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  • A review by Keung and colleagues summarized cases of

    2019-06-19

    A 2002 review by Keung and colleagues summarized cases of hematologic malignancy in patients with KS reported between 1961 and 2002 [3]. They described 12 cases of AML in KS in the literature during that time period. Based on the review of the current literature, we found approximately 10 additional cases of AML in patients with constitutional 47,XXY. Overall, this is a rather small number of reported cases over a period of greater than 5 decades, and studies to date have not definitively established an epidemiological link [3,6]. Intriguingly, almost half the cases of AML with KS occurred in the pediatric population (≤18 years old at diagnosis) [3,7,8]. No cases were diagnosed over the age of 64 [4,9,10]. This is noteworthy given that the median age of AML at diagnosis is above 65, and that the majority of AML cases are diagnosed in older patients. These observations raise the question of whether KS, like certain other constitutional abnormalities, may predispose to an earlier onset of AML.
    Authors׳ contributions
    Acknowledgments MMJ is supported by the Betty Lea Stone Fellowship from the American Cancer Society.
    We have erroneously used the untransformed values for hemoglobin in the article (). The figure states that the values are g/dL, but the values are mmol/L. The hemoglobin values should therefore be multiplied by a transformation factor of 1,61. Please see the new figure with transformed values.
    Introduction Acute myeloid leukemia (AML) is the most common acute leukemia in adults, with an incidence of 2.5 cases per 100,000 persons worldwide per year [1]. In AML a variety of genetic aberrations, often relevant for cellular differentiation and cell survival, lead to an accumulation of clonal, myeloid precursor cells in the bone marrow and ultimately to failure of hematopoiesis [1]. Acute promyelocytic leukemia (APL) is characterized by the PML-RARA onco-fusion protein that initiates the disease by promoting a block in myeloid differentiation and proliferation of the promyelocytic blasts [2]. APL patients are currently treated with all-trans 4-Hydroxytamoxifen retinoic 4-Hydroxytamoxifen (ATRA), which directly targets PML-RARA for degradation by activated caspases and the proteasome, thereby overcoming the differentiation block [2]. Interestingly, PML-RARA degradation by arsenic trioxide is triggered by sumoylation [3–5]. In an attempt to identify novel genes with a role in AML differentiation, we performed gene expression profiling experiments focusing on genes involved in cellular proliferation and survival including the sentrin-specific protease 5 (SENP5). SENP proteins are a family of proteins that remove small ubiquitin-like modifiers (SUMO׳s) from SUMOylated proteins, thereby altering protein function [6]. Recently, sumoylation was associated with resistance towards chemotherapy in AML [7], but a possible role in differentiation has not been studied yet. We found that SENP5 is downregulated in primary AML patient samples and its expression is induced upon ATRA-mediated neutrophil differentiation of primary APL cells and different AML cell lines. Moreover, impairing SENP5 expression in APL cells decreased ATRA-induced granulocytic differentiation.
    Materials and methods
    Results and discussion
    Authorship and disclosure
    Acknowledgments This project has been supported by grants from the Swiss National Science Foundation31003A_143739 (to M.P.T.) and 31003A_129702 (to M.F.F. and M.P.T.), the Marlies-Schwegler Foundation, the Ursula-Hecht-Foundation for Leukemia Research, the Bernese Foundation of Cancer Research (to MFF), the Werner and Hedy Berger-Janser Foundation of Cancer Research (to MFF and MPT), and the Bern University Research Foundation (to MPT). EAF is a recipient of fellowship from the Swiss National Science Foundation (PBBEP3_146108).
    Introduction Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired clonal stem cell disorder characterized by intravascular hemolysis and thrombophilia. Pregnancy in PNH is associated with increased maternal and fetal morbidity and mortality due to thromboembolism, infection and premature birth [1]. Therefore, PNH has been considered a relative contraindication for pregnancy. The terminal complement cascade inhibitor eculizumab has become the standard treatment in patients with symptomatic PNH. There are limited published data regarding the use of eculizumab in pregnancy in either animal studies or in humans. We report a patient with PNH who became pregnant while on eculizumab, required dose escalation during the third trimester to control breakthrough hemolysis, and successfully delivered a healthy baby.