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  • 1
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    Thrombosis and survival in essential thrombocythemia: A regional study of 1,144 patients
    Wiley ; 2014
    In:  American Journal of Hematology Vol. 89, No. 5 ( 2014-05), p. 542-546
    Details
    In: American Journal of Hematology, Wiley, Vol. 89, No. 5 ( 2014-05), p. 542-546
    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 1,144 patients with essential thrombocythemia (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 years ( P   〈  0.0054, 95% CI 1.18–2.6), previous thrombosis ( P   〈  0.0001, 95% CI 1.58–4.52) and the presence of at least one cardiovascular risk factor ( P  = 0.036, 95% CI 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, 95% CI 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, 95% CI 0.64–3.21). At multivariate analysis for TFS, only the occurrence of a previous thrombosis maintained its prognostic impact ( P  = 0.0004, 95% CI 1.48–3.79, RR 2.36). The 10‐year OS was 89.9% (95% CI 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 white blood cell 〉 15 × 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. Am. J. Hematol. 89:542–546, 2014. © 2014 Wiley Periodicals, Inc.
    Type of Medium: Online Resource
    ISSN: 0361-8609 , 1096-8652
    URL: Issue
    URL: Article
    DOI: 10.1002/ajh.v89.5
    DOI: 10.1002/ajh.23685
    Language: English
    Publisher: Wiley
    Publication Date: 2014
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  • 2
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    Hematological Improvement during Deferasirox Treatment in Patients with Myeloproliferative Neoplasms (MPN)
    American Society of Hematology ; 2014
    In:  Blood Vol. 124, No. 21 ( 2014-12-06), p. 3189-3189
    Details
    In: Blood, American Society of Hematology, Vol. 124, No. 21 ( 2014-12-06), p. 3189-3189
    Abstract: Background Deferasirox (DFX) is an oral iron chelator widely employed to reduce iron overload in patients with myelodysplastic syndromes; in this subset, an unexpected hematological improvement (HI) has been reported in about 20% of cases by several groups. At present, only few case reports showed a similar HI in patients with Ph- Myeloproliferative Neoplasms (MPN) and transfusional requirement receiving DFX treatment. Aim To address the incidence of HI during DFX treatment in MPN patients, we revised all patients with MPN and iron overload secondary to transfusional requirement enrolled in the database of our regional cooperative group who received a treatment with deferasirox. Methods Twenty-eight patients [M/F 22/6, median age at diagnosis 68.8 years, interquartile range (IQR) 63.2 – 74.3] were reported in the database. Of them, 25 had a primary Myelofibrosis, 2 a post Essential Thrombocythemia myelofibrotic phase (MP) and 1 a post Polycythemia Vera MP. An HI was considered as a rise in Hb values ≥ 1.5 g/dl and/or a reduction in the transfusional requirement ≥ 50% lasting at least 3 months. Results Treatment with DFX was started after a median interval from diagnosis of 12.8 months (IR 7.1 – 43.1) and from transfusion requirement of 10.0 months (IR 5.7 – 15.9). Median Hb and ferritin values at baseline were 7.8 g/dl (IQR 7.2 – 8.4) and 1,415 ng/ml (IQR 1,168 – 1768), respectively. Starting DFX dose was 1,500 mg/day in 13 patients, 1,250 mg/day in 5 patients, 1,000 mg/day in 9 patients and 〈 1,000 mg/day in 1 patient. Extra-hematological toxicity was reported in 16/28 patients (57.1%) and a dose reduction/temporary discontinuation was needed in 12 cases (42.8%), but only 2 patient went off treatment due to toxicity. Twenty-six patients were considered 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 reduction of iron overload with ferritin levels 〈 1,000 ng/ml. As to HI, 6/26 patients (23.0%) showed an unexpected and persistent ( 〉 3 months) rise of Hb levels 〉 1.5 g/dl, with disappearance of transfusional requirement in 4 cases and reduction ≥ 50% in the remaining 2 cases. The main clinical features of these 6 patients at DFX initiation and at HI are reported in the Table. Table 1.GenderAge (yrs)Months from diagnosisMonths from transfusionsHb (g/dl)Ferritin (ng/ml)Hb at HI (g/dl)Ferritin at HI (ng/ml)Months to HI# 1M68.7101.545.28.21,59010.127218# 2M67.513.313.28.7130911.265912# 3M77.29.86.08.017909.6168212# 4F76.721.715.39.1121410.5104112# 5M71.86.94.66.8264110.884924# 6M67.179.46.78.070610.05006 The HI was achieved together with a reduction of ferritin levels in 4 cases, while it was independent by ferritin response in the remaining 2 cases. To identify factors predicting the achievement of HI, many features at baseline (age, gender, Hb and ferritin levels, interval from diagnosis to DFX treatment) were compared between patients obtaining or not HI: however, none of them showed a significant role. Conclusions Treatment with DFX is feasible and effective in MPN with iron overload. Moreover, it is worth of note and deserves further biological and clinical insights that also in this setting an hematological improvement can occur in a sizeable rate of patients. Disclosures Latagliata: Novartis: Consultancy; Bristol Myers-Squibb: Consultancy; Celgene: Consultancy; Shire: Consultancy. Breccia:Celgene: Consultancy; BMS: Consultancy; novartis: Consultancy.
    Type of Medium: Online Resource
    ISSN: 0006-4971 , 1528-0020
    URL: Article
    DOI: 10.1182/blood.V124.21.3189.3189
    RVK:
    XA 33000
    RVK:
    XA 10000
    Language: English
    Publisher: American Society of Hematology
    Publication Date: 2014
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  • 3
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    Risk Factors for Thrombosis Vary According to Age in Patients with Essential Thrombocythemia: a Retrospective Analysis of 1090 Patients from the “Gruppo Laziale SMPC Ph Negative “,
    American Society of Hematology ; 2011
    In:  Blood Vol. 118, No. 21 ( 2011-11-18), p. 3854-3854
    Details
    In: Blood, American Society of Hematology, Vol. 118, No. 21 ( 2011-11-18), p. 3854-3854
    Abstract: Abstract 3854 Increasing age is a well-recognised risk factor for thrombotic events in patients with Essential Thrombocythemia (ET): however, few data exist on the role of other clinical and biological features in different age groups. To address this issue, we analysed retrospectively 1090 ET patients (M/F 403/687, median age 63 years, IR 17 – 96) diagnosed at 11 Hematological Institutions in the Lazio region from 1980 to 2010 and with a median period of follow-up of 84 months (IR 1 – 371). Based on the commonly adopted age threshold, 480 patients (44 %) were 〈 60 years (Group A) and 610 (56 %) were ≥ 60 years (Group B). Clinical and biological features as well as cardiovascular risk factors analyzed for the impact on the thrombotic risk in the two age groups are reported in the Table.Group A 〈 60 yearsGroup B ≥ 60 yearsPutative risk factorsRisk ratio (95% CI)P valueRisk ratio (95% CI)P valueM/F167/3132.68 (1.03–6.94)0.0029236/3741.12 (0.17–2.59)0.73WBC median (range) x 109/l8.9 (4.29–22.35)0.387 (0.149–1,004)0.06458.9 (1.2–57.7)0.79 (0.41–1.47)0.445PLTS median (range) x 109/l837 (451–3582)0.37 (0.258–1.70)0.66802 (450–3104)0.52 (0.28–0.99)0.0052Hb median, g/dL (range)14.1 (6.0–18.4)0.86 (0.33–2.24)0.76914.0 (7.0–17.8)0.87 (0.45–1.67)0.674*JAK-2 mutational status: wild type/mutated (%)53.2/46.81.57 (0.50–4.87)0.4434.1/65.90.498 (0.17–1.48)0.209Previous thrombotic events: n° (%)· All events72 (15)2.18 (0.59–7.96)0.12149 (24.4)3.01 (1.38–6.57)0.0004· within 24 months from diagnosis48 (10)1.43 (0.19–10.4)0.7464 (10.5)0.506 (0.18–1.39)0.189· within 60 months from diagnosis60 (12.5)NA0.5191 (14.9)0.323 (0.11–0.95)0.023Cardiovascular risk factors: Y/N %○ Arterial hypertension41.7/58.31.68(0.64–4.36)0.2880.7/19.30.96 (0.36–2.57)0.935○ Diabetes10.2/89.81.11 (0.23–5.15)0.8925.0/75.01.09 (0.38–3.11)0.86○ Smoking attitude45.6/54.42.78 (1.01–7.65)0.06758.3/41.71.04 (0.35–3.09)0.94○ Hyperlipidemia31.0/69.03.11(0.917–10.592)0.03951.6/48.42.31 (0.70–7.55)0.203 In Group A, 39 patients (8.1%) had at least one thrombotic event during follow-up; there were 20 (51.3%) arterial thrombosis and 19 (48.7%) venous thrombosis. In Group B, 63 patients (10.3%) had at least one thrombotic event during follow-up; there were 38 (69.4%) arterial thromboses and 25 (39.6%) venous thromboses. In group A univariate analysis for thrombosis-free survival performed by Kaplan-Meier method, disclosed a significant impact of male gender (p=0.0029, CI 1.03–6.94, HR 2.68), 〉 2 cardiovascular risk factors (p=0.0002, CI 1.87 – 190, HR 18.94) and isolated hyperlipidemia (p=0.039, CI 0.917 – 10.59, HR 3.11), while previous thrombotic events had no significant impact (p=0.27). By contrast, the presence of a previous thrombotic event was the only feature with a significant impact on thrombotic risk in Group B (p=0.0004, CI 1.38 – 6.55, HR 3.01). WBC and PLTS values at different cut-off levels as well as JAK-2 mutational status did not have any impact on thrombosis in either age groups. However, in group B, we observed a trend (p=0.052, CI 0.28–0.99, HR 0.52) towards a protective effect of higher PLTS values ( 〉 800 × 109/l). In conclusion, our data seem to reinforce the need of a different thrombotic risk assessment in distinct age groups: in particular, younger patients could benefit from early recognition and treatment of well-known cardiovascular risk factors. Disclosures: No relevant conflicts of interest to declare.
    Type of Medium: Online Resource
    ISSN: 0006-4971 , 1528-0020
    URL: Article
    DOI: 10.1182/blood.V118.21.3854.3854
    RVK:
    XA 33000
    RVK:
    XA 10000
    Language: English
    Publisher: American Society of Hematology
    Publication Date: 2011
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  • 4
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    Are ET and PV Patients Two Similar Populations As Concern Thrombotic Risk Factors?
    American Society of Hematology ; 2015
    In:  Blood Vol. 126, No. 23 ( 2015-12-03), p. 2811-2811
    Details
    In: Blood, American Society of Hematology, Vol. 126, No. 23 ( 2015-12-03), p. 2811-2811
    Abstract: Thrombotic events are major complications in patients (pts) affected by Essential Thrombocytemia (ET) and Polycytemia Vera (PV). To compare thrombotic risk in these 2 groups, we evaluated retrospectively our database of 1249 ET and 623 PV pts diagnosed and followed in 11 hematological centers in the Latium region between 1/1980 and 12/2010: the diagnosis was done according to PVSG, WHO 2001 and 2008criteria based on the time of first observation. Baseline features of ET pts: 797F/452M,median age 62.9 yrs (range 19-96),median WBC count 8.8 x 109/L (range 1.2-57.7), median PLT count 812 x 109/L (range 457-3582), median Hb level 14.0 g/dl (range 6-20.5), JAK-2V617F positivity 59.7% with a median allele burden of 19,6% (range 0.2- 99.9), spleen enlargement in 18.7% of pts, previous thrombosis223/1239 evaluable pts (17.9%) [arterial 176/223 (14.1%), venous 47/223 (3.8%)]. Baseline features of PV pts: 289F/334M, median age 63.0yrs (range 21-91), median WBC count 10.1 x 109/L (range 3.5-37.6), median PLT count 457 x 109/L (range 169-1790), median Hb level 18.2 g/dl (range 10.5-24.8), JAK-2V617F positivity 94.3% with a median allele burden of 59.1% (range 0.3-99.9), spleen enlargement in 42% of patients, previous thrombosis 146/617 evaluable pts (23.7%)[arterial 114/617 (18.5%), venous 32/617 (5,2%)] .in the ET cohort, after a median follow-up of 7.7 yrs, thrombotic complications were seen in 107/1141 evaluable pts (9.4%) [arterial60 (5.25%), venous 47 (4.11%)]; in the PV cohort, after a median follow-up of 8.5 yrs, thrombotic complications were seen in 107/623pts (17.2%) [arterial 67 (10.8%),venous 40 (6.4%)] .All common risk factors for thrombosis were evaluated in multivariate analysis, searching the cut-off number for continuous variables with ROC curves. The significant variables at multivariate analysis for ET and PV pts are shown in the table; age, previous thromboses and spleen enlargement were risk factors in ET pts, while previous thromboses and JAK-2V617F allele burden were risk factors in PV pts. PLT count above ROC value seemed to be a protective factor in both cohorts. In conclusion, in contrast with the tendency to evaluate in a similar manner the thrombotic risk of PV and ET, data from our retrospective database showed that these 2 groups should be considered populations with different risk factors for thrombosis. Table 1.Putative prognostic factorsPolycythemia VeraEssential ThrombocythemiaHR95% C.I.pHR95% C.I .pPrevious thromboses2,311,13 - 4,740,021,871,08 -3,230,026Age ≥ 60 y1,540,79 - 2,990,211,901,18 - 3,060,009JAK2V617FPV: allelic burden ≥ 81% ET: pos1,951,03 - 3,710,040,760,48 - 1,210,25Plt countPV ≥ 452.109/L ET ≥ 944.109/L0,490,25 - 0,950,040,520,31 - 0,890,017Spleen enlargement0,670,34 -1,310,241,711,02 - 2,890,04CV risk factors (at least 1)0,920,41 - 2,030,830,870,51 - 1,490,62WBCPV ≥ 10,175.109/L ET ≥ 9,630.109/L1,090,57 - 2,080,801,410,89 -2,260,15 Disclosures No relevant conflicts of interest to declare.
    Type of Medium: Online Resource
    ISSN: 0006-4971 , 1528-0020
    URL: Article
    DOI: 10.1182/blood.V126.23.2811.2811
    RVK:
    XA 33000
    RVK:
    XA 10000
    Language: English
    Publisher: American Society of Hematology
    Publication Date: 2015
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  • 5
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    Imatinib In Very Elderly CML Patients: What Can We Achieve?.
    American Society of Hematology ; 2010
    In:  Blood Vol. 116, No. 21 ( 2010-11-19), p. 1229-1229
    Details
    In: Blood, American Society of Hematology, Vol. 116, No. 21 ( 2010-11-19), p. 1229-1229
    Abstract: Abstract 1229 In the “real world” of clinical practice, many very elderly CML patients have been treated with imatinib (IM), but there are few data on the results and the best initial dosage in such patients. To highlight peculiar aspects of toxicity and efficacy of IM in this subset which accounts for at least 10–15% of all CML cases, we retrospectively revised 156 CML patients in chronic phase treated with IM when aged 〉 75 years from 23 haematological Institutions in Italy; there were 85 males and 71 females, median age at IM start was 78.4 years (IR 76.1 – 81.4), Sokal Risk at diagnosis was low in 2 patients, intermediate in 90, high in 50 and not evaluable in 14. One or more concomitant diseases requiring specific treatments were present in 144/156 patients (92.3%), with 94 patients (60.2%) assuming 3 or more concomitant drugs. Thirty patients (19.2%) were in late chronic phase (≥ 12 months from diagnosis) and pretreated (25 with HU and 5 with IFN) before starting IM; on the whole, median time from diagnosis to IM was 1.2 months (IR 0.5 – 3.6). Starting dose of IM was 400 mg/day in 117 patients (75.0%) and 300 mg/day or less in 39 patients (25.0%); overall, 59 patients (37.8%) (52/117 at 400 mg starting dose and 7/39 at 3 300 mg starting dose) needed a dose reduction and 18 (11.5%) discontinued IM for toxicity (early toxicity in 13 and late toxicity in 5). Excluding the 13 patients who discontinued IM due to early toxicity, maximum tolerated daily dose during treatment was 400 mg in 63 patients, 300 mg in 51 patients and 〈 300 mg in 29 patients. According to CTC-AE, grade 3 – 4 hematological and extra-hematological toxicities were observed in 34 (21.7%) and 34 (21.7%) patients, respectively; 5 patients (3.2%) presented a pleural effusion during IM treatment. After a median treatment period of 29.4 months (IR 7.9 – 54.4), 8 patients (5.1%) are still too early ( 〈 6 months of treatment), 13 (8.3%) discontinued IM due to early toxicity, 3 (1.9%) were resistant and 1 (0.7%) died from unrelated cause early after IM initiation: the remaining 131 patients (84.6%) achieved a complete haematological response (CHR). Among these 131 patients in CHR, 11 refused any other karyotipic or molecular evaluation (1 lost CHR and shifted to hydroxyurea, 4 are still alive in CHR, 6 died in CHR from unrelated causes), 17 achieved CHR only and 103 (66.0% of all 156 patients) achieved a cytogenetic response (CyR), which was major in 11 patients and complete (CCyR) in 92 (58.9% of all 156 patients). In addition, among the 92 patients in CCyR, 62 (39.7% of all 156 patients) achieved a molecular response (major molecular response in 40 patients and complete molecular response with an undetectable BCR/ABL hybrid gene at qualitative nested PCR in 22 patients). After a median follow-up of 34.0 months (IR 12.9 – 60.0), 36 patients have died (5 from disease progression and 31 from unrelated causes), 4 patients were lost to follow-up and 116 are still alive: 2-year and 4-year overall survival were 90.2% (CI95% 84.8 – 95.6) and 76.8% (CI95% 68.6 – 85.0), respectively. In conclusion, results from this large unselected cohort of patients show that should be definitely considered unethical to avoid IM therapy to any elderly patient; no upper age limit should be given but also very elderly (and with concomitant severe diseases) patients should have this chance of cure. The role of a reduced starting dose of IM warrants further studies. Disclosures: No relevant conflicts of interest to declare.
    Type of Medium: Online Resource
    ISSN: 0006-4971 , 1528-0020
    URL: Article
    DOI: 10.1182/blood.V116.21.1229.1229
    RVK:
    XA 33000
    RVK:
    XA 10000
    Language: English
    Publisher: American Society of Hematology
    Publication Date: 2010
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  • 6
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    Essential Thrombocythemia: A Comparison of Overall and Thrombosis Free Survival in Two Discrete Periods of the First Decade of 2000. a Retrospective Analysis
    American Society of Hematology ; 2016
    In:  Blood Vol. 128, No. 22 ( 2016-12-02), p. 5469-5469
    Details
    In: Blood, American Society of Hematology, Vol. 128, No. 22 ( 2016-12-02), p. 5469-5469
    Abstract: To evaluate the prognosis of patients with Essential Thrombocythemia (ET) in the first decade of the century we assessed retrospectively the thrombosis free survival (TFS) and the overall survival (OS) of the patients diagnosed from 01/01/2000 to 31/12/2009 and collected in the database of our group. The diagnosis of ET was performed with PVSG, WHO 2001 or WHO 2008 criteria, according to the period of the first observation. The whole population of 757 patients was then divided in two groups: the first (group I) with the diagnosis performed between 01/01/2000 to 31/12/2005 (334 patients) with a median follow-up of 111,9 months, the second (group II) diagnosed between 01/01/2006 to 31/12/2009 (385 patients) with a median follow-up of 58,2 months. The main clinical features of the two groups of patients are reported in the Table 1. No difference was observed between the two groups as to age, gender, platelet and WBC count, Hb level, Cardio-Vascular Risk Factors (CVRF), spleen enlargement and occurrence of previous thrombotic events. The frequency of the JAK-2 V617F mutation resulted significantly different (49.1% vs 68.4%) but in the group I the search of the mutation was never performed at the diagnosis. The TFS and OS were calculated from the date of diagnosis to the date of any appropriate event or to the date of last follow-up with Kaplan-Meier product limit method; the comparison of proportions and median values was computed with the Chi-squared and the Mann-Withney tests, as indicated. No significant difference emerged neither for TFS (p= 0,09, HR 1,42, 95% C.I. 0.89-2.30) nor for OS (p= 0,15, HR 1,34, 95% C.I. 0,87-2,06). We also considered the type of treatment used in the two groups to assess the potential link between the therapy and TFS or OS. No difference emerged between the two groups as to anti-aggregating treatment (mainly ASA), equally utilized in both groups [287/369, 77,8%, and 330/383, 78,3%, respectively (p = 0,95)]. As for the cyto-reductive therapy, Hydroxyurea was used in 74.8% vs 67.9% (p= 0.60) and alkylating agents in 1.9% vs 2.1% (p= 0.85), whereas Anagrelide was used in 10,6% vs 3,9% (p= 0,001) and Interferon in 9,5% vs 5,2% (p= 0,037), respectively. This more frequent use of Anagrelide and Interferon in the first group (2000-2005) did not modify TFS and OS of the patients. In conclusion, no improvement was observed in the prognosis of ET patients in the recent years: thus, new efforts to identify patients at risk and the introduction of new drugs as JAK-2 inhibitors are warranted to improve the prognosis of these patients. Table Table. Disclosures Breccia: Bristol Myers Squibb: Honoraria; Pfizer: Honoraria; Novartis: Consultancy, Honoraria; Celgene: Honoraria; Ariad: Honoraria. Cimino:Celgene: Honoraria; Bristol-Mayer: Honoraria. Lo Coco:Pfizer: Consultancy; Baxalta: Consultancy; Novartis: Consultancy; Lundbeck: Honoraria, Speakers Bureau; Teva: Consultancy, Honoraria, Speakers Bureau. Latagliata:Novartis: Consultancy, Honoraria; Bristol Myers Squibb: Honoraria; Celgene: Honoraria; Janssen: Consultancy, Honoraria; Shire: Honoraria.
    Type of Medium: Online Resource
    ISSN: 0006-4971 , 1528-0020
    URL: Article
    DOI: 10.1182/blood.V128.22.5469.5469
    RVK:
    XA 33000
    RVK:
    XA 10000
    Language: English
    Publisher: American Society of Hematology
    Publication Date: 2016
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  • 7
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    Role of treatment on the development of secondary malignancies in patients with essential thrombocythemia
    Wiley ; 2017
    In:  Cancer Medicine Vol. 6, No. 6 ( 2017-06), p. 1233-1239
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    In: Cancer Medicine, Wiley, Vol. 6, No. 6 ( 2017-06), p. 1233-1239
    Abstract: Aim of this study is to explore the role of different treatments on the development of secondary malignancies ( SM s) in a large cohort of essential thrombocythemia ( ET ) patients. We report the experience of a regional cooperative group in a real‐life cohort of 1026 patients with ET . We divided our population into five different groups: group 0, no treatment; group 1, hydroxyurea ( HU ); group 2, alkylating agents ( ALK ); group 3, ALK  +  HU sequentially or in combination; and group 4, anagrelide ( ANA ) and/or α ‐interferon ( IFN ) only. Patients from groups 1, 2, and 3 could also have been treated either with ANA and/or IFN in their medical history, considering these drugs not to have an additional cytotoxic potential. In all, 63 of the 1026 patients (6%) developed 64 SM during the follow‐up, after a median time of 50 months (range: 2–158) from diagnosis. In univariate analysis, a statistically significant difference was found only for gender ( P  = 0.035) and age ( P  = 0.0001). In multivariate analysis, a statistically significant difference was maintained for both gender and age (gender HR 1.7 [ CI 95% 1.037–2.818] P  = 0.035; age HR 4.190 [ CI 95% 2.308–7.607] P  = 0.0001). The impact of different treatments on SM s development was not statistically significant. In our series of 1026 ET patients, diagnosed and followed during a 30‐year period, the different therapies administered, comprising HU and ALK , do not appear to have impacted on the development of SM . A similar rate of SM s was observed also in untreated patients. The only two variables which showed a statistical significance were male gender and age 〉 60 years.
    Type of Medium: Online Resource
    ISSN: 2045-7634 , 2045-7634
    URL: Issue
    URL: Article
    DOI: 10.1002/cam4.2017.6.issue-6
    DOI: 10.1002/cam4.1081
    Language: English
    Publisher: Wiley
    Publication Date: 2017
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  • 8
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    Anagrelide in Essential Thrombocythemia (ET): Results from 150 patients over 25 years by the “Ph1‐negative Myeloproliferative Neoplasms Latium Group”
    Wiley ; 2020
    In:  European Journal of Haematology Vol. 105, No. 3 ( 2020-09), p. 335-343
    Details
    In: European Journal of Haematology, Wiley, Vol. 105, No. 3 ( 2020-09), p. 335-343
    Abstract: Anagrelide is a drug effective in reducing platelet counts in essential thrombocythemia (ET) and Ph1‐negative myeloproliferative neoplasms. The aim of this study was to evaluate the real‐life use of anagrelide in patients with ET followed over 25 years at the Haematological Institutes belonging to “Ph1‐negative Myeloproliferative Neoplasms Latium Group.” Patients and methods Eligibility criteria were diagnosis of ET and treatment with anagrelide. Data were collected through an ad hoc case report form. Results One hundred and fifty patients received anagrelide for a median time of 7.4 years (0.1‐23.2). Anagrelide was administered as first‐line therapy in 34.7% of patients, as second‐line in 52% and as third‐line in 13.3%: 85.4% responded to therapy. Sixty‐eight/136 evaluable patients reported side effects: palpitations, peripheral vasodilation, anaemia, diarrhoea and gastric distress. Fourteen thrombotic (arterial 10, venous 4) and 51 bleeding events (minor 48, major 3) occurred. Sixteen/150 (10.6%) patients developed secondary myelofibrosis and 3/150 (2%) an acute myeloid leukaemia. Conclusions In our experience, anagrelide is an effective drug in reducing platelet levels in a high percentage of patients with ET. It is especially addressed to younger people. A careful assessment of the thrombotic risk and monitoring of cardiac function, at diagnosis and during follow‐up, is mandatory.
    Type of Medium: Online Resource
    ISSN: 0902-4441 , 1600-0609
    URL: Issue
    URL: Article
    DOI: 10.1111/ejh.v105.3
    DOI: 10.1111/ejh.13454
    Language: English
    Publisher: Wiley
    Publication Date: 2020
    detail.hit.zdb_id: 2027114-1
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  • 9
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    Application of the International Prognostic Score of Thrombosis for Essential Thrombocytemia(ET) (IPSET-Thrombosis) in a Cohort of ET Patients: Experience from Gruppo Laziale for Myeloproliferative Ph Negative Neoplasms
    American Society of Hematology ; 2015
    In:  Blood Vol. 126, No. 23 ( 2015-12-03), p. 2821-2821
    Details
    In: Blood, American Society of Hematology, Vol. 126, No. 23 ( 2015-12-03), p. 2821-2821
    Abstract: INTRODUCTION: Essential Thrombocytemia (ET) is the most common of the myeloproliferative neoplasms, vascular complications contribute mostly to both morbidity and mortality. The ability to identify thrombotic risk of the individual patient is necessary for a correct therapeutic management. Traditionally, risk stratification for thrombosis in ET pts was based on the respective absence and/or presence of either age 〉 60 years or history of thrombosis. Recently, the IPSET score (International Prognostic Score Of Thrombosis for ET) was developed to better predict the occurrence of thrombotic events in ET patients. Risk factors included in the new score were: age, cardiovascular risk factors, previous thrombosis, presence of JAK 2 V617F mutation. AIM: to evaluate the validity of IPSET-thrombosis score in a cohort of ET patients from "Gruppo Laziale for Myeloproliferative Ph negative Neoplasms" in predicting thrombosis incidence. METHODS: from January 1978 to December 2011 we observed 1249 ET patients, median follow up was 105 months (range 12.1-417.7). We were able to retrospectively evaluate all the IPSET risk factors in 680 ET patients and estimated the clinical implication of the IPSET-thrombosis score system. According to the score 27.3 %, 19.1% and 53.5% of pts were stratified in low, intermediate and high risk group respectively. RESULTS: median age at the time of diagnosis was 61.8 yrs (range 19.9 -94; 64% females), median hemoglobin was 14 g/dl (range 6-20); median leukocyte count was 8.8 x 109/L (range 1,2-57); median platelets count was 812x 109/L (range 108-3582). We observed, during a median follow-up of 200 months, 82 thrombotic events (total incidence 17,89 %). According IPSET-thrombosis risk, in our ET population was documented a statistically different thrombosis free survival (TFS) (Gray test 0.1316): 85%, 78%, 77% in low,intermediate and high risk group respectively. During all the observation period the intermediate and high risk group showed a similar probability of thrombotic events. CONCLUSIONS: in our retrospective study the IPSET score was able to differentiate the rate of thrombosis events in low-risk (0-1 risk factors) from that of intermediate ( 〉 = 2 risk factors) and high risk ( 〉 = 3 risk factors) ET pts. Unfortunately the intermediate and high risk groups show a similar incidence of thrombotic events during the whole observation period. We were not able to verify the clinical benefit resulting from the different treatment strategies. Because treatment options in ET pts are tailored according to thrombotic risk, and since we have specified therapeutic indications for patients with low and high risk, it is necessary to validate the score IPSET in a large prospective, long term study to discriminate a true "intermediate" subset of patients for which there are no currently defined therapeutic guidelines. Disclosures No relevant conflicts of interest to declare.
    Type of Medium: Online Resource
    ISSN: 0006-4971 , 1528-0020
    URL: Article
    DOI: 10.1182/blood.V126.23.2821.2821
    RVK:
    XA 33000
    RVK:
    XA 10000
    Language: English
    Publisher: American Society of Hematology
    Publication Date: 2015
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  • 10
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    A Retrospective Epidemiological Analysis of 1572 Cases of Ph1- Myeloprolypherative Neoplasms (MPNs) From 9 Centers In the Latium: Preliminary Results
    American Society of Hematology ; 2010
    In:  Blood Vol. 116, No. 21 ( 2010-11-19), p. 5065-5065
    Details
    In: Blood, American Society of Hematology, Vol. 116, No. 21 ( 2010-11-19), p. 5065-5065
    Abstract: Abstract 5065 The Latium is a region of central Italy that counts approximatively 5.600.000 of residents. We reported herein a preliminary analysis of 1572 patients affected by MPN diagnosed in the last 20 years and treated in 9 of the Centers belonging to our group. Our centers are mainly located in Rome (7 centers) and neighboring districts (2 centers, Latina and Viterbo). The diagnosis was performed according to PVSG criteria until 2001 and then according to the WHO criteria: the majority of the patients underwent bone marrow biopsy. 218 patients were affected by primary myelofibrosis (PMF), 779 by Essential thrombocytemia (ET) and 575 by Policytemia Vera (PV). Epidemiological and clinical findings of all patients at diagnosis as well as thrombotic complications and evolution are reported in the table:ET (779 pts)PV (575 pts)PMF (218 pts)M/F288/491350/225134/84Age (yrs)59 (r: 20-93)60 (r: 19-91)67 (r: 30-86)WBC (x109/L)9192 (+/− 3483 SD)10423 (+/− SD 4307)13313 (+/−5220)Hb (g/dL)13, 9 (+/− 1,8)18,1 (+/− 2,3)11,7 (+/− 2,8)Plts (x 109/L)877 (+/− 353)445 (+/−247)457 (+/− 358)Splenomegaly (%)193579Bone marrow biopsy (performed/total))557/779 (71,5%)254/575 (44,2%)207/218 (95%)JAK-2 V617F mut (%)284/484 (59%)256/369 (69,4%)69/116 (59,5 %)Jak-2 allele burdenPerformed in 188/284 cases 26,49% (SD +/− 24)Performed in 199/256 cases: 59,4 % (SD +/−30,9)Performed in 51/69 cases 57,5% (SD +/− 29,4)Median Follow-up (years)7,157,863,89Thrombosis pre-diagnosis of MPNN. of patients (%)108/779 (13,8%)100/575 (17,4%)29/218 (13,3%)(arterial+venous)74+3476+2424+5Thrombosis post diagnosis of MPNN. of patients (%)71/779 (9,1%)66/575 (11,5%)20/218(9,17%)(arterial+venous)42+2942+2412+8EvolutionMyelofibrosis9/779 (1,1%)24/575 (4,1%)AML9/779 (1,1%)13/575 (2,3%)17/143 (11,8%) Comments: In our opinion, this casistic of patients with different types of MPNs reflects clinical presentation and evolution of three variants of the same disease. Many clinical findings in our unselected cohort of patients are similar to those reported in literature; in particular, thrombotic events were seen in about 13 – 17% of patients, without any correlation with Jak-2 status in both pre-diagnosis and follow-up. However, 2 main differences were noted: a lower incidence of JAK-2 V617F mutation among our PV patients and a lower rate of evolution in myelofibrosis and AML among our ET and PV patients. Both these features warrant further insights to be fully elucidated. Disclosures: No relevant conflicts of interest to declare.
    Type of Medium: Online Resource
    ISSN: 0006-4971 , 1528-0020
    URL: Article
    DOI: 10.1182/blood.V116.21.5065.5065
    RVK:
    XA 33000
    RVK:
    XA 10000
    Language: English
    Publisher: American Society of Hematology
    Publication Date: 2010
    detail.hit.zdb_id: 1468538-3
    detail.hit.zdb_id: 80069-7
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