Preface: Gaucher disease and cancer.
نویسندگان
چکیده
In this special issue the relationship of Gaucher disease to malignancy is explored in depth. For many years, multiple reports have attempted to substantiate this relationship, but until recently, the reported studies have not been convincing. Rosenbloom and Weinreb present an up-to-date review on Gaucher disease so the reader will understand the clinical, pathophysiological, and molecular aspects of the illness as well as the available treatment options. With this background, the other authors in this issue have dissected specific aspects of the disease and ultimately, the relationship of Gaucher disease to cancer. Pandey and Grabowski extensively review the immunological aspects of Gaucher disease. Specifically, they untangle the effects of accumulated glucocerebroside on macrophage function and its potential for causing cancer. Cabot and his colleagues examine the impaired sphingolipid metabolism in Gaucher disease and how cancers may arise. Weinreb and Lee examine the causes of death in Gaucher disease before the era of enzyme replacement therapy. A clear-cut increase in the risk of certain cancers is noted. More specifically, Ayto and Hughes review the relationship of Gaucher disease with multiple myelomas by examining the possible causes of immune system dysfunction. Lastly, Mistry et al. very effectively synthesize a conceptual model of how an inborn error of metabolism can lead to macrophage dysfunction and immune dysregulation and ultimately the development of cancer.
منابع مشابه
Report of Four Children with Gaucher Disease and Review of Literature
Gaucher Disease (GD) is the most common type of Lysosomal Storage Disorder and it is divided into three distinct subtypes. The authors here report four different cases of Gaucher Disease, with varying clinical manifestations, and the diagnosis of each established by the low level of Beta-Glucosidase enzyme as well as genetic DNA testing. The study also highlights the importance of early diagnos...
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Gaucher disease is an inherited disorder caused by a deficiency in the lysosomal hydrolase glucocerebrosidase. There is a wide spectrum of clinical presentations, with the most common features being hepatosplenomegaly, skeletal disease, and cytopenia. Gaucher disease has been classified into three broad phenotypes based upon the presence or absence of neurological involvement: Type 1 (nonneuron...
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BACKGROUND Some, but not all, reports suggest that patients with Gaucher disease are at increased risk of developing malignancies, particularly hematopoietic tumors. The aim of this study was to assess the pattern of Gaucher disease and subsequent malignancies among male veterans admitted to US Veterans Affairs hospitals. METHODS Among 832 294 African American and 3 668 983 white male veteran...
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ورودعنوان ژورنال:
- Critical reviews in oncogenesis
دوره 18 3 شماره
صفحات -
تاریخ انتشار 2013