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      Essential Thrombocythemia in a 15-Year-Old Female: Presentation, Workup, and Treatment Considerations in the Pediatric Population

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      , MPAS, PA-C, , MPAS, PA-C
      Journal of the Advanced Practitioner in Oncology
      Harborside Press LLC

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          Abstract

          Essential thrombocythemia (ET) is a diagnosis most often seen in adults but can also present in children in rare cases. This article reviews the presentation, diagnosis, and treatment of ET in a 15-year-old female followed by a review of the literature regarding special considerations in the workup, diagnosis, treatment, and follow-up of ET in the pediatric population.

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          Most cited references16

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          The 2016 WHO classification and diagnostic criteria for myeloproliferative neoplasms: document summary and in-depth discussion

          The new edition of the 2016 World Health Organization (WHO) classification system for tumors of the hematopoietic and lymphoid tissues was published in September 2017. Under the category of myeloproliferative neoplasms (MPNs), the revised document includes seven subcategories: chronic myeloid leukemia, chronic neutrophilic leukemia, polycythemia vera (PV), primary myelofibrosis (PMF), essential thrombocythemia (ET), chronic eosinophilic leukemia-not otherwise specified and MPN, unclassifiable (MPN-U); of note, mastocytosis is no longer classified under the MPN category. In the current review, we focus on the diagnostic criteria for JAK2/CALR/MPL mutation-related MPNs: PV, ET, and PMF. In this regard, the 2016 changes were aimed at facilitating the distinction between masked PV and JAK2-mutated ET and between prefibrotic/early and overtly fibrotic PMF. In the current communication, we (i) provide practically useful resource tables and graphs on the new diagnostic criteria including outcome, (ii) elaborate on the rationale for the 2016 changes, (iii) discuss the complementary role of mutation screening, (iv) address ongoing controversies and propose solutions, (v) attend to the challenges of applying WHO criteria in routine clinical practice, and (vi) outline future directions from the perspectives of the clinical pathologist.
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            Pregnancy outcomes in myeloproliferative neoplasms: UK prospective cohort study

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              Ruxolitinib reduces JAK2 p.V617F allele burden in patients with polycythemia vera enrolled in the RESPONSE study

              In patients with polycythemia vera (PV), an elevated JAK2 p.V617F allele burden is associated with indicators of more severe disease (e.g., leukocytosis, splenomegaly, and increased thrombosis risk); however, correlations between allele burden reductions and clinical benefit in patients with PV have not been extensively evaluated in a randomized trial. This exploratory analysis from the multicenter, open-label, phase 3 Randomized Study of Efficacy and Safety in Polycythemia Vera With JAK Inhibitor INCB018424 Versus Best Supportive Care trial evaluated the long-term effect of ruxolitinib treatment on JAK2 p.V617F allele burden in patients with PV. Evaluable JAK2 p.V617F-positive patients randomized to ruxolitinib (n = 107) or best available therapy (BAT) who crossed over to ruxolitinib at week 32 (n = 97) had consistent JAK2 p.V617F allele burden reductions throughout the study. At all time points measured (up to weeks 208 [ruxolitinib-randomized] and 176 [ruxolitinib crossover]), mean changes from baseline over time in JAK2 p.V617F allele burden ranged from −12.2 to −40.0% (ruxolitinib-randomized) and −6.3 to −17.8% (ruxolitinib crossover). Complete or partial molecular response was observed in 3 patients (ruxolitinib-randomized, n = 2; ruxolitinib crossover, n = 1) and 54 patients (ruxolitinib-randomized, n = 33; ruxolitinib crossover, n = 20; BAT, n = 1), respectively. Among patients treated with interferon as BAT (n = 13), the mean maximal reduction in allele burden from baseline was 25.6% after crossover to ruxolitinib versus 6.6% before crossover. Collectively, the data from this exploratory analysis suggest that ruxolitinib treatment for up to 4 years provides progressive reductions in JAK2 p.V617F allele burden in patients with PV who are resistant to or intolerant of hydroxyurea. The relationship between allele burden changes and clinical outcomes in patients with PV remains unclear. Electronic supplementary material The online version of this article (doi:10.1007/s00277-017-2994-x) contains supplementary material, which is available to authorized users.
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                Author and article information

                Journal
                J Adv Pract Oncol
                J Adv Pract Oncol
                J Adv Pract Oncol
                JADPRO
                Journal of the Advanced Practitioner in Oncology
                Harborside Press LLC
                2150-0878
                2150-0886
                May 2021
                1 May 2021
                : 12
                : 4
                : 423-429
                Affiliations
                From Marietta College, Marietta, Ohio
                Author notes
                Correspondence to: Brad Pierce, MPAS, PA-C, Marietta College, 215 Fifth Street, Marietta, OH 45750. E-mail: brp001@ 123456marietta.edu
                Article
                2021.12.4.6
                10.6004/jadpro.2021.12.4.6
                8163254
                34123478
                0802abb1-0cb2-4e4e-bfd7-b35b288c6687
                © 2021 Harborside™

                This article is distributed under the terms of the Creative Commons Attribution Non-Commercial Non-Derivative License, which permits unrestricted non-commercial and non-derivative use, distribution, and reproduction in any medium, provided the original work is properly cited.

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