Publications by authors named "Maria Luisa Moleti"

34 Publications

Myeloid Sarcoma: Diagnostic and Treatment Tools from a Monocentric Retrospective Experience.

Acta Haematol 2021 Jul 20:1-5. Epub 2021 Jul 20.

Hematology, Department of Translational and Precision Medicine, "Sapienza" University, Rome, Italy.

Myeloid sarcoma (MS) is a very rare disease in both adults and children. Prognosis is poor in adults; in the pediatric age, the prognostic impact of extramedullary disease is controversial. Systemic therapy represents the mainstay of treatment even in isolated MS, but a comparison between different induction regimens is very limited in the literature. To date, it is still not clear if induction treatment should differ from that of other acute myeloid leukemias and stem cell transplant is considered for consolidation in both leukemic patients and in those with isolated disease. Our study describes a retrospective series of 13 cases of MS (adults and children), diagnosed and treated at our institute over 18 years. We report the results of immunophenotypic, cytogenetic and molecular studies, therapeutic approaches, and outcome, in order to establish the best strategy for patients' workup.
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http://dx.doi.org/10.1159/000517389DOI Listing
July 2021

Autoimmune Hemolitic Anemia in a Boy With Inactive Ulcerative Colitis.

Inflamm Bowel Dis 2021 Apr;27(5):e63-e64

Hematology, Department of Translational and Precision Medicine, Sapienza University, Rome, Italy.

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http://dx.doi.org/10.1093/ibd/izaa363DOI Listing
April 2021

Adolescent and young adult acute lymphoblastic leukemia. Final results of the phase II pediatric-like GIMEMA LAL-1308 trial.

Am J Hematol 2021 03 29;96(3):292-301. Epub 2020 Dec 29.

Hematology, Department of Translational and Precision Medicine, Sapienza University, Rome, Italy.

Adolescents and young adults (AYA) with acute lymphoblastic leukemia (ALL) represent a unique patient population with specific characteristics and needs. Growing evidences suggest that pediatric-inspired approaches improve the outcome in AYA. These results prompted the design of a pediatric AIEOP-BFM ALL 2000-based regimen - the GIMEMA LAL-1308 protocol - for newly diagnosed AYA (range 18-35 years) with Philadelphia negative (Ph-) ALL. The protocol included minimal residual disease (MRD) analysis at two different time-points (TP), that is, at the end of induction IA and consolidation IB, and a modulation in post-consolidation intensity according to MRD. Seventy-six patients were eligible between September 2010 and October 2014. The regimen was well tolerated, with 2.7% induction deaths and no deaths in the post-consolidation phase. The complete response (CR) rate was 92%; the 48-month overall survival (OS) and disease-free survival (DFS) were 60.3% and 60.4%. Both OS and DFS were significantly better in T-ALL than B-ALL. A molecular MRD <10 at TP1 was associated with a significantly better OS and DFS (77% vs 39% and 71.9% vs 34.4%, respectively);similar results were documented at TP2 (OS and DFS 74.5% vs 30.6% and 71.5% vs 25.7%, respectively). The LAL-1308 results were compared to those from similar historic AYA populations undergoing the two previous GIMEMA LAL-2000 and LAL-0904 protocols. Both OS and DFS improved significantly compared to the two previous protocols. These results indicate that this pediatric-inspired and MRD-oriented protocol is feasible and effective for Ph- AYA ALL patients, and underline the prognostic value of MRD determinations at specific TPs.
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http://dx.doi.org/10.1002/ajh.26066DOI Listing
March 2021

Polyostotic Fibrous Dysplasia Mimicking Bone Involvement in Hodgkin Lymphoma: A Pediatric Case and Literature Review.

Acta Haematol 2021 24;144(2):212-217. Epub 2020 Jul 24.

Hematology, Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy,

Bone involvement in Hodgkin lymphoma (HL) is rare. The differential diagnosis between HL bone localization and other malignant or benign skeletal diseases is challenging. We report the case of a girl affected by classic HL, initially staged IVA because of supradiaphragmatic lymph nodes and skeletal involvement. After 6 ABVD (adriamycin, bleomycin, vinblastine, dacarbazine) cycles, positron emission tomography (PET) showed a complete metabolic response of the nodal localizations and a persistent, high metabolic activity of bone lesions. Salvage treatment followed by autologous stem cell transplant was carried out. After the transplant, the bone lesions maintained a high metabolic activity at PET. A targeted bone biopsy led to the diagnosis of a fibrous dysplasia excluding the presence of HL. To our knowledge, the concomitant presence of HL and fibrous dysplasia has not been previously reported. An in-depth evaluation of disease response to frontline treatment with a biopsy of the PET-hypercaptant bone lesions could have avoided overtreatment in this patient.
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http://dx.doi.org/10.1159/000508261DOI Listing
April 2021

A review of current induction strategies and emerging prognostic factors in the management of children and adolescents with acute lymphoblastic leukemia.

Expert Rev Hematol 2020 07 1;13(7):755-769. Epub 2020 Jun 1.

Hematology, Department of Translational and Precision Medicine, 'Sapienza" University of Rome , Rome, Italy.

Introduction: Acute lymphoblastic leukemia is the most frequent hematologic malignancy in children. Almost 95% of children potentially achieve a complete remission after the induction treatment, but over the last years, new insights in the genomic disease profile and in minimal residual disease detection techniques have led to an improvement in the prognostic stratification, identifying selected patients' subgroups with peculiar therapeutic needs.

Areas Covered: According to a comprehensive search of peer-review literature performed in Pubmed, in this review we summarize the recent evidences on the induction treatment strategies comprised in the children acute lymphoblastic leukemia scenario, focusing on the role of key drugs such as corticosteroids and asparaginase and discussing the crucial significance of the genomic characterization at baseline which may drive the proper induction treatment choice.

Expert Opinion: Current induction strategies already produce durable remissions in a significant proportion of standard-risk children with acute lymphoblastic leukemia. A broader knowledge of the biologic features related to acute lymphoblastic leukemia subtypes with worse prognosis, and an optimization of targeted drugs now available, might lead to the achievement of long-term molecular remissions in this setting.
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http://dx.doi.org/10.1080/17474086.2020.1770591DOI Listing
July 2020

Atypical Chronic Myeloid Leukemia in a Patient with Aplastic Anemia.

Acta Haematol 2019 21;142(3):185-186. Epub 2019 May 21.

Hematology, Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy.

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http://dx.doi.org/10.1159/000497137DOI Listing
February 2020

Long-term results with the adapted LMB 96 protocol in children with B-cell non Hodgkin lymphoma treated in Iraq: comparison in two subsequent cohorts of patients.

Leuk Lymphoma 2019 05;60(5):1224-1233

b College of Medicine-Iraq, Department of Pediatrics , Children's Welfare Teaching Hospital-Baghdad , Baghdad , Iraq.

Since 2000, an adapted LMB 96 protocol was implemented at the Children-Welfare-Teaching-Hospital in Baghdad for the treatment of childhood B-cell non-Hodgkin lymphoma. The first experience (2000-2005) demonstrated efficacy and feasibility of this protocol in Iraq. In 2006, further adjustments were made in an attempt to reduce therapy-related toxicities. The outcome of the second cohort of 190 children (2006-2010) and the comparison with the previous study are hereby reported. Out of the 180 treated patients, 120 achieved a complete response; during treatment 51 died and 9 abandoned. The 60-month overall survival (OS) and event-free survival (EFS) were 64.7 and 56.3%, respectively. No differences were observed in the 24-month OS and EFS between the 2000-2005 and 2006-2010 cohorts (66.3% vs. 65.1%; p = .89 and 53.3% vs. 57.3%; p = .28, respectively). Therapeutic group-B in the second cohort showed better outcome, although not significant, compared to the first one (EFS 62.9% vs. 53.8%; p = .088). Therapy-related mortality remained high.
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http://dx.doi.org/10.1080/10428194.2018.1519810DOI Listing
May 2019

Real-Life Management of Children and Adolescents with Chronic Myeloid Leukemia: The Italian Experience.

Acta Haematol 2018 18;140(2):105-111. Epub 2018 Sep 18.

Hematology, Department of Cellular Biotechnologies and Hematology, Policlinico Umberto I, Sapienza University, Rome, Italy.

Background: To date, no data on the adherence to specific guidelines for children with chronic myeloid leukemia (CML) in chronic phase (CP) have been reported.

Methods: Since 2001, guidelines for treatment with imatinib mesylate (IM) and monitoring in patients younger than 18 years with CP-CML have been shared with 9 pediatric referral centers (P centers) and 4 reference centers for adults and children/adolescents (AP centers) in Italy. In this study, the adherence to these guidelines was analyzed.

Results: Thirty-four patients with a median age of 11.4 years and 23 patients with a median age of 11.0 years were managed at 9 P and at 4 AP centers, respectively. Evaluations of bone marrow (BM) and/or peripheral blood (PB) were available for more than 90% of evaluable patients. Cytogenetics and molecular monitoring of PB were more consistently performed in AP centers, whereas molecular analysis of BM was carried out more frequently in P centers. Before 2009, some patients who responded to IM underwent a transplantation, contrary to the guidelines' recommendations.

Conclusions: Our experience shows that having specific guidelines is an important tool for an optimal management of childhood CP-CML, together with exchange of knowledge and proactive discussions within the network.
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http://dx.doi.org/10.1159/000491546DOI Listing
June 2019

FDG PET in response evaluation of bulky masses in paediatric Hodgkin's lymphoma (HL) patients enrolled in the Italian AIEOP-LH2004 trial.

Eur J Nucl Med Mol Imaging 2019 01 15;46(1):97-106. Epub 2018 Sep 15.

Pediatric Onco-hematologic Unit, University Hospital S. Anna, Ferrara, Italy.

Purpose: We present the results of an investigation of the role of FDG PET in response evaluation of bulky masses in paediatric patients with Hodgkin's lymphoma (HL) enrolled in the Italian AIEOP-LH2004 trial.

Methods: We analysed data derived from 703 patients (388 male, 315 female; mean age 13 years) with HL and enrolled in 41 different Italian centres from March 2004 to September 2012, all treated with the AIEOP-LH2004 protocol. The cohort comprised 309 patients with a bulky mass, of whom 263 were evaluated with FDG PET at baseline and after four cycles of chemotherapy. Responses were determined according to combined functional and morphological criteria. Patients were followed up for a mean period of 43 months and for each child we calculated time-to-progression (TTP) and relapse rates considering clinical monitoring, and instrumental and histological data as the reference standard. Statistical analyses were performed for FDG PET and morphological responses with respect to TTP. Multivariate analysis was used to define independent predictive factors.

Results: Overall, response evaluation revealed 238 PET-negative patients (90.5%) and 25 PET-positive patients (9.5%), with a significant difference in TTP between these groups (mean TTP: 32.67 months for negative scans, 23.8 months for positive scans; p < 0.0001, log-rank test). In the same cohort, computed tomography showed a complete response (CR) in 85 patients (32.3%), progressive disease (PD) in 6 patients (2.3%), and a partial response (PR) in 165 patients (62.7%), with a significant difference in TTP between patients with CR and patients with PD (31.1 months and 7.9 months, respectively; p < 0.001, log-rank test). Similarly, there was a significant difference in relapse rates between PET-positive and PET-negative patients (p = 0000). In patients with PR, there was also a significant difference in TTP between PET-positive and PET-negative patients (24.6 months and 34.9 months, respectively; p < 0.0001). In the multivariate analysis with correction for multiple testing, only the PET result was an independent predictive factor in both the entire cohort of patients and the subgroup showing PR on CT (p < 0.01).

Conclusion: After four cycles of chemotherapy, FDG PET response assessment in paediatric HL patients with a bulky mass is a good predictor of TTP and disease outcome. Moreover, in patients with a PR on CT, PET was able to differentiate those with a longer TTP. In paediatric HL patients with a bulky mass and in patients with a PR on CT, response on FDG PET was an independent predictive factor.
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http://dx.doi.org/10.1007/s00259-018-4155-4DOI Listing
January 2019

Second cancer incidence in primary mediastinal B-cell lymphoma treated with methotrexate with leucovorin rescue, doxorubicin, cyclophosphamide, vincristine, prednisone, and bleomycin regimen with or without rituximab and mediastinal radiotherapy: Results from a monoinstitutional cohort analysis of long-term survivors.

Hematol Oncol 2017 Dec 12;35(4):554-560. Epub 2017 Jan 12.

Department of Cellular Biotechnologies and Hematology, Policlinico Umberto I, "Sapienza" University of Rome, Rome, Italy.

Our aim is to assess the incidence of second cancer in long-time surviving primary mediastinal B-cell lymphoma (PMBCL) patients treated with combined radiochemoimmunotherapy (standard methotrexate with leucovorin rescue, doxorubicin, cyclophosphamide, vincristine, prednisone, and bleomycin with rituximab and mediastinal radiation therapy at a dose of 30 to 36 Gy). For this purpose, 92 points were evaluated. After a median overall survival of 137 months (range 76-212), we recorded second cancer in 3 of 80 long-surviving patients (3.75%) with cumulative incidence of 3.47% at 15 years and 11% at 17 years, with a 17-year second cancer-free survival of 82%. We observed 2 papillary thyroid cancers with a standardized incidence ratio (SIR) of 7.97 and an absolute excess risk (AER) of 17. 84 and 1 acute myeloid leukemia (AML) with an SIR of 66.53 and an AER of 10.05. No breast cancer occurred. Although we should take into account the limits of the proposed statistical analysis, combined modality treatment was related to a significant SIR and AER for thyroid cancer and acute myeloid leukemia.
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http://dx.doi.org/10.1002/hon.2377DOI Listing
December 2017

Very late relapse in a patient with acute promyelocytic leukemia (APL) rescued with a chemotherapy-free protocol.

Leuk Lymphoma 2017 04 23;58(4):999-1001. Epub 2016 Sep 23.

a Department of Cellular Biotechnologies and Hematology , 'Sapienza', University , Rome , Italy.

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http://dx.doi.org/10.1080/10428194.2016.1222377DOI Listing
April 2017

Epstein-Barr virus-positive diffuse large B-cell lymphoma in children: a disease reminiscent of Epstein-Barr virus-positive diffuse large B-cell lymphoma of the elderly.

Hum Pathol 2015 May 3;46(5):716-24. Epub 2015 Feb 3.

Department of Clinical and Molecular Medicine, Pathology Unit, Sant'Andrea Hospital, Sapienza University, Rome, Italy.

Pediatric Epstein-Barr virus (EBV)-positive diffuse large B-cell lymphoma (EBV+ DLBCL) is a rare disease in nonimmunocompromised hosts. In a review of 231 cases of malignant lymphoma (87 Hodgkin lymphoma and 144 non-Hodgkin lymphoma) occurring in Iraqi children, 7 cases (5% of NHLs) were classified as EBV+ DLBCL. Six children presented with nodal disease, and 1 presented with extranodal localization (bone). In all cases, the disease was at an advanced clinical stage (III/IV). Evidence of immunodeficiency (Evans syndrome and selective IgA deficiency) was observed in a single case. Two cases were "monomorphic" with immunoblastic histology, and 5 cases were "polymorphic" with histologic aspects reminiscent of nodular lymphocyte-predominant Hodgkin lymphoma (2 cases) and of CD30+ classical Hodgkin lymphoma (3 cases). In all cases, tumor cells were EBV infected (EBER+/LMP-1+), were medium-large B-cells (CD20+/CD79a+/PAX-5+/BOB-1+/OCT-2+) of non-germinal center (non-GC) origin (CD10-/MUM-1+), and had high proliferative activity (50%-70%). Chromosomal translocations involving BCL2, MYC, and IGH genes were not observed. IGH monoclonality could be demonstrated in 3 of 3 investigated cases. Six cases of EBV-negative DLBCL (4% of NHL) were present in the same series. All had monomorphic histology with centroblastic/immunoblastic morphology; 3 cases were of GC type and 3 of non-GC type. Our findings indicate that in Iraq, DLBCLs are 9% of NHLs. Moreover, 2 different types of the disease do exist; the EBV-positive cases, with strong histologic and immunohistochemical resemblance with EBV+ DLBCL of the elderly, and the EBV-negative cases, which are similar to the pediatric DLBCL usually observed in Western populations.
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http://dx.doi.org/10.1016/j.humpath.2015.01.011DOI Listing
May 2015

Enteropathy-associated T-cell lymphoma in childhood: a case report and review of the literature.

Leuk Lymphoma 2015 9;56(9):2743-6. Epub 2015 Feb 9.

a Division of Hematology, Department of Biotechnologies and Hematology , "Sapienza" University of Rome , Rome , Italy.

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http://dx.doi.org/10.3109/10428194.2015.1009059DOI Listing
August 2016

Proven Epstein-Barr encephalitis with negative EBV-DNA load in cerebrospinal fluid after allogeneic hematopoietic stem cell transplantation in a child with acute lymphoblastic leukemia.

Pediatr Transplant 2015 Feb 12;19(1):E19-24. Epub 2014 Nov 12.

Division of Hematology, Department of Cellular Biotechnologies and Hematology, "Sapienza" University, Rome, Italy.

We report a case of EBV encephalitis in a seven-yr-old child with Ph+ ALL. Two months after an allogeneic HSCT from his HLA mismatched mother, the patient showed an altered sensorium, generalized seizures, and a left hemiparesis. Brain MRI demonstrated multiple lesions highly suggestive for viral encephalitis. Blood and CSF PCR analyses were negative for the most common viruses involved in immunocompromised patients including EBV. A cerebral biopsy was performed, which showed intense gliosis and perivascular lymphocytic cuffing. PCR analysis performed on brain tissue was positive only for the EBV genome, while extensive investigations for other viral infections were negative. The patient's neurological symptoms rapidly worsened and he died two months later. This case report suggests that in patients presenting neurological and radiological signs of encephalitis after an HSCT, an EBV involvement should be considered, even in the absence of CSF and blood PCR virus detection.
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http://dx.doi.org/10.1111/petr.12386DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7167730PMC
February 2015

Thrombocythemia and polycythemia in patients younger than 20 years at diagnosis: clinical and biologic features, treatment, and long-term outcome.

Blood 2012 Mar 18;119(10):2219-27. Epub 2012 Jan 18.

Department of Cellular Biotechnologies and Hematology, Sapienza University, Rome, Italy.

Sixty-four patients < 20 years of age, investigated for a suspicion of Philadelphia-negative myeloproliferative disease (MPD), were retrospectively evaluated to characterize the different forms and to examine the treatments used and long-term outcome. JAK2 mutations, endogenous erythroid colony growth, and clonality were investigated in 51 children. Mutations of thrombopoietin, the thrombopoietin receptor (MPL), and the erythropoietin receptor and mutations of other genes involved in the pathogenesis of MPD were investigated in JAK2 wild-type patients. Based on our criteria for childhood MPD, we identified 34 patients with sporadic thrombocythemia (ST), 16 with hereditary thrombocytosis (HT), 11 with sporadic polycythemia (SP), and 3 with hereditary polycythemia (HP). JAK2(V617F) mutations were present in 47.5% of ST and in no HT. The MPL(S505A) mutation was detected in 15/16 HT patients and in no ST (P < .00001). The JAK2(V617F) mutation occurred in 27% of SP patients diagnosed according to the Polycythemia Vera Study Group or World Health Organization 2001 criteria. Children with ST received more cytoreductive drugs than those with HT (P = .0006). After a median follow-up of 124 months, no patient had developed leukemia or myelofibrosis and 5% had thrombosis; the miscarriage rate in thrombocythemic patients was 14%. The low complication rate in our population suggests that children with MPD may be managed by tailored approaches.
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http://dx.doi.org/10.1182/blood-2011-08-371328DOI Listing
March 2012

Clinical outcome and monitoring of minimal residual disease in patients with acute lymphoblastic leukemia expressing the MLL/ENL fusion gene.

Am J Hematol 2011 Dec 22;86(12):993-7. Epub 2011 Sep 22.

Department of Cellular Biotechnologies and Hematology, Sapienza University, Via Benevento 6, Rome, Italy.

We analyzed 12 MLL/ENL positive ALL patients consecutively diagnosed between 1999 and 2009. The MLL/ENL fusion was identified in 4/150 (2.6%), 8/993 (0.8%), and 0/70 of pediatric, adult, and elderly patients, respectively. Eight patients had a WBC count >50 × 10(9) /L. Ten cases had an evaluable immunophenotyping. A B or T precursor ALL occurred in 7 and 3 patients, respectively. Eleven/12 patients (92%) achieved CR. At 48 months, overall survival and event-free survival rates were 73.3% and 67%, respectively. At CR, a parallel RT-PCR evaluation of the MLL/ENL expression was available in 5 cases. Of these latter, 2 tested MLL/ENL-negative and 3 positive. The minimal residual disease molecular monitoring showed that MLL/ENL status did not correlate with outcome. In fact, all the 2 PCR-negative and 1 of the 3 PCR-positive cases relapsed. Further, a MLL/ENL expression, not preceding a relapse, was detected several times during the follow-up of five long-survivors. In conclusion, also in adults, the MLL/ENL fusion identifies a rare leukemic entity with a favorable prognosis. The observed inconsistency between the clinical cure and the presence of detectable MLL/ENL transcript suggests the existence of a MLL/ENL-expressing "preleukemia" stem cells, similar to what demonstrated for the AML1/ETO-positive leukemia setting.
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http://dx.doi.org/10.1002/ajh.22161DOI Listing
December 2011

Treatment of children with B-cell non-Hodgkin lymphoma in a low-income country.

Pediatr Blood Cancer 2011 Apr 1;56(4):560-7. Epub 2010 Dec 1.

Division of Hematology, Department of Cellular Biotechnologies and Hematology, Sapienza University, Rome, Italy.

Background: An adapted LMB 96 derived protocol for B-cell non-Hodgkin lymphoma (NHL) was implemented at the pediatric oncology unit of the Children Welfare Teaching Hospital in Baghdad (Iraq) from 2000 to present. The purpose was to evaluate the feasibility and efficacy of this intensive therapeutic regimen in a limited resource country.

Methods: Patients <15 years of age with high grade B-cell NHL were included. A modified LMB 96 regimen was employed with a reduction of cyclophosphamide and methotrexate dosages due to inadequate laboratory facilities and supportive care.

Results: Between 2000 and 2005, 261 children with non-lymphoblastic NHL were registered; 239 were eligible for the analysis. Two patients had stage I disease, 20 stage II, 179 stage III, and 38 stage IV. Fifty-two patients (22%) had bulky disease. Twelve children were assigned to therapeutic group A (low risk), 184 to group B (intermediate risk), and 43 to group C (high risk). One hundred and eighty-four patients (77%) had a complete response after the COP pre-phase. Sixty-nine patients (29%) died during treatment. Twenty-nine patients abandoned treatment. At 24 months, the overall survival rate of the entire patient population was 66% (CI 95%: 62.2-70.6) and the event-free survival rate 53.3% (CI 95%: 50.0-56.8).

Conclusions: The treatment schedule proved effective, but the treatment-related mortality due to infections and metabolic complications was very high owing to the limited supportive care available. The high rate of treatment abandonment was also an important cause of failure, especially for children living far away from the hospital.
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http://dx.doi.org/10.1002/pbc.22905DOI Listing
April 2011

MACOP-B and involved-field radiotherapy is an effective and safe therapy for primary mediastinal large B cell lymphoma.

Int J Radiat Oncol Biol Phys 2008 Nov 9;72(4):1154-60. Epub 2008 May 9.

Department of Radiation and Radiotherapy, University of Rome La Sapienza, Rome, Italy.

Purpose: To report the clinical findings and long-term results of front-line, third-generation MACOP-B (methotrexate, doxorubicin, cyclophosphamide, vincristine, prednisone, and bleomycin) chemotherapy and mediastinal involved-field radiotherapy (IFRT) in 85 consecutive, previously untreated patients with primary mediastinal large B cell lymphoma (PMLBCL) diagnosed and managed at a single institution.

Methods And Materials: Between 1991 and April 2004, 92 consecutive, untreated patients with PMLBCL were treated at our institution. The median age was 33 years (range, 15-61 years), 46 patients (50%) showed a mediastinal syndrome at onset; 52 patients (57%) showed a low/low-intermediate (0 to 1) and 40 patients (43%) an intermediate-high/high (2 to 3) International Prognostic Index (IPI) score. Eighty-five patients were treated with standard chemotherapy (MACOP-B), and 80 underwent mediastinal IFRT at a dose of 30-36 Gy.

Results: After a MACOP-B regimen, the overall response rate was 87% and the partial response rate 9%. After chemotherapy, (67)Ga scintigraphy/positron emission tomography results were positive in 43 of 52 patients (83%), whereas after IFRT 11 of 52 patients (21%) remained positive (p < 0.0001). After a median follow-up of 81 months (range, 2-196 months), progression or relapse was observed in 15 of 84 patients (18%). The projected 5-year overall survival and progression-free survival rates were 87% and 81%, respectively. The 5-year overall survival and progression-free survival rates were better for patients with an IPI of 0 to 1 than for those with an IPI of 2 to 3 (96% vs. 73% [p = 0.002] and 90% vs. 67% [p = 0.007], respectively).

Conclusions: Combined-modality treatment with intensive chemotherapy plus mediastinal IFRT induces high response and lymphoma-free survival rates. Involved-field RT plays an important role in inducing negative results on (67)Ga scintigraphy/positron emission tomography in patients responsive to chemotherapy.
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http://dx.doi.org/10.1016/j.ijrobp.2008.02.036DOI Listing
November 2008

Unrelated cord blood transplant in children with high-risk acute lymphoblastic leukemia: a long-term follow-up.

Haematologica 2007 Aug 20;92(8):1051-8. Epub 2007 Jul 20.

Division of Hematology, Department of Cellular Biotechnologies and Hematology, University La Sapienza, Rome, Italy.

Background And Objectives: The aim of this single center study was to assess the impact of pre-transplant factors on long-term follow-up in young patients affected by high-risk acute lymphoblastic leukemia (ALL) who underwent an unrelated cord blood transplant (CBT). The conditioning regimens, graft-versus-host disease (GVHD) prophylaxis and supportive policies were uniform for all patients.

Design And Methods: We analyzed the results of CBT performed in 30 patients, aged <18 years, affected by high risk ALL. As conditioning regimen, all patients received 12 Gy fractionated total body irradiation, etoposide, cyclophosphamide and horse anti-lymphocyte globulin. GVHD prophylaxis consisted of 6-methylprednisolone and cyclosporine A. RESULTS The cumulative incidence of engraftment was 93% (95% CI:0.85-0.93). The cumulative incidence of grade III-IV acute and chronic GVHD was 7% (95% CI:0.01-0.19) and 33% (95% CI: 0.17-0.64), respectively. The 9-year cumulative incidence of transplant-related mortality and relapse was 34% (95% CI:0.13-0.45) and 31% (95% CI:0.16-0.61), respectively. The 9-year overall survival, leukemia-free survival and event-free survival were 42% (95% CI:0.52-0.93), 47% (95% CI:0.25-0.61) and 46% (95% CI:0.33-0.61), respectively. A number of CFU-GM <1 x 10(4)/Kg of recipient body weight was the only factor that negatively affected all outcome parameters both in univariate and multivariate analyses.

Interpretation And Conclusions: The infused cell dose expressed as in vitro progenitor cell growth represents the most important pre-transplant factor affecting the long-term outcome after an unrelated CBT in young patients with high risk ALL. The number of CFU-GM should thus be considered in the selection process of cord blood units for transplant.
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http://dx.doi.org/10.3324/haematol.11271DOI Listing
August 2007

CODOX-M/IVAC (NCI 89-C-41) in children and adolescents with Burkitt's leukemia/lymphoma and large B-cell lymphomas: a 15-year monocentric experience.

Leuk Lymphoma 2007 Mar;48(3):551-9

Division of Haematology, Department of Cellular Biotechnologies, University La Sapienza, Rome, Italy.

During the last 15 years, we have used the National Cancer Institute (NCI) 89-C-41 protocol in patients aged younger than 21 years with Burkitt's leukemia/lymphoma (BLL) and diffuse large B-cell lymphoma (DLBCL). According to the Magrath staging system, patients were classified as low and high risk. Low-risk received three cycles of the CODOX-M regimen; high-risk patients received four alternating cycles with the CODOX-M and IVAC regimens. Thirty-five patients entered the study: 32 (91%) achieved complete remission (CR); three were non-responders and died and one patient died in CR. Two responders relapsed after 2 months and one presented early B acute lymphoblastic leukemia 33 months from the end of therapy. The 5-year overall survival and event free-survival are 83% and 80%, respectively. No late toxicity was registered. In our experience with a median follow-up of 11 years, the NCI 89-C-41 protocol has confirmed its high cure rate in BLL and DLBCL children and adolescents.
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http://dx.doi.org/10.1080/10428190601078944DOI Listing
March 2007

Impact of international collaboration on the prognosis of childhood acute promyelocytic leukemia in Iraq.

Haematologica 2006 Apr 1;91(4):509-12. Epub 2006 Mar 1.

Division of Hematology, Department of Cellular Biotechnologies and Hematology, University La Sapienza, Rome, Italy.

The promotion of an operational network between hospitals and medical schools in Iraq and in Western countries is a primary humanitarian objective of international collaboration. As a consequence of a collaborative project between the Al Mansour Hospital for Pediatrics in Baghdad and the Pediatric Unit of Hematology of "La Sapienza" University, in Rome, in October 2003 a specific all-trans-retinoic acid-based protocol was designed in order to offer a modern therapeutic program for the management of Iraqi children with acute promyelocytic leukemia, adapted to the severe local difficulties. The preliminary encouraging results represent a substantial improvement over the earlier experience in childhood acute promyelocytic leukemia in Iraq.
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April 2006

Gamma-delta hepatosplenic T-cell lymphoma. Description of a case with immunophenotypic and molecular follow-up successfully treated with chemotherapy alone.

Leuk Lymphoma 2006 Feb;47(2):333-6

Division of Haematology, Department of Cellular Biotechnologies and Hematology, University La Spienza, Rome, Italy.

This study hereby reports the case of a 19-year old boy with a gamma-delta hepatosplenic T-cell lymphoma (HSTCL). Initial therapy consisted of four cycles of the IEV (Ifosphamide, Epirubicin and Etoposide) scheme. Further treatment strategy was then adapted according to minimal residual disease monitoring by immunophenotypic and T-cell receptor gamma chain gene evaluation. The patient remains in complete clinical, immunological and molecular remission and in good clinical conditions 48 months after diagnosis and 40 months after stopping therapy.
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http://dx.doi.org/10.1080/10428190500293321DOI Listing
February 2006

Anaplastic large cell lymphoma treated with a leukemia-like therapy: report of the Italian Association of Pediatric Hematology and Oncology (AIEOP) LNH-92 protocol.

Cancer 2005 Nov;104(10):2133-40

Clinica di Oncoematologia Pediatrica, Azienda Ospedaliera-Università di Padova, Padova, Italy.

Background: Childhood anaplastic large cell lymphoma (ALCL) is a well defined entity with a rather poor prognosis. Different approaches have been adopted in the treatment of ALCL in various cooperative trials, including short high-dose intensive therapy and leukemia-like protocols. In the early 1990s, the Italian Association of Pediatric Hematology and Oncology (AIEOP) initiated a multicenter trial for the treatment of ALCL based on a modified LSA2-L2 protocol.

Methods: Thirty-four consecutive eligible children with newly diagnosed ALCL were enrolled in the AIEOP LNH-92 protocol. Treatment was comprised of an induction of remission phase, followed by consolidation and maintenance for a total duration of 24 months, independently of disease stage.

Results: Thirty of 34 patients (88%) achieved complete disease remission and 8 patients experienced disease recurrence. With a median follow-up of 8.4 years, the probabilities of survival and event-free survival were 85% (range, 79-91%) and 65% (range, 57-73%), respectively. Therapy was well tolerated and hematologic toxicity was the most frequent toxicity.

Conclusions: The leukemia-like protocol AIEOP LNH-92 was found to be an effective treatment for childhood ALCL. Its long duration may be beneficial to specific patient subgroups, but optimal treatment duration in ALCL remains to be elucidated.
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http://dx.doi.org/10.1002/cncr.21438DOI Listing
November 2005

Breakthrough Candida krusei fungemia during fluconazole prophylaxis followed by breakthrough zygomycosis during caspofungin therapy in a patient with severe aplastic anemia who underwent stem cell transplantation.

J Clin Microbiol 2005 Oct;43(10):5395-6

Dipartimento di Biotecnologie Cellulari ed Ematologia, University "La Sapienza," Rome 00161, Italy.

We report a case of breakthrough invasive zygomycosis in a stem cell transplant recipient who was receiving caspofungin for treatment of a breakthrough Candida krusei fungemia that occurred during fluconazole prophylaxis. Also, patients receiving the echinocandin caspofungin remain at risk for pathogens, such as zygomycetes, that are intrinsically resistant to this agent.
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http://dx.doi.org/10.1128/JCM.43.10.5395-5396.2005DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1248476PMC
October 2005
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