16 research outputs found

    Association of 3q21q26 syndrome with different RPN1/EVI1 fusion transcripts

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    Patients with acute myeloblastic leukemia (AML) with features of myelodysplastic syndrome and abnormalities of megakaryocytopoiesis often have cytogenetic aberrations of 3q21 and 3q26 bands involving the paracentric inversion [inv(3) (q21q26)] or a reciprocal translocation [t(3;3) (q21;q26)]. These abnormalities frequently cause inappropriate expression of the EVI1 gene located at 3q26. Other genes that have been implicated at the rearrangement breakpoint are GR6 and RPN1 (both on 3q21). The aim of this study was to investigate the expression of the EVI1 fusion genes in AML patients with 3q21q26 syndrome

    Nutritional intervention with TGF-beta enriched food for special medical purposes (TGF-FSMP) is associated with a reduction of malnutrition, acute GVHD, pneumonia and may improve overall survival in patients undergoing allogeneic hematopoietic stem transplantation

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    : Malnutrition in allogeneic stem cell transplant (allo-SCT) is associated with poor outcomes. Supplementation with Foods for Special Medical Purposes may be a valid alternative to enteral nutrition or total parental nutrition to reduce malnutrition in allo-SCT. In this study, 133 patients consecutively allo-transplanted were assessed for nutritional status by Patient- Generated Subjective Global Assessment (PG-SGA) and supplemented with TGF-beta enriched Food for Special Medical Purposes (TGF-FSMP). PG-SGA, gold standard for nutritional assessment in oncologic patients, was assessed at admission and on day 0, +7, +14, +21, and + 28 from transplant and categorized as follows: A = good nutritional status; B = moderate malnutrition; C = severe malnutrition. TGF-FSMP (Modulen-IBD) is currently used in Inflammatory Bowel Diseases (IBD) as primary nutritional support and in this study the dose was calculated according to BMI and total daily energy expenditure (TDEE). The patients assuming ≥50% of the prescribed TGF-FSMP dose were classified in Group A; the patients who received < 50% were included in Group B per protocol. The primary endpoint of the study was the assessment of the malnourished patients in Group A and B at day+28 after transplantation, according to the criteria of PG-SGA C categorization. At day +28 after transplant: i) patients in Group A were significantly less severely malnourished than patients in the Group B (21/76,28% vs 42/53, 79% respectively, OR 2.86 - CI 1.94-4.23 -, p = 0.000); ii) the incidence of severe (MAGIC II-IV) aGVHD and of any grade gastrointestinal (GI) aGVHD was higher in Group B than in Group A, (43% vs 21% p = 0.003) and (34.5% vs 9.2% p = 0.001); iii) Pneumonia was more frequent in the malnourished patients of Group B than in well/moderate nourished patients of Group A (52.7% vs 27.6% p = 0.002). In group A parenteral nutrition was avoided more frequently than in group B (67.5% vs 33.3% p = 0.000) and a median hospital stay of 27 days in comparison to 32 was reported (p = 0.006). The estimated median overall survival (OS) of the population was 33 months in Group A and 25.1 months in group B (p = 0.03). By multivariate and ANN analysis, TGF-FSMP TR < 50% assumption was significantly correlated with malnutrition, severe and GI aGVHD, pneumonia and reduced OS

    Instillazione diretta intranasale di amfotericina B liposomiale nella terapia delle micosi nasali.

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    Le infezioni fungine viscerali sono una severa complicanza durante la chemioterapia nei pazienti con leucemia acuta mieloide (LAM) (1). In particolare, le Aspergillus spp. e le Candida species sono spesso responsabili di gravi infezioni in questo tipo di pazienti, durante le fasi di agranulocitosi. Il polmone ed i seni nasali sono le sedi più frequentemente interessate da questi patogeni. Entrambi questi tipi di infezione possono essere letali, essendo possibili eventi terminali drammatici l’interessamento dei vasi bronchiali principali (con conseguente emottisi massiva) e l’infiltrazione del sistema nervoso centrale attraverso la lamina cribrosa dell’osso etmoide. Anche l’interessamento epato-splenico è un evento non raro. Il recupero granulocitario ed un tempestivo trattamento specifico per via sistemica sono necessari per controllare l’infezione.Al momento, l’amfotericina-B deossicolato per e.v. è il farmaco più comunemente impiegato per il trattamento di queste forme; peraltro, la nefrotossicità e le reazioni infusionali sono effetti indesiderati molto comuni (2). La formulazione liposomiale dell’amfotericina- B (AmBisome) (GILEAD SCIENCE) si è rivelata particolarmente efficace e con un favorevole profilo di tossicità e viene pertanto comunemente utilizzata per i pazienti che risultino intolleranti alla formulazione convenzionale (3). Questi due farmaci possono essere impiegati anche in forma di aerosol, al fine di aumentare la concentrazione a livello polmonare riducendo la tossicità sistemica (4, 5). Inoltre, sono stati riportati successi con l’instillazione diretta di agenti antifungini in aspergillomi polmonari (6). In casi selezionati, per aspergillomi isolati,può essere proposta una terapia chirurgica.Infine, in caso di micosi invasiva, possono essere utilizzate anche trasfusioni di granulociti (7).Alcuni nuovi farmaci, sviluppati recentemente, hanno mostrato risultati promettenti, ma, allo stato attuale, non sono ancora comunemente adottati. Noi abbiamo utilizzato con successo AmBisome, e. v. e per la prima volta anche per diretta instillazione intranasale, in due pazienti con LAM e micosi dei seni nasali. In un caso, è stata anche associata la trasfusione di granulociti

    CT-290: Clinical Frailty Scale as a Novel Tool to Evaluate Patients’ Eligibility for Allogeneic Stem Cell Transplant: A Single-Center Experience on 234 Patients >50 Years Old

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    Context Clinical frailty scale (CFS) is a scale ranging from 1 (very fit) to 9 (terminally ill) for increasing degrees of frailty extensively used in several geriatric contexts. Currently, no study on CFS is available in an allo-HCT setting. Objective To evaluate the prognostic value of CFS on OS and NRM in allo-HCT. Patients and Methods Overall, 234 consecutive patients aged >50 y were transplanted at our center from 2006 to 2020. Median follow-up: 4.03 y (95%CI: 3.54–5.90). CFS was retrospectively calculated by an external physician blind to transplant outcome. Results Cohort characteristics were the following: median age 59 y (range: 50–73), males 147 (63%), AML (44.4%). DRI was high/very-high in 36.8% of cases. Matched related donor was used in 41.5%, unrelated in 46.2%, alternative in 12.3% of cases. Overall, 170 patients (72.6%) received a reduced-intensity conditioning regimen. For the evaluation of patients’ fitness at transplant, the following scores were applied: Karnofsky performance status (≥90 in 91.5%), HCT-CI (≥3 in 43.2%), FIL score (unfit/frail in 6.8%) and CFS (very fit [score 1] in 6.8%, fit [score 2] in 51.3%; managing well [score 3] in 29.9%, and frail [>3] in 12.0%). An increasing CFS score was associated with a higher proportion of FIL frailty and a lower Karnofsky performance status. No significant differences were observed in terms of comorbidities. At last follow-up, 149 (63.7%) patients had died (NRM 41.6%, relapse 58.4%). CFS was strongly associated with OS (2-y-OS of 85.6%, 63.7%, 25.8%, and 7.1% for patients with score 1, 2, 3, and >3, respectively; p<0.0001) and NRM (2-y-NRM of 0%, 15.4%, 33.7%, and 39.2%; p=0.0003). By multivariate analysis, CFS had independent negative prognostic value on OS (HR: 1.87, 95%CI: 1.58–2.22, p<0.001) and NRM (HR: 1.73, 95%CI: 1.30–2.32, p<0.001). As evaluated by the likelihood ratio test and C-statistics, CFS showed a strong predictive value (65.47 and 30.75; 0.695 [SE 0.019] and 0.708 [SE 0.031], for OS and NRM, respectively). Conclusions CFS appears a simple and highly effective tool for transplant outcome prediction among oncohematological patients aged >50 y. These results might suggest the use of this score for improving patient selection
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