Publications by authors named "Adele Santoni"

8 Publications

  • Page 1 of 1

First reported case of secondary mixed phenotype acute leukemia after multiple myeloma.

Am J Blood Res 2021 20;11(1):123-131. Epub 2021 Feb 20.

Hematology Unit, University of Siena, Azienda Ospedaliera Universitaria Senese Siena, Italy.

In recent years the outcome of patients with multiple myeloma (MM) has significantly improved, due to new drugs. However, some agents, i.e. the alkylating drug melphalan, can be associated with an increased incidence of secondary malignancies. Myelodysplastic syndromes and acute myeloid leukemia are reported in the literature, and rarely acute lymphoblastic leukemia. Here we describe a unique case of a 56-years old female patient affected by MM since 2015 in complete remission after autologous stem cell transplant and in lenalidomide maintenance, who developed 2 years later mixed phenotype acute leukemia (MPAL). The patient, refractory to both lymphoblastic and myeloid acute leukemia regimens, achieved complete remission with bi-specific anti-CD19/anti-CD3 monoclonal antibody blinatumomab and with hypomethylating agent azacytidine plus the BCL-2 inhibitor venetoclax. She then underwent hematopoietic stem cell transplantation from HLA-identical sibling donor and she is still in complete remission after 9 months. To the best of our knowledge, there are no cases in the literature describing MPAL after autologous transplant for MM. Our patient was treated with blinatumomab and venetoclax and achieved complete remission 9 months from allogeneic transplant. The mechanism underlying the development of MPAL is not completely understood and therapies are still lacking. In this context the combination of blinatumomab, azacytidine and venetoclax successfully used in this patient may provide food for thought for further studies in this rare setting of patients.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8010609PMC
February 2021

Fludarabine, High-Dose Cytarabine and Idarubicin-Based Induction May Overcome the Negative Prognostic Impact of -ITD in Mutated AML, Irrespectively of -ITD Allelic Burden.

Cancers (Basel) 2020 Dec 24;13(1). Epub 2020 Dec 24.

Clinic of Hematology, Department of Internal Medicine (DiMI), University of Genoa, 16132 Genova, Italy.

The mutations of and -ITD represent the most frequent genetic aberration in acute myeloid leukemia. Indeed, the presence of an mutation reduces the negative prognostic impact of -ITD in patients treated with conventional "3+7" induction. However, little information is available on their prognostic role with intensified regimens. Here, we investigated the efficacy of a fludarabine, high-dose cytarabine and idarubicin induction (FLAI) in 149 consecutive fit AML patients (median age 52) carrying the and/or -ITD mutation, treated from 2008 to 2018. One-hundred-and-twenty-nine patients achieved CR (86.6%). After a median follow up of 68 months, 3-year overall survival was 58.6%. Multivariate analysis disclosed that both mut ( < 0.05) and ELN 2017 risk score ( < 0.05) were significant predictors of survival. -mutated patients had a favorable outcome, with no significant differences between patients with or without concomitant -ITD ( = 0.372), irrespective of -ITD allelic burden. Moreover, in landmark analysis, performing allogeneic transplantation (HSCT) in first CR proved to be beneficial only in ELN 2017 high-risk patients. Our data indicate that FLAI exerts a strong anti-leukemic effect in younger AML patients with mut and question the role of HSCT in 1st CR in mut patients with concomitant -ITD.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3390/cancers13010034DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7796342PMC
December 2020

Efficacy and safety of eltrombopag during conception and first trimester of pregnancy in a case of refractory severe immune thrombocytopenia.

Blood Coagul Fibrinolysis 2020 Sep;31(6):416-418

Hematology Unit, Azienda Ospedaliera Universitaria Senese e Università di Siena, Siena, Italy.

: Immune thrombocytopenia (ITP) is a relatively frequent cause of thrombocytopenia during pregnancy. Thrombopoietin receptor agonists (TPO-RAs) are the most recent drugs approved for second-line treatment of ITP. Limited data are available about their use in pregnancy with only a few published cases; yet no data exist about their effect when administered only during conception and first trimester of gestation. We describe the case of a woman with refractory ITP who took eltrombopag during conception and first trimester of pregnancy. No fetal or maternal complications were reported. Moreover, the patient remained in complete response after delivery despite therapy discontinuation. The analysis of this case and the revision of the available literature suggest that the use of TPO-RAs, thanks to their short time to response, may be effective and feasible during the first trimester of pregnancy, even if not yet recommended by current guidelines.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1097/MBC.0000000000000936DOI Listing
September 2020

Translation, cultural adaptation and validation of the Italian version of the Brief Index of Lupus Damage: the BILDit.

Lupus 2020 Sep 13;29(10):1198-1205. Epub 2020 Jul 13.

Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.

Objectives: The Brief Index of Lupus Damage (BILD) is an instrument of self-evaluation of organ damage for systemic lupus erythematosus (SLE) patients. The objectives of this study were the translation, cultural adaptation and validation of the Italian version of the BILD (BILDit).

Methods: The process of translation and cultural adaptation followed published guidelines. The BILDit was pretested in a pilot study with 30 SLE patients in order to evaluate acceptability, reliability, comprehension and feasibility, and then validated in consecutive SLE patients attending our clinic.

Results: A total of 167 SLE patients were enrolled. In the pilot study, the BILDit demonstrated good acceptability, feasibility and comprehensibility and a very high degree of reliability (Cronbach's α = 1). In the validation cohort, the BILDit showed a significant positive correlation with the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI; ρ = 0.69;  < 0.001). Analysing the item-by-item correlation between the BILDit and the SDI, a good correlation ( < 0.001) was found for 73.1% of the items. In the multivariate analysis, the BILDit showed a significant positive correlation with age and disease duration ( < 0.01).

Conclusions: The BILDit seems to be an acceptable and reliable instrument for patient self-evaluation of disease damage, with a good correlation with the SDI. It can be considered as a screening tool for the evaluation of organ damage starting from the patient's perceptive.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1177/0961203320940012DOI Listing
September 2020

Coexistence of Serum Monoclonal Gammopathy of Uncertain Significance and Hodgkin Lymphoma.

Indian J Hematol Blood Transfus 2020 Apr 12;36(2):390-392. Epub 2019 Dec 12.

1Hematology Unit, University Hospital, Azienda Ospedaliera Universitaria Senese, Viale Bracci, 16, 53100 Siena, Italy.

An uncommon association between multiple myeloma and Hodgkin lymphoma (HL) was observed in some case reports, while an association with monoclonal gammopathy of uncertain significance (MGUS) is exceedingly rare. We have diagnosed 110 HL cases from 2008 to 2018; here we report 4 HL cases associated with MGUS. MGUS was diagnosed before HL (1 case), together with HL (1 case) or after HL (2 cases). M-component was IgG/k (3 cases) and IgG/k and IgG/λ (1 case), MGUS was not influenced by HL treatment (2 cases), raised after therapy (1 case), while in the last case MGUS appeared after ASCT while HL was in complete remission. We suggest to further study a possible link between MGUS and HL and to perform serum immunofixation if protein electrophoresis shows a suspected discrete band. The comprehension of pathophysiology of this association and a possible role of cytokines such as IL-6 and antilymphoma therapies such as nivolumab or ASCT to MGUS development could represent an interesting research field that requires further investigations.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s12288-019-01242-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7229058PMC
April 2020

Impact of fatigue on health-related quality of life and illness perception in a monocentric cohort of patients with systemic lupus erythematosus.

RMD Open 2020 02;6(1)

Department of Clinical and Experimental Medicine, Rheumatology Unit, University of Pisa, Pisa, Italy

Background: Fatigue is a very common and debilitating symptom in patients with systemic lupus erythematosus (SLE), even among those with a mild or inactive disease. The objective of this study is to define fatigue determinants and describe the impact of fatigue on health-related quality of life (HRQoL) and illness perception in a monocentric cohort of patients with SLE.

Methods: This is a cross-sectional study. Adult patients with SLE were included. For each patient, demographics, medications, comorbidities, organ damage (Systemic Lupus International Collaborating Clinics Damage Index), active disease manifestations and Systemic Lupus Disease Activity Index scores were collected. It was evaluated if each patient met the definitions of remission and low disease activity. At enrolment, each patient completed the Short Form-36 (SF-36), Functional Assessment Chronic Illness Therapy-Fatigue (FACIT-F), Lupus Impact Tracker (LIT), Systemic Lupus Activity Questionnaire (SLAQ) and Brief Index of Lupus Damage (BILD). The FACIT-F questionnaire was also administered to a group of healthy controls.

Results: 223 patients were included (mean age 44.9±13.2 years, median disease duration 13 years). 18.2% had an active disease, 43.5% met the definition of remission on treatment, and 11.8% had a concomitant fibromyalgia. The median FACIT-F score of our cohort was significantly lower compared with that of healthy controls (40 vs 47; p<0.001). FACIT-F scores were irrespective of age, disease duration, disease activity and damage. FACIT-F score was significantly lower in patients with fibromyalgia (p<0.01). FACIT-F scores demonstrated a significant correlation with all other patient-reported outcomes: SF-36 (r=0.53-0.77), LIT (r=-0.78), SLAQ (r=-0.72) and BILD (r=-0.28).

Conclusions: Fatigue in patients with SLE has a strong negative impact on HRQoL and patient perception of the disease burden. Fatigue seems irrespective of disease activity but significantly influenced by the presence of fibromyalgia.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1136/rmdopen-2019-001133DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7046978PMC
February 2020

Hodgkin lymphoma in the elderly: new perspectives and a 10-year monocenter real-life experience.

Hematol Oncol 2019 Oct 11;37(4):493-495. Epub 2019 Sep 11.

Unit of Hematology, Azienda Ospedaliera Universitaria Senese & University of Siena, Siena, Italy.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1002/hon.2675DOI Listing
October 2019