Systemic mastocytosis is a rare disease characterized by the uncontrolled clonal proliferation of abnormal mast cells in one or more extracutaneous organs. It is no longer considered a myeloproliferative neoplasia but a distinct subgroup following the review of the classification by WHO in 2016. The average age at diagnosis is 60 years regardless of gender.
View Article and Find Full Text PDFCancer patients have an increased thrombotic risk of arterial and venous thrombosis. Thrombocytopenia, particularly with anticoagulation, exposes the patient to an increased risk of bleeding but does not reduce the risk of recurrent thrombosis. When platelets are < 50 × 109/l, the strategy regarding anticoagulation must be reassessed.
View Article and Find Full Text PDFTreatment results of AML in elderly patients are unsatisfactory. We hypothesized that addition of tosedostat, an aminopeptidase inhibitor, to intensive chemotherapy may improve outcome in this population. After establishing a safe dose in a run-in phase of the study in 22 patients, 231 eligible patients with AML above 65 years of age (median 70, range 66-81) were randomly assigned in this open label randomized Phase II study to receive standard chemotherapy (3+7) with or without tosedostat at the selected daily dose of 120 mg ( = 116), days 1-21.
View Article and Find Full Text PDFAcute Myeloid Leukaemia (AML) is a rare and heterogeneous haematological malignancy with increasing incidence in the elderly. We performed a population-based, observational analysis of AML cases reported to the Cantonal Cancer Registries in Switzerland. Data was aggregated by the National Institute for Epidemiology and Cancer Registration and stratified for the two time periods 2001-2007 and 2008-2013.
View Article and Find Full Text PDFWe performed IGH clonotypic sequence analysis in WM in order to determine whether a preferential IGH gene rearrangement was observed and to assess IGHV mutational status in blood and/or bone marrow samples from 36 WM patients. In addition we investigated the presence of MYD88 L265P somatic mutation. After IGH VDJ locus amplification, monoclonal VDJ rearranged fragments were sequenced and analyzed.
View Article and Find Full Text PDFBackground. Serum free light chains (sFLC), the most commonly detected paraprotein in CLL, were recently proposed as useful tools for the prognostication of CLL patients. Objective.
View Article and Find Full Text PDFObjective: This study aimed to evaluate the short-term psychological impact on family members of intensive care unit (ICU) patients during their stay in the unit.
Methods: Thirty-two first-degree relatives of patients treated in the ICU of two general hospitals were investigated for symptoms of early posttraumatic stress reaction, anxiety, and depression. Patients' and relatives' sociodemographic data and information pertaining to the patients' ICU treatment were collected.
Primary Objective: To investigate the circadian rhythm of serum melatonin in patients with traumatic brain injury (TBI) during Intensive Care Unit (ICU) stay and its relationship with core body temperature fluctuations and measures of severity of their condition.
Methods And Procedures: The pilot study was conducted in the ICU of a general hospital in Athens, Greece. Blood melatonin was determined in eight patients consecutively admitted at the ICU following severe head injury, eight times per day during the first and second day following admission.
The incidence of FLT3 mutations (internal tandem duplication and Asp835) was investigated in bone marrow samples from 97 patients with myelodysplastic syndrome [(MDS); excluding cases with refractory anaemia with excess blasts in transformation] at the time of diagnosis and several time points thereafter. Three patients had FLT3 mutations at presentation. Forty-two patients progressed to acute myeloid leukaemia (AML), including the three patients with FLT3 mutations at MDS diagnosis.
View Article and Find Full Text PDFIn this prospective, non-comparative case series, four patients with severe and highly active adult-onset Still's disease (AOSD), refractory to high doses of corticosteroids (which had been combined with methotrexate in three of them) and methotrexate were treated with infliximab (initial dose 3-5 mg/kg, continuing at intervals depending on the patient's individual disease activity). Resolution of their symptoms, which was evident within few days after the first infusion, and a parallel rapid improvement of the acute inflammatory response indices were observed in all. Concomitant corticosteroid treatment was reduced after the first courses of treatment with infliximab, which was well tolerated, and complete disease remission was sustained during a 5-18-month follow-up period.
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