9 results on '"Pasquale Niscola"'
Search Results
2. Bone marrow involvement by primary oxalosis
- Author
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Stefano Fratoni and Pasquale Niscola
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Pathology ,medicine.medical_specialty ,Primary Oxalosis ,medicine.anatomical_structure ,business.industry ,Medicine ,Hematology ,Bone marrow ,business ,Images of Hematology - Published
- 2020
3. Successful re-treatment with azacitidine in a patient with low blast count AML transformed from MDS after suspension of this agent
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Stefano Fratoni, Pasquale Niscola, Marco Giovannini, Laura Scaramucci, Andrea Tendas, and Paolo de Fabritiis
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Oncology ,Pediatrics ,medicine.medical_specialty ,business.industry ,Azacitidine ,Myeloid leukemia ,Hematology ,Settore MED/15 ,Blast Count ,Discontinuation ,medicine.anatomical_structure ,hemic and lymphatic diseases ,Internal medicine ,medicine ,Hypomethylating Therapy ,Bone marrow ,business ,Letter to the Editor ,medicine.drug - Abstract
TO THE EDITOR: The treatment approach to acute myeloid leukemia (AML) transformed from high-risk myelodysplastic syndrome (MDS) after azacitidine failure is not standardized and the clinical results achievable in this setting are generally disappointing [1,2,3]. Similarly, poor outcomes have also been reported in the setting of AML transformation following azacitidine discontinuation due to reasons other than hematologic progression in MDS patients initially responding to hypomethylating therapy [4]. Little is known of azacitidine-responsive MDS patients who progressed to AML with 20-30% bone marrow (BM) blasts after suspension of this agent, nor of the possibility to re-induce hematological control after the resumption of hypomethylating therapy.
- Published
- 2015
4. Evolution of chronic myelomonocytic leukemia from refractory anemia: the unusual course of a myelodysplastic syndrome
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Laura Scaramucci, Pasquale Niscola, Andrea Tendas, Marco Giovannini, Stefano Fratoni, and Paolo de Fabritiis
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medicine.medical_specialty ,business.industry ,Anemia ,Chronic myelomonocytic leukemia ,Hematology ,Erythroid dysplasia ,Settore MED/15 ,medicine.disease ,Gastroenterology ,Surgery ,Monocytosis ,International Prognostic Scoring System ,hemic and lymphatic diseases ,Refractory anemia with ring sideroblasts ,Internal medicine ,medicine ,Leukocytosis ,Macrocytic anemia ,medicine.symptom ,business ,Letter to the Editor - Abstract
TO THE EDITOR: Transformation from myelodysplastic syndrome (MDS) to chronic myelomonocytic leukemia (CMML) is rarely observed. However, this has been reported in cases of refractory anemia with ring sideroblasts or excess of blasts [1-4]. Moreover, MDS patients may present with monocytosis that does not meet the diagnostic criteria of CMML, which makes diagnosis and classification of these atypical mixed disorders a challenge [5, 6]. These difficulties in diagnostic classification and prognostic stratification may be concerning with regard to decision-making, particularly in this new era of effective disease-modifying therapies, such as hypomethylating agents [6, 7]. Recently, we faced such concerns during the management of a patient who developed CMML 7 years after having been diagnosed with refractory anemia (RA). The full clinical onset of CMML was preceded by progressive loss of response to ongoing treatment with an erythropoiesis-stimulating agent (ESA), worsening anemia, thrombocytopenia, leukocytosis, and increasing monocytosis. A morphological study of the peripheral blood (PB) and bone marrow (BM) revealed the coexistence of myelodysplastic and myeloproliferative syndromes. A cytogenetic alteration (45, X0,-Y), which was not present at diagnosis of RA 7 years earlier, was also detected during the CMML phase. The patient received azacitidine and showed a good response. Here, we describe this rare case and its implications in disease classification and management. On January 2005, a 74-year-old man presented with moderately macrocytic slight thrombocytopenia. Five years earlier (in 2000), he had received postoperative radiotherapy after radical prostatectomy for prostate cancer. Apart from this prostatic neoplasm, for which a regular oncological follow-up had confirmed a persistent complete remission until then, he mentioned one previously cured gastric ulcer and well-controlled hypertension. He complained of fatigue and general unease for the past few weeks. A complete blood count prescribed by his general practitioner had revealed macrocytic anemia with a low reticulocyte count, mild thrombocytopenia, and slight neutropenia. On admission, he appeared pale and fatigued. A comprehensive laboratory evaluation did not reveal any remarkable abnormalities. His coagulative profile and renal and hepatic function were normal. Suspicions of hemolytic disorders, virus infections, and iron and vitamin deficiencies were also dismissed. Morphological examination of PB smears showed isolated erythrocyte macrocytosis but did not provide any other diagnostic findings. BM aspiration and trephine biopsy were performed. BM examination revealed hypercellular BM with evident erythrodysplasia and 6% of blasts without fibrosis. Conventional karyotyping and fluorescence in situ hybridization analyses did not show abnormalities. Therefore, the patient was diagnosed with MDS, RA, according to the French-American-British classification. The patient had an International Prognostic Scoring System score of 1 (intermediate-1 risk). On admission and during the diagnostic phase, he received 4 units of red blood cell (RBC) concentrates. Thus, the patient was scheduled to receive ESA at a weekly dose of 40,000 units by subcutaneous injection (in January 2005). Since then, the patient was regularly followed-up at our clinic and continued to receive erythropoietin treatment. Normal PB values were maintained without the requirement for transfusion, clinical complications, or side effects until June 2012, when his hematologic status slowly began to deteriorate. Worsening anemia (requiring RBC transfusions), thrombocytopenia, and leukocytosis with absolute monocytosis became prominent. A comprehensive medical examination was performed. The examination of PB smears showed absolute monocitosis with 2% of circulating blasts. BM examination showed marked erythroid dysplasia and monocytosis with 15% blasts. A karyotype abnormality, such as 45, X0,-Y, which was not detected at initial diagnosis of RA 7 years earlier, was also found; however, the JAK2 V617F mutation was absent. Therefore, the patient was diagnosed with RA, coexisting with type II CMML. The patient had a MD Anderson Prognostic Score of 3 (intermediate-2 risk) [8]. After a brief course of hydroxycarbamide, administered in order to reduce leukocytosis, the patient received 6 cycles of azacitidine (75 mg/m2, schedule 5+2+2, each cycle every 4 weeks), according to approved indications [9]. After the second course, transfusion independence was achieved and the PB counts significantly improved with disappearance of monocytosis. Complete remission of CMML was achieved after 6 courses of hypomethylating therapy, without any adverse events. In conclusion, this case represents an atypical presentation of CMML secondary to MDS. In addition to its rarity and anecdotal interest, we believe that this report should be discussed with regard to several topics, such as the possible evolution of low-risk MDS in CMML, the existence of secondary CMML as a distinct disease, its absence in the current classification systems, and, finally, the efficacy of hypomethylating therapy [4, 10].
- Published
- 2013
5. Hematologists and nephrologists working together: moving forward with a new integrated care model for blood-related malignancies?
- Author
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Roberto Palumbo, Pasquale Niscola, Agostina Siniscalchi, Paolo de Fabritiis, Tommaso Caravita, and Paola Tatangelo
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0301 basic medicine ,medicine.medical_specialty ,business.industry ,Hematology ,Settore MED/15 ,Integrated care ,03 medical and health sciences ,030104 developmental biology ,0302 clinical medicine ,030220 oncology & carcinogenesis ,medicine ,Intensive care medicine ,business ,Letter to the Editor - Published
- 2017
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6. Steroid-refractory immune thrombocytopenia in the era of the new thrombomimetic drugs: is there still a role for rituximab?
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Paolo de Fabritiis, Laura Scaramucci, Pasquale Niscola, Massimiliano Palombi, Malgorzata Monika Trawinska, and Marco Giovannini
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medicine.medical_specialty ,medicine.medical_treatment ,Splenectomy ,Eltrombopag ,Disease ,030204 cardiovascular system & hematology ,01 natural sciences ,03 medical and health sciences ,chemistry.chemical_compound ,0302 clinical medicine ,Refractory ,hemic and lymphatic diseases ,Medicine ,0101 mathematics ,Letter to the Editor ,Thrombopoietin ,Romiplostim ,business.industry ,Hematology ,Settore MED/15 ,Immune thrombocytopenia ,Surgery ,010101 applied mathematics ,chemistry ,Rituximab ,business ,medicine.drug - Abstract
TO THE EDITOR: Although corticosteroids and splenectomy represents the main therapeutic strategies [1,2] for immune thrombocytopenia (ITP), some steroid-refractory patients may be unsuitable for the surgical procedure and therefore require alternative treatment options currently available, such as rituximab [2,3] and thrombomimetic drugs [4,5]. Although rituximab is usually used in refractory ITP patients who have failed multiple previous treatments [3], its role in the era of thrombomimetic drugs represents an open question. Herein, we would like to provide our perspective on this topic in the light of our clinical experience, reporting indications to rituximab in a small series of consecutive steroid–refractory/relapsed ITP patients treated with rituximab. There were seven patients (4 male) with a median age of 38 (19-43) years. Rituximab (4 wk infusions of 375 mg/m2) was given after 50 (6–84) months from the date of ITP diagnosis. One patient suffered from active bleeding requiring platelet transfusions and was refractory to all measures, including thrombomimetic agents (eltrombopag and then romiplostim); he achieved a complete response (CR) with rituximab and was soon after splenectomized, having taken into account the high risk of recurrence and his severe bleeding tendency. Splenectomy was offered to four other patients who refused; in the remaining two patients, splenectomy as well as thrombomimetic agents were not indicated given suspected underlying autoimmune disorders and thrombophilic states. All patients who received rituximab achieved a fast response; indeed, a significant increase in platelet count was recorded early during the course of treatment: one week after the first rituximab infusion in five and after two weeks in the remaining two patients respectively. All patients maintained a sustained CR; six did not necessitate further therapy whereas one was splenectomized soon after the response to rituximab. In all patients, CR was durable and persisted after a median follow-up of 19 months (range, 6-64). No patients relapsed. Based on these findings, rituximab therapy allowed, in our experience, for long-lasting remission in patients with relapsed or refractory ITP, with a good safety profile. All patients but one, who successfully received rituximab as a bridge to splenectomy, refused or presented contraindications for the surgical procedure. Although thrombopoietin agonists, such as eltrombopag or romiplostim, in light of their high efficacy, have substantially changed the clinical scenario and the management of steroid refractory ITP [4,5], this treatment option requires prolonged administration, namely for the remainder of one's life and without a predictable suspension of therapy. Indeed, the option of a potentially life-long treatment with thrombopoietin agonists was offered to patients who refused splenectomy after a thorough explanation of the expected benefits and possible disadvantages from the therapy. The long-lasting and dependent therapy, virtually to be administered for life, was the main reason patients sought out an alternative treatment; they wanted to induce a prolonged remission of their disease without needing to depend on the therapy for life. This concern, in the setting of patients who refuse or are unsuitable for splenectomy, can allow them to find a therapy the administration of which can be predictable and carried out in limited time. This includes rituximab, the role of which is yet to be defined for selected indications even in the era of the new thrombomimetic medications.
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- 2016
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7. Regaining the response to erythropoietin following azacitidine in chronic myelomonocytic leukemia previously evolved from refractory anemia
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Laura Scaramucci, Giulia Orlandi, Andrea Tendas, Roberta Merola, Paolo de Fabritiis, and Pasquale Niscola
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Ineffective erythropoiesis ,Oncology ,medicine.medical_specialty ,business.industry ,Azacitidine ,Chronic myelomonocytic leukemia ,Myeloid leukemia ,Context (language use) ,Hematology ,Settore MED/15 ,medicine.disease_cause ,medicine.disease ,medicine.anatomical_structure ,Erythropoietin ,hemic and lymphatic diseases ,Internal medicine ,Immunology ,medicine ,Bone marrow ,business ,Refractory cytopenia with multilineage dysplasia ,Letter to the Editor ,medicine.drug - Abstract
TO THE EDITOR: In a previously published issue of the Journal, we reported an unusual case of chronic myelomonocytic leukemia (CMML) type 2 (CMML-2) that evolved from refractory anemia (RA) [1]. The complete development of CMML was preceded by the loss of response to epoetin alpha (EPO). EPO had allowed the patient to maintain the transfusion independence (TI) for 7 years until then. The patient received azacitidine, achieving complete remission (CR) of CMML-2 after 6 treatment courses. However, the TI was observed after 2 cycles of hypomethylating therapy despite the persistence of myelodysplastic features. The optimal response to azacitidine in this patient was in line with that reported by our group earlier [2]. Moreover, the limited clinical efficacy of azacitidine in transfusion-dependent and erythropoiesis-stimulating agent (ESA)-resistant, low and intermediate-1 risk myelodysplastic syndrome (MDS) has been recently outlined [3]. Therefore, we could speculate that several and different biological components and pathogenic mechanisms, stemming from different patterns of response to distinct therapeutic agents, may be responsible for the myelodysplasia and the resulting clinical phenotype. These findings might be observed more easily during treatment with hypomethylating agents as these agents induce downstaging of high-risk MDS to low-risk MDS. Here, we report the updated follow-up of the clinical course of our patient, wherein we observed a dynamic evolution of MDS clones that showed different responses to azacitidine and EPO. The response to EPO was likely related to the persistence of RA clones that re-emerged despite azacitidine. After the achievement of the TI and the CR following the second and the sixth azacitidine courses, respectively, the patient continued to receive hypomethylating therapy as maintenance. However, after the fourteenth course, preceded by several weeks in which the patient complained of profound weakness and fatigue [4], progressive anemia (hemoglobin ~7.0 g/dL) requiring red blood cell (RBC) transfusions was noted. Therefore, the patient was reevaluated in the light of the suspicion of a loss of response to azacitidine and evolution to acute myeloid leukemia. However, examination of the bone marrow (BM) showed the disappearance of blast cells and a marked reduction of monocytes in a MDS framework, which was characterized by trilineage dysplasia and erythroid predominance with the features of ineffective erythropoiesis, as observed at the onset of MDS when the patient was diagnosed with RA [1]. According to its disease-modifying effects in MDS, azacitidine had "down-staged" the patient's malignancy from the transformed form of CMML-2 to refractory cytopenia with multilineage dysplasia (RCMD), resulting in a clinical phenotype similar to primary RA. On the basis of these findings, the patient continued to receive azacitidine as maintenance hypomethylating therapy and EPO was reintroduced. A rapid response with the achievement of TI was soon recorded. Therefore, the patient displaying near normal peripheral blood counts, continued to receive azacitidine concomitant with EPO, in order to maintain CR and TI. Our case presents some interesting observations. Initially, our patient was responsive to EPO for many years until the occurrence of an important transfusion requirement corresponding to transformation towards CMML-2. The treatment with azacitidine resulted in the clearance of this transformed, aggressive leukemic component probably by restoring a previous framework consistent with a low MDS risk, such as RCMD, as observed at the onset of primary MDS diagnosis. Therefore, it is likely that the clinical picture of the patient reflected maintained response to azacitidine, with good control of the CMML-2 and re-emergence of ineffective erythropoiesis typically associated with RA/RCMD MDS, which was again responsive to EPO. The patient was 83 years old and had a MDS history for 9 years; he was well after 21 courses of azacitidine and 6 months of TI with EPO. Although both the use of ESA [5] (to reduce the high burden of transfusion requirement in MDS patients [6]) and azacitidine [7,8] (to contain or reverse the natural progression of disease) are well established, very few studies have explored the therapeutic role exerted by the association of azacitidine with EPO in the setting of higher-risk MDS. A retrospective French study that included 32 higher-risk MDS patients, who concomitantly received ESA and azacitidine found that the addition of ESA to the hypomethylating therapy significantly improved overall survival independent of azacitidine schedule and duration [9]. These findings may reflect synergistic effects exerted by two agents or their separate actions on distinct mechanisms and/or components of MDS. However, no prospective studies have confirmed these findings and there is a need for prospective studies for confirmation [10]. However, we have not been able to thoroughly investigate this case with molecular techniques, owing to which we cannot clarify the biological and pathogenic bases for what we observed. Our case showed that MDS over its course can display different clinical manifestations, combining aspects associated with both high- and low-risk MDS forms. These different manifestations could correspond to underlying biological heterogeneity of different neoplastic clones or their transformation over time in the context of a continuum clonal evolution that may be influenced by the pattern of response to disease-modifying treatments.
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- 2015
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8. Acute myeloid leukemia arising from chronic myelomonocytic leukemia during hypomethylating therapy
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Laura Scaramucci, Marco Giovannini, Paolo de Fabritiis, Daniela Piccioni, Andrea Tendas, and Pasquale Niscola
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Oncology ,medicine.medical_specialty ,business.industry ,medicine.medical_treatment ,Azacitidine ,Myeloid leukemia ,Decitabine ,Chronic myelomonocytic leukemia ,Hematology ,Hematopoietic stem cell transplantation ,medicine.disease ,Pancytopenia ,Immunophenotyping ,Hypomethylating agent ,hemic and lymphatic diseases ,Internal medicine ,Immunology ,medicine ,business ,Letter to the Editor ,medicine.drug - Abstract
TO THE EDITOR: Acute myeloid leukemia (AML) arising from chronic myelomonocytic leukemia (CMML) exhibits a poor prognosis [1, 2] and represents a great challenge during hematological clinical practice. The introduction of hypomethylating therapies, such as azacitidine [3, 4] and decitabine [5], in CMML management has provided some important advances; however, currently the majority of CMML cases progress into AML. We present the characteristics of 2 patients, in whom the atypical and sudden progression from CMML to AML was observed when they were administered azacitidine. Previously, both cases had achieved a very good response to this agent. The first case is a 60-year-old man with a suspected therapy-related CMML, which arose 3 years after receiving chemo-radiotherapy treatment for a solid head and neck tumor. When diagnosed as having CMML, hypercellular bone marrow (BM) with 15% blasts was noted. Furthermore, the JAK2 V617F mutation was identified by quantitative PCR analysis, whereas standard cytogenetic and FISH analysis using BM and peripheral blood showed no abnormalities (normal karyotype). The MD Anderson Prognostic Scoring System (MDAPS) [6] classification was 4 (high risk). The patient received azacitidine at a dosage of 75 mg/m2 for 5+2 days (excluding weekends) every 4 weeks. After the fourth cycle of azacitidine, complete remission (CR) of CMML (BM blasts
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- 2014
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9. Primary diffuse large B-cell lymphoma of the bone marrow in a frail and elderly patient successfully treated with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone
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Stefano Fratoni, Pasquale Niscola, Alessio Perrotti, Massimiliano Palombi, and Paolo de Fabritiis
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Oncology ,medicine.medical_specialty ,Chemotherapy ,Vincristine ,Pathology ,Cyclophosphamide ,business.industry ,medicine.medical_treatment ,Hematology ,Settore MED/15 ,medicine.disease ,Lymphoma ,immune system diseases ,hemic and lymphatic diseases ,Internal medicine ,medicine ,Rituximab ,business ,Extranodal Involvement ,Letter to the Editor ,Anaplastic large-cell lymphoma ,Diffuse large B-cell lymphoma ,medicine.drug - Abstract
TO THE EDITOR: The involvement of bone marrow (BM) in aggressive non-Hodgkin lymphomas (NHLs) usually indicates systemic dissemination. However, although uncommon, extranodal involvement of the BM as an isolated and unique localization of aggressive NHLs [1] such as anaplastic large cell lymphoma [2] or diffuse large B-cell lymphoma (DLBCL) [3-6], has also been reported. In particular, primary DLBCL of the BM is a rare type of extranodal lymphoma with poor prognosis [1, 4]. Approximately 10 cases of primary DLBCL of the BM have been described thus far [3], and few of them have been reported in individuals of very advanced age. Here, we report a case of primary DLBCL of the BM that was successfully treated using rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP chemotherapy; 3-week standard schedule) in a frail 76-year-old woman.
- Published
- 2013
- Full Text
- View/download PDF
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