[Show abstract][Hide abstract] ABSTRACT: At present, very few data are available on deferasirox (DFX) in the treatment of patients with Philadelphia negative myeloproliferative neoplasms in fibrotic phase (FP-MPN) and transfusion dependence. To address this issue, a retrospective analysis of 28 patients (22 male, 6 female) with FP-MPN and iron overload secondary to transfusion dependence was performed, based on patients enrolled in the database of our regional cooperative group who received treatment with DFX. DFX was started after a median interval from diagnosis of 12.8 months (IR 7.1 - 43.1) with median ferritin values of 1,415 ng/ml (IR 1,168 - 1768). Extra-hematological toxicity was reported in 16/28 patients (57.1%) but only 2 patients discontinued treatment due to toxicity. Among 26 patients evaluable for response (≥ 6 months of treatment), after a median treatment period of 15.4 months (IR 8.1 - 22.3), 11 patients (42.3%) achieved a stable and consistent reduction of ferritin levels < 1,000 ng/ml. As for hematological improvement, 6/26 patients (23%) showed a persistent (> 3 months) rise of Hb levels > 1.5 g/dl, with disappearance of transfusion dependence in 4 cases. Treatment with DFX is feasible and effective in FP-MPN with iron overload. Moreover, in this setting an erythroid response can occur in a significant proportion of patients. This article is protected by copyright. All rights reserved.
This article is protected by copyright. All rights reserved.
European Journal Of Haematology 08/2015; DOI:10.1111/ejh.12674 · 2.07 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: To identify prognostic factors affecting Thrombosis-free survival (TFS) and Overall Survival (OS), we report the experience of a Regional cooperative group in a real-life cohort of 1144 patients with ET diagnosed from January 1979 to December 2010. There were 107 thrombotic events (9.4%) during follow-up [60 (5.3%) arterial and 47 (4.1 %) venous thromboses]. At univariate analysis, risk factors for a shorter TFS were: age > 60 yrs (p < 0,0054, CI95% 1.18 - 2.6), previous thrombosis (p< 0.0001, CI95% 1.58 - 4.52) and the presence of at least 1 cardiovascular risk factor (p= 0,036, CI95% 1.15 - 3.13). Patients with a previous thrombosis occurred ≥ 24 months before ET diagnosis had a shorter TFS compared to patients with a previous thrombosis occurred < 24 months (p=0.0029, CI95% 1.5 - 6-1); furthermore, patients with previous thrombosis occurred < 24 months did not show a shorter TFS compared with patients without previous thrombosis (p=0.303, CI95% 0.64 - 3.21). At multivariate analysis for TFS, only the occurrence of a previous thrombosis maintained its prognostic impact (p=0.0004, CI95% 1.48 - 3.79, RR 2.36). The 10-year OS was 89.9% (CI95% 87.3 - 92.5): at multivariate analysis for OS, age > 60 years (p<0.0001), anemia (p<0.0001), male gender (p=0.0019), previous thromboses (p=0.0344) and WBC > 15 x 10(9) /l (p=0.0370) were independent risk factors. Previous thrombotic events in ET patients are crucial for TFS but their importance seems related not to the occurrence per se but mainly to the interval between the event and the diagnosis.
American Journal of Hematology 05/2014; 89(5). DOI:10.1002/ajh.23685 · 3.80 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Bisphosphonates (BPs) are used intravenously to treat cancer-related conditions for the prevention of pathological fractures. Osteonecrosis of the jaw (BRONJ) is a rare complication reported in 4-15% of patients. We studied, retrospectively, 55 patients with multiple myeloma or Waldenstrom's macroglobulinemia followed up from different haematological departments who developed BRONJ. All patients were treated with BPs for bone lesions and/or fractures. The most common trigger for BRONJ was dental alveolar surgery. After a median observation of 26 months, no death caused by BRONJ complication was reported. In all, 51 patients were treated with antibiotic therapy, and in 6 patients, this was performed in association with surgical debridement of necrotic bone, in 16 with hyperbaric O(2) therapy/ozonotherapy and curettage and in 12 with sequestrectomy and O(2)/hyperbaric therapy. Complete response was observed in 20 cases, partial response in 21, unchanged in 9 and worsening in 3. The association of surgical treatment with antibiotic therapy seems to be more effective in eradicating the necrotic bone than antibiotic treatment alone. O(2) hyperbaric/ozonotherapy is a very effective treatment. The cumulative dosage of BPs is important for the evolution of BRONJ. Because the most common trigger for BRONJ was dental extractions, all patients, before BP treatment, must achieve an optimal periodontal health.
Blood Cancer Journal 03/2012; 2(3):e62. DOI:10.1038/bcj.2012.9 · 3.47 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: The current study was conducted to evaluate severe mucocutaneous toxicity during treatment with hydroxyurea (HU) in a large cohort of patients with Philadelphia chromosome-negative myeloproliferative neoplasms (MPN).
Among 993 consecutive patients newly diagnosed with MPN at 4 centers in Rome between January 1980 and December 2009, 614 patients (277 men and 337 women with a median age of 64.4 years [interquartile range (IR), 54.4 years-72.7 years]) received HU. HU was administered as first-line treatment in 523 patients (85.2%) and as ≥ second-line treatment in 91 patients (14.8%).
Mucocutaneous toxicity was reported in 51 patients (8.3%) after a median period from the initiation of HU treatment of 32.1 months (IR, 10.5 months-74.6 months) and a mean HU dose of 1085 mg (± 390 mg); 30 patients (58.8%) developed a painful ulcerative skin toxicity, mainly located in the perimalleolar area; 11 patients (21.6%) had oral aphthous ulcers; and 10 patients (19.6%) developed a nonulcerative skin toxicity with erythema and skin infiltration. After the mucocutaneous toxicity occurred, HU treatment was continued at the same dose in 5 patients (9.8%), reduced in 12 patients (23.5%), and temporarily discontinued in 7 patients (13.7%); the remaining 27 patients (52.9%) required a permanent drug discontinuation. After a median period of 4.3 months (IR, 2.4 months-9.0 months) from the onset of the skin toxicity, 39 patients (76.5%) had a complete resolution and 12 patients (23.5%) had improvement without complete resolution.
Mucocutaneous toxicity during HU treatment is more common than expected and may present with different clinical features. Moreover, it often requires a permanent drug discontinuation and only a partial resolution is reported to occur in approximately 25% of patients.
Cancer 01/2012; 118(2):404-9. DOI:10.1002/cncr.26194 · 4.89 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Evidence of long-term response to lenalidomide in heavily pretreated patients with multiple myeloma is lacking. This study sought to assess whether long-term responders exist, long-term responders' characteristics, and predictive factors of a long-term response. One hundred and four patients with multiple myeloma treated with lenalidomide and dexamethasone after ≥2 therapy lines (median, 3) were analyzed. Long-term response was defined as at least a partial response (≥PR) lasting ≥12 months. The overall response rate was 73%, and 80.3% of the responses were achieved within 5 months. The median response was 14.3 months. Patients evaluable for long-term response numbered 87, and a total of 47% were long-term responders. Compared to non-long-term responders, long-term responders had better overall survival, less light-chain multiple myeloma, and higher incidence of t(11;14). Previous allogeneic transplant (alloSCT) and the response quality predicted a long-term response. In conclusion, patients treated with lenalidomide can become long-term responders; alloSCT and response quality predict long-term response.
[Show abstract][Hide abstract] ABSTRACT: Epidemiological and clinical information on primary plasma cell leukemia (pPCL) are rarely reported. The aims are to evaluate the clinical features, prognostic factors, and efficacy of treatments in pPCL.
A multicenter retrospective cohort study was carried out from January 2000 to December 2008 in 26 Italian hematology divisions. A total of 128 cases of plasma cell leukemia were collected, and 73 of them (57%) were classified as primary (male/female 43/30).
Sixty-four patients had at least 1 sign of end-organ damage and 10 had extramedullary localization. One patient died early; of the remaining patients, 36 (50%) received anthracycline-based regimens as first-line therapy, 17 (24%) single alkylating agents, and 30 (42%) bortezomib or thalidomide as additional (n = 11) or unique treatments (n = 19). Twenty-three patients (31%) underwent autologous and/or allogeneic hematopoietic stem cell transplantation (HSCT). The median overall survival (OS) was 12.6 months; complete or partial response was achieved in 22 (30%) and 18 patients (25%), respectively; the median duration of response (DOR) was 16.4 months. HSCT patients had a longer OS and DOR (median 38.1 and 25.8 months, respectively) compared with nontransplanted patients (9.1 and 7.3 months, respectively, P < 0.001). OS was influenced by nonresponse to treatment, hypoalbuminemia, and HSCT. DOR was favorably influenced only by HSCT.
pPCL is an aggressive disease with a poor prognosis and a low response rate to conventional therapy. HSCT is effective, increasing OS and DOR by 69% and 88%, respectively. The use of bortezomib and thalidomide may improve outcomes.
Annals of Oncology 06/2011; 22(7):1628-35. DOI:10.1093/annonc/mdq646 · 7.04 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Anemia is defined as a reduction of hemoglobin (Hb) levels of <13 g/dL for a man and <12 g/dL for a woman. It is very frequent
among hospitalized patients and can be a serious problem in patients who undergo cervical spine surgery. Anemia can be classified
Mild (Hb: >10 g/dL)
Moderate (Hb: 8–10 g/dL)
Severe (Hb: 6–8 g/dL)
Very severe (Hb: <6 g/dL)
To identify the causes of anemia, we can use functional and morphologic criteria. However, from a practical point of view,
the morphologic criteria are preferred and require the reticulocytes absolute number and the mean corpuscular volume (MCV),
respectively. The flowchart for identifying the causes of anemia using morphologic criteria is reported in Fig. 2.1.
[Show abstract][Hide abstract] ABSTRACT: The presenting clinico-hematologic features of 1,283 patients with IgG and IgA monoclonal gammopathies of undetermined significance (MGUS) were correlated with the frequency of evolution into multiple myeloma (MM).
Two IgG MGUS populations were evaluated: a training sample (553 patients) and a test sample (378 patients); the IgA MGUS population consisted of 352 patients.
Forty-seven of the 553 training group patients and 22 of 378 test group IgG patients developed MM after a median follow-up of 6.7 and 3.6 years, respectively. Multivariate analysis showed that serum monoclonal component (MC) levels of < or =1.5 g/dL, the absence of light-chain proteinuria and normal serum polyclonal immunoglobulin levels defined a prognostically favorable subset of patients, and could be used to stratify the patients into three groups at different 10-year risk of evolution (hazard ratio, 1.0, 5.04, 11.2; P < 0.001). This scoring system was validated in the test sample. Thirty of the 352 IgA patients developed MM after a median follow-up of 4.8 years, and multivariate analysis showed that hemoglobin levels of <12.5 g/dL and reduced serum polyclonal immunoglobulin correlated with progression. A pooled statistical analysis of all of the patients confirmed the validity of Mayo Clinic risk model showing that IgA class, serum MC levels, and light-chain proteinuria are the most important variables correlated with disease progression.
Using simple variables, we validated a prognostic model for IgG MGUS. Among the IgA cases, the possible prognostic role of hemoglobin emerged in addition to a decrease in normal immunoglobulin levels.
Clinical Cancer Research 06/2009; 15(13):4439-45. DOI:10.1158/1078-0432.CCR-08-3150 · 8.72 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Liver plasmacytoma is a very rare form of solitary plasmacytoma, in fact the presence of plasma cells in the liver is generally associated with a more aggressive form of multiple myeloma. We report an unusual case of liver plasmacytoma without systemic disease, diagnosed by percutaneous needle biopsy of the hepatic lesion, treated with six courses of melphalan and prednisone who achieved a good clinical remission after five years of follow-up.
Leukemia and Lymphoma 07/2003; 44(6):1075-6. DOI:10.1080/1042819031000067882 · 2.89 Impact Factor