Javier López, Pau Montesinos, María-Carmen Chillón, María-Isabel Prieto-Conde, María Díez-Campelo, Marcos González, María-Belén Vidriales, María-Victoria Mateos, Jesús F San Miguel
This phase Ib/II trial combined the pan-deacetylase inhibitor panobinostat with chemotherapy followed by panobinostat maintenance in elderly patients with newly diagnosed acute myeloid leukemia. Patients with prior history of myelodysplastic syndrome were excluded and 38 evaluable patients were included in the study (median age: 71 years; range: 65-83). Study patients received an induction with idarubicin (8 mg/m(2) iv days 1-3) plus cytarabine (100 mg/m(2) iv days 1-7) plus panobinostat po at escalating doses (days 8, 10, 12, 15, 17 and 19) that could be repeated in non-responding patients...
October 2015: Haematologica
A Bacigalupo, A Dominietto, A Ghiso, C Di Grazia, T Lamparelli, F Gualandi, S Bregante, M T Van Lint, S Geroldi, S Luchetti, R Grasso, S Pozzi, N Colombo, E Tedone, R Varaldo, A M Raiola
This is a report of 148 patients with hematologic malignancies who received an unmanipulated haploidentical bone marrow transplant (BMT), followed by post-transplant high-dose cyclophosphamide (PT-CY). All patients received a myeloablative conditioning consisting of thiotepa, busulfan, fludarabine (n=92) or TBI, fludarabine (n=56). The median age was 47 years (17-74); 47 patients were in first remission (CR1), 37 in second remission (CR2) and 64 had an active disease; all patients were first grafts. The diagnosis was acute leukemia (n=75), myelodisplastic syndrome (n=24), myelofibrosis (n=16), high-grade lymphoma (n=15) and others (n=18)...
June 2015: Bone Marrow Transplantation
J M Bastida, M Cabrero, O Lopez-Godino, M Lopez-Parra, F Sanchez-Guijo, L Lopez-Corral, L Vazquez, D Caballero, C Del Cañizo
The impact of donor age in patients with acute myeloid leukemia and myelodysplastic syndrome who underwent allogeneic hematopoietic stem cell transplant (HSCT) remains unclear. In the current study, we evaluate 179 consecutive patients who received an HSCT, from January 2000 to January 2013, in our Institution. Most of the HSCT (91%) were HLA-matched. Patient and donor median age were 51 years (18-69) and 47 years (12-75) respectively, and 81 donors (45%) were older than 50 years. The median follow-up was 38 months (range 1-138), Kaplan-Meier estimated 3-year overall survival (OS) was 63% and disease free survival (DFS) was 56%...
August 2015: Leukemia Research
Lucia Tattoli, Michael Tsokos, Julia Sautter, Joannis Anagnostopoulos, Eloisa Maselli, Giuseppe Ingravallo, Mario Delia, Biagio Solarino
In forensic sciences, bone marrow (BM) is an alternative matrix in postmortem toxicology because of its good resistance to autolysis and contaminations. Nevertheless, few studies have been focused on postmortem BM morphological changes after pathological stimuli. We examined 73 BM samples from forensic autopsies; causes of death were both natural and traumatic. BM samples were collected from the sternum by needle aspiration and biopsy; in selected cases, immunohistochemistry was performed. Few autolytic changes were found; BM cellularity decreased with increasing age and postmortem interval...
January 2014: Forensic Science International
Anwar Zeb Jan, Bakhtyar Zahid, Samreen Ahmad, Zahid Gul
OBJECTIVE: To determine the various spectrum of pancytopenia with its frequency on the basis of bone marrow examination in children from 6 months to 14 years. METHODS: A retrospective descriptive study was carried out at Department of Pediatric Rehman Medical Institute Peshawar from January 2006 to December 2012. A total of 205 patient's age between 6 months and 14 years, fulfilling the inclusion and exclusion criteria were included in the study. Complete blood count, peripheral smear, bone marrow examination and Serum vitamin B12 level was done in all the cases...
September 2013: Pakistan Journal of Medical Sciences Quarterly
Sophia Adamia, Patrick M Pilarski, Michal Bar-Natan, Richard M Stone, James D Griffin
Although the imatinib based therapy of chronic myeloid leukemia (CML) represents a triumph of medicine, not all patients with CML benefit from this drug due to the development of resistance and intolerance. The interruption of imatinib treatment is often followed by clinical relapse, suggesting a failure in the killing of residual leukaemic stem cells. There is need to identify alternative selective molecular targets for this disease and develop more effective therapeutic approaches. Alternative pre-mRNA splicing (AS) is an epigenetic process that greatly diversifies the repertoire of the transcriptome...
September 2013: Current Cancer Drug Targets
Otávio Clark, Enéas José de Matos Faleiros
INTRODUCTION: Myelodysplastic syndrome is an incurable and rare hematological disease that affects the production of blood cells. One aim of treatment is to maintain the blood-cell count to near-normal levels. This is mainly achieved with hematopoietic- growth factors and transfusions. Our objective was to determine the cost of supportive treatment/care for patients with low and intermediate I risk myelodysplastic syndrome in respect to private healthcare plans in Brazil. METHOD: We adapted the National Comprehensive Cancer Network treatment guidelines for intermediate risk myelodysplastic syndrome patients to the Brazilian reality, adopting a decision tree to explore treatment combinations...
2011: Revista Brasileira de Hematologia e Hemoterapia
Darko Antic, Ivo Elezovic, Natasa Milic, Nada Suvajdzic, Ana Vidovic, Maja Perunicic, Irena Djunic, Mirjana Mitrovic, Dragica Tomin
Isolated myeloid sarcoma is an extramedullary tumor of immature myeloid cells defined by the absence of leukemia history, myelodisplastic syndrome, or myeloproliferative neoplasma with a negative bone marrow biopsy. Myeloid sarcoma is a very rare condition, and few cases have been reported. We reviewed data of 12 patients with isolated myeloid sarcoma managed at a single center to determine the possible prognostic factors affecting patient survival, such as age, sex, type, localization, and treatment options...
February 2013: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
V André, D Longoni, S Bresolin, C Cappuzzello, E Dander, M Galbiati, C Bugarin, A Di Meglio, E Nicolis, E Maserati, M Serafini, A J Warren, G Te Kronnie, G Cazzaniga, L Sainati, M Cipolli, A Biondi, G D'Amico
Shwachman-Diamond syndrome (SDS) is a rare inherited disorder characterized by bone marrow (BM) dysfunction and exocrine pancreatic insufficiency. SDS patients have an increased risk for myelodisplastic syndrome and acute myeloid leukemia. Mesenchymal stem cells (MSCs) are the key component of the hematopoietic microenvironment and are relevant in inducing genetic mutations leading to leukemia. However, their role in SDS is still unexplored. We demonstrated that morphology, growth kinetics and expression of surface markers of MSCs from SDS patients (SDS-MSCs) were similar to normal MSCs...
October 12, 2012: Blood Cancer Journal
Marco Gunnellini, Lorenzo Falchi
Mantle cell lymphoma (MCL) comprises 3-10% of NHL, with survival times ranging from 3 and 5 years. Indolent lymphomas represent approximately 30% of all NHLs with patient survival largely dependent on validated prognostic scores. High response rates are typically achieved in these patients with current first-line chemoimmunotherapy. However, most patients will eventually relapse and become chemorefractory with poor outcome. Alternative chemoimmunotherapy regimens are often used as salvage strategy and stem cell transplant remains an option for selected patients...
2012: Advances in Hematology
S V Andreeva
Cytogenetic investigation results in bone marrow cells of 70 children and teenagers from 1 to 18 years with myelodysplastic syndromes (MDS) were presented. Between them were 29 girls and 41 boys. According MDS categories were registrated refractory anemia (RA), refractory cytopenia with multilineage dysplasia (RCMD) and refractory anemia with excess blasts (RAEB) (30, 29 and 11 patients, respectively). High spectrum of mosaic clones in bone marrow cells were found: 1) normal and near-tetraploidy 2) abnormal and normal, 3) abnormal, near-tetraploidy and normal, 4) evolution of clonal chromosomal abnormalities and 5) unrelated clones...
July 2011: Likars'ka Sprava
Raffaele Landolfi, Leonardo Di Gennaro
Myeloproliferative neoplasms (MPNs) and myelodisplastic syndromes (MDs) are clonal disorders caused by mutations of myeloid stem cells. Among MPNs, polycythemia vera and essential thrombocythemia are relatively benign disorders in which arterial and venous thromboses represent the main cause of morbidity and mortality. The natural history of MDs is often complicated by both thromboses and haemorrhages, mainly due to platelet quantitative and quantitative anomalies, as well as to treatment complications. In this short review, we focus the attention on the main aspects of thrombophilia in both disorders...
April 2012: Hematology (Amsterdam, Netherlands)
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