Bendamustine plus rituximab is an effective first-line treatment in hairy cell leukemia variant: A report of three cases

Hairy cell leukemia variant (HCLv) is a chronic lymphoproliferative disorder classified as a provisional entity in the 2016 WHO Classification of Lymphoid Tumors. HCLv is characterized by unfavorable prognosis, low complete remission rates and limited disease control following classical hairy cell leukemia-based regimens. In this study, we report 3 cases of elderly patients with treatment-naive, TP53 un-mutated HCLv, who were effectively treated with four cycles of bendamustine plus rituximab. The regimen was completed in all the patients with acceptable toxicity. All patients achieved a complete clinical response with no evidence of residual disease at bone marrow biopsy and flow-cytometry examination. After a median follow-up of 19 months, the 3 subjects are still in complete remission. In this work, bendamustine plus rituximab proved to be an effective and feasible first-line treatment strategy for elderly patients with TP53 un-mutated HCLv

Григорій Епік

Епік Григорій Данилович (1901–1937) – український письменник, кіносценарист, перекладач і публіцист

STAT3 mutation impacts biological and clinical features of T-LGL leukemia

STAT3 mutations have been described in 30-40% of T-large granular lymphocyte (T-LGL) leukemia patients, leading to STAT3 pathway activation. Considering the heterogeneity of the disease and the several immunophenotypes that LGL clone may express, the aim of this work was to evaluate whether STAT3 mutations might be associated with a distinctive LGL immunophenotype and/or might be indicative for specific clinical features.Our series of cases included a pilot cohort of 101 T-LGL leukemia patients (68 CD8+/CD4- and 33 CD4+/CD8\ub1) from Padua Hematology Unit (Italy) and a validation cohort of additional 20 patients from Rennes Hematology Unit (France).Our results indicate that i) CD8+ T-LGL leukemia patients with CD16+/CD56- immunophenotype identify a subset of patients characterized by the presence of STAT3 mutations and neutropenia, ii) CD4+/CD8\ub1 T-LGL leukemia are devoid of STAT3 mutations but characterized by STAT5b mutations, and iii) a correlation exists between STAT3 activation and presence of Fas ligand, this molecule resulting highly expressed in CD8+/CD16+/CD56- patients. Experiments with stimulation and inhibition of STAT3 phosphorylation confirmed this relationship. In conclusion, our data show that T-LGL leukemia with specific molecular and phenotypic patterns is associated with discrete clinical features contributing to get insights into molecular bases accounting for the development of Fas ligand-mediated neutropenia

Whole-body low-dose CT recognizes two distinct patterns of lytic lesions in multiple myeloma patients with different disease metabolism at PET/MRI

We evaluated differences in density and 18F-FDG PET/MRI features of lytic bone lesions (LBLs) identified by whole-body low-dose CT (WB-LDCT) in patients affected by newly diagnosed multiple myeloma (MM). In 18 MM patients, 135 unequivocal LBLs identified by WB-LDCT were characterized for inner density (negative or positive Hounsfield unit (HU)), where negative density (HU\u2009<\u20090) characterizes normal yellow marrow whereas positive HU correlates with tissue-like infiltrative pattern. The same LBLs were analyzed by 18F-FDG PET/DWI-MRI, registering DWI signal with ADC and SUV max values. According to HU, 35 lesions had a negative density (-\u200956.94\u2009\ub1\u200931.87 HU) while 100 lesions presented positive density (44.87\u2009\ub1\u200923.89 HU). In seven patients, only positive HU LBLs were demonstrated whereas in eight patients, both positive and negative HU LBLs were detected. Intriguingly, in three patients (16%), only negative HU LBLs were shown. At 18F-FDG PET/DWI-MRI analysis, negative HU LBLs presented low ADC values (360.69\u2009\ub1\u2009154.38\u2009 7\u200910-6 mm2/s) and low SUV max values (1.69\u2009\ub1\u20090.56), consistent with fatty marrow, whereas positive HU LBLs showed an infiltrative pattern, characterized by higher ADC (mean 868.46\u2009\ub1\u2009207.67\u2009 7\u200910-6 mm2/s) and SUV max (mean 5.04\u2009\ub1\u20091.94) values. Surprisingly, histology of negative HU LBLs documented infiltration by neoplastic plasma cells scattered among adipocytes. In conclusion, two different patterns of LBLs were detected by WB-LDCT in MM patients. Both types of lesions were indicative for active disease, although only positive HU LBL were captured by 18F-FDG PET/DWI-MRI imaging, indicating that WB-LDCT adds specific information

Alkaline phosphatase variation during carfilzomib treatment is associated with best response in multiple myeloma patients

The ubiquitin–proteasome pathway regulates bone formation through osteoblast differentiation. We analyzed variation alkaline phosphatase (ALP) during carfilzomib treatment. Data from 38 patients enrolled in the PX‐171‐003 and 29 patients in PX‐171‐004 studies, for patients with relapsed/refractory myeloma, were analyzed. All patients received 20 mg/m 2 of carfilzomib on Days 1, 2, 8, 9, 15, and 16 of a 28‐day cycle. Sixty‐seven patients from ALP data were evaluable. In PX‐171‐003, the ORR (>PR) was 18% and the clinical benefit response (CBR; >MR) was 26%, while in PX‐171‐004, the ORR was 35.5% overall and 57% in bortezomib‐naive patients. ALP increment from baseline was statistically different in patients who achieved ≥VGPR compared with all others on Days 1 ( P  = 0.0049) and 8 ( P  = 0.006) of Cycle 2. In patients achieving a VGPR or better, ALP increased more than 15 units per liter at Cycle 2 Day 1 over baseline. An ALP increase over the same period of time was seen in 26%, 13% and 11% of patients achieving PR, MR, and SD, respectively. This retrospective analysis of patients with relapsed or refractory myeloma treated with single‐agent carfilzomib indicates that early elevation in ALP is associated with subsequent myeloma response.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/86807/1/j.1600-0609.2011.01602.x.pd

Richtlijn Terugvalpreventie Anorexia Nervosa (18+): Een richtlijn voor verpleegkundigen en verzorgenden. Deel 1: Theoretische onderbouwing. Deel 2 Practische handleiding. Werkboek

De richtlijn bestaat uit twee delen: Deel 1: Theoretische onderbouwing met conclusies en aanbevelingen. Gebaseerd op literatuurgegevens en op praktijkervaring van experts. Deel 2: Praktische handleiding voor de hulpverlener. Werkboek. Dit kan digitaal ingevuld worden. Aan de hand van de handleiding en het werkboek wordt een terugvalpreventieplan opgesteld. Deze richtlijn is gericht op terugvalpreventie bij jeugdige patiënten met anorexia nervosa

Richtlijn Terugvalpreventie Anorexia Nervosa (voor jeugdigen): Een richtlijn voor verpleegkundigen en verzorgenden (deel 1 theoretische onderbouwing, deel 2 praktische handleiding, Werkboek)

Deel 1 biedt een theoretische onderbouwing en hiervan afgeleide conclusies en aanbevelingen. Ze zijn gebaseerd op literatuurgegevens en op praktijkervaring van experts. 'Het werken met een signaleringsplan ter preventie van terugval bij jeugdige patiënten met anorexia nervosa'. Deel 2. Hierin worden de procedures aangereikt die van belang zijn om om tot een terugvalpreventieplan te komen. Dit deel bevat tevens het Werkboek Terugvalpreventie, maar kunt u hieronder ook als 'los bestand' downloaden

The Anorexia Relapse Prevention Guidelines in practice: a case report

The purpose of this case report is to illustrate the application of the Anorexia Relapse Prevention Guidelines in nursing practice. In a single case report, the implementation of the intervention was described. A purposive use of the Anorexia Relapse Prevention Guidelines provides insight into the actual process of relapse, which contributes to an early recognition of relapse symptoms and permits early intervention aimed at recovery. Use of the Guidelines will lead to the implementation of well-structured professional procedures which are likely to support the patient's recover