Annals of hematology
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Annals of hematology · Jan 2016
Idiopathic erythrocytosis: a study of a large cohort with a long follow-up.
Idiopathic erythrocytosis (IE) is an absolute erythrocytosis with no known cause, diagnosed by exclusion of primary and secondary erythrocytosis. Familial erythrocytosis (FE) is a rare disease and as the rare patients with JAK2-wild-type polycythemia vera (PV) may be misdiagnosed as IE. We compared 78 patients with IE, 21 with FE and 136 with PV in the effort to identify simple features capable of discriminating between them. ⋯ Phlebotomies to obtain a haematocrit lower than 45 % induce platelet count increase in 70 % of PV but not in IE. Mainly in men, normal spleen, normal platelet counts and no history of thrombosis at diagnosis argue against PV; diagnosis of IE could be supported by means of a cycle of venesection to see how it affects their platelet count. No simple data capable of distinguishing between IE and FE were identified; therefore, a case of sporadic erythrocytosis in a young patient should be investigated as a possible genetic cause.
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Annals of hematology · Jan 2016
Pain management trend of vaso-occulsive crisis (VOC) at a community hospital emergency department (ED) for patients with sickle cell disease.
Pain management at the emergency department (ED) for vaso-occulsive crisis (VOC) for patients with sickle cell disease has not been optimum, with a long delay in giving the initial analgesic. We conducted a retrospective survey over a 7-year period to determine our ED's timing in giving pain medication to patients with VOC as a quality improvement project. We compared different periods, children vs adults, and the influence of gender in the analgesic administration timing. ⋯ Delay in administration of the first analgesic was more pronounced for female adult patients than male adult patients in spite of their higher pain score. Health care providers working in ED should make conscious efforts to respect pain in women as well as pain in men. Though not proven from this study, we believe that a significantly wider use of hydroxyurea by adult patients most likely would reduce their utilization of ED for the purpose of relief of pain, and further pediatric hematologists may be better positioned to increase hydroxyurea adherence by young adult patients, since they have had established rapport with them before transitioning to adult care.
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Annals of hematology · Dec 2015
Multicenter StudyPhase II study of bendamustine combined with rituximab in relapsed/refractory mantle cell lymphoma: efficacy, tolerability, and safety findings.
In most cases of relapsed/refractory mantle cell lymphoma (MCL), patients respond to salvage therapy, though typically responses are partial and/or transient followed by disease progression, even with newer agents (e.g., ibrutinib). In this multicenter, open-label, single-arm, phase II study, patients with relapsed/refractory non-blastoid MCL received bendamustine 90 mg/m(2) (days 1 and 2) and rituximab 375 mg/m(2) (day 1) for 6 planned 28-day cycles. Functional imaging with 18-fluoro-2-deoxy-D-glucose positron emission tomography/computed tomography (18F-FDG PET/CT) was conducted at baseline and after cycle 6. ⋯ Main non-hematologic adverse events were nausea (69 %), fatigue (56 %), decreased appetite (42 %), constipation (38 %), diarrhea (36 %), vomiting (36 %), and decreased weight (31 %). Grade 3/4 neutropenia and lymphopenia occurred in 44 and 89 % of patients, respectively. ORR and CR rate compared favorably with single-agent ibrutinib (ORR, 67 %; CR, 23 %); bendamustine-rituximab is an effective therapy with manageable toxicity in relapsed/refractory MCL.
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Annals of hematology · Dec 2015
Randomized Controlled TrialEfficacy and safety of the thrombopoietin receptor agonist romiplostim in patients aged ≥ 65 years with immune thrombocytopenia.
Thrombopoietin receptor agonists increase platelet counts and reduce bleeding risk in patients with immune thrombocytopenia (ITP). Studies have reported that these agents may represent a risk factor for thromboembolic events, especially in the elderly, who are at increased risk for such complications relative to younger patients. In this retrospective analysis, efficacy and safety data for romiplostim in patients with ITP aged ≥65 years versus those aged <65 years are described. ⋯ Duration-adjusted AE rates were similar for romiplostim versus placebo/SOC in older and younger patients. The risks for grade ≥ 3 bleeding (RR 1.92; 95% CI, 0.47-7.95) and thromboembolic events (RR 3.85; 95% CI, 0.53-27.96) were numerically but not significantly higher for romiplostim versus placebo/SOC in patients ≥ 65 years. Romiplostim is effective and, with the exception of nonsignificant trends showing increased risks of grade ≥ 3 bleeding and thromboembolic events (a trend observed in other studies), generally well tolerated in older patients with ITP.
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Annals of hematology · Sep 2015
Clinical TrialPrediction of central venous catheter-related bloodstream infections (CRBSIs) in patients with haematologic malignancies using a modified Infection Probability Score (mIPS).
The aim of this study was to predict the probability of central venous catheter-related bloodstream infections (CRBSIs) in patients with haematologic malignancies using a modified version of the Infection Probability Score (mIPS). In order to perform a prospective, mono-centric surveillance of complications in clinical routine due to short-term central venous catheters (CVCs) in consecutive patients receiving chemotherapy from March 2013 to September 2014, IPS was calculated at CVC insertion and removal (mIPSin and mIPSex, respectively). We used the 2012 Infectious Diseases Working Party of the German Society of Haematology and Medical Oncology (AGIHO/DGHO) criteria to define CRBSI. ⋯ For patients with an mIPSex ≥8, the risk for a CRBSI was high (odds ratio [OR] = 5.9; p < 0.001) and even increased if, additionally, CVC had been in use for about 10 days (OR = 9.8; p < 0.001). In case other causes of infection are excluded, a mIPSex ≥8 and duration of CVC use of about 10 days predict a very high risk of CRBSI. Patients with a mIPSex <8 have a low risk of CRBSI of 8 %.