Abstract
Myeloproliferative neoplasms (MPNs) are clonal disorders, derived from abnormal hematopoietic stem cells and result in an excessive production of blood cells. This MPN group of conditions ...encompasses different diseases with overlapping clinical and biologic similarities. The majority of the conventional therapies of MPN are palliative in nature. However, with the discovery of Janus Kinase 2 (JAK2) mutation and development of targeted JAK1/2 inhibition therapy, the therapeutic options in treatment landscape have changed dramatically. This article presents the revised Indian MPNs Working Group consensus recommendations. It highlights and brings into attention about the recent findings that have defined the state of the art of the diagnosis and therapy in the MPN area, including identification of the new driver and prognostic mutations, treatment goals in the management of myelofibrosis and polycythemia vera (PV), role of the recently approved, targeted tyrosine kinase inhibitor ruxolitinib in PV, and special issues such MPN consideration in patients with splenic vein thrombosis and the management of the disease in pregnancy.
On Sep 27, 2012, the NYC Department of Health and Mental Hygiene (DOHMH) alerted health-care providers and the public about 12 cases of invasive serogroup C Neisseria meningitidis disease occurring ...in NYC since Aug 2010 among men who have sex with men. Cases were identified through existing mandatory notifiable disease reporting and classified according to the Council of State and Territorial Epidemiologists case definitions. Among other things Weiss shares that DOHMH publicized this outbreak among the population at risk through advertising, mass e-mail messages on MSM websites, posters distributed at MSM bars and clubs, and outreach to community leaders and physician's groups.
E X E C U T I V E S U M M A R Y
According to the 2008 revision of the World Health Organization (WHO) classification of myeloid malignancies, philadelphia chromosome (Ph)-negative myeloproliferative ...neoplasms (MPNs) include clonal, hematologic disorders such as polycythemia vera, primary myelofibrosis, and essential thrombocythemia. Recent years have witnessed major advances in the understanding of the molecular pathophysiology of these rare subgroups of chronic, myeloproliferative disorders. Identification of somatic mutations in genes associated with pathogenesis and evolution of these myeloproliferative conditions (Janus Kinase 2; myeloproliferative leukemia virus gene; calreticulin) led to substantial changes in the international guidelines for diagnosis and treatment of Ph-negative MPN during the last few years. The MPN-Working Group (MPN-WG), a panel of hematologists with expertise in MPN diagnosis and treatment from various parts of India, examined applicability of this latest clinical and scientific evidence in the context of hematology practice in India. This manuscript summarizes the consensus recommendations formulated by the MPN-WG that can be followed as a guideline for management of patients with Ph-negative MPN in the context of clinical practice in India.
A significant proportion of implantable cardioverter-defibrillators (ICDs) have been subject to Food and Drug Administration (FDA) advisories. The impact of device advisories on mortality or patient ...care is poorly understood. Although estimated risks of ICD generators under advisory are low, dependency on ICD therapies to prevent sudden death justifies the assessment of long-term mortality.
To test the association of FDA advisory status with long-term mortality.
The study was a retrospective, single-center review of clinical outcomes, including device malfunctions, in patients from implantation to either explant or death. Patients with ICDs first implanted at Cleveland Clinic between August 1996 and May 2004 who became subject to FDA advisories on ICD generators were identified. Mortality was determined by using the Social Security Death Index.
In 1644 consecutive patients receiving first ICD implants, 704 (43%) became subject to an FDA advisory, of which 172 (10.5%) were class I and 532 (32.3%) were class II. ICDs were explanted before advisory notifications in 14.0% of class I and 10.1% of class II advisories. Among ICDs under advisory, 28 (4.0%) advisory-related and 15 non-advisory- related malfunctions were documented. Over a median follow-up of 70 months, 814 patients died. Kaplan-Meier 5-year survival rate was 65.6% overall, and 64.2, 61.1, and 69.3% in patients with no, class I, and class II advisories, respectively (P = .17).
ICD advisories impacted 43% of the patients. Advisory-related malfunctions affected 4% within the combined advisory group. Based on a conservative management strategy, ICDs under advisory were not associated with increased mortality over a background of significant disease-related mortality.
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