Gaucher Disease and Cancer: Concept and Controversy
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 b...
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| Format: | Article |
| Language: | English |
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Wiley
2011-01-01
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| Series: | International Journal of Cell Biology |
| Online Access: | http://dx.doi.org/10.1155/2011/150450 |
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| _version_ | 1849409298563596288 |
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| author | Francis Y. M. Choy Tessa N. Campbell |
| author_facet | Francis Y. M. Choy Tessa N. Campbell |
| author_sort | Francis Y. M. Choy |
| collection | DOAJ |
| description | 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 (nonneuronopathic), Type 2 (acute neuronopathic), and Type 3 (subacute neuronopathic). The two main treatment options include enzyme replacement therapy and substrate reduction therapy. Recently, discussion has escalated around the association of Gaucher disease and cancer, with conflicting reports as to whether Gaucher patients have an increased risk of malignancy. In this review, we present both the concept and controversy surrounding the association of Gaucher disease with cancer. |
| format | Article |
| id | doaj-art-7e2d42bcf645453e9f1097a5cd3cf0f7 |
| institution | Kabale University |
| issn | 1687-8876 1687-8884 |
| language | English |
| publishDate | 2011-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | International Journal of Cell Biology |
| spelling | doaj-art-7e2d42bcf645453e9f1097a5cd3cf0f72025-08-20T03:35:33ZengWileyInternational Journal of Cell Biology1687-88761687-88842011-01-01201110.1155/2011/150450150450Gaucher Disease and Cancer: Concept and ControversyFrancis Y. M. Choy0Tessa N. Campbell1Department of Biology, Centre for Biomedical Research, University of Victoria, P.O. Box 3020, Station CSC, Victoria, BC, V8W 3N5, CanadaTNC Scientific Consulting, Calgary, AB, CanadaGaucher 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 (nonneuronopathic), Type 2 (acute neuronopathic), and Type 3 (subacute neuronopathic). The two main treatment options include enzyme replacement therapy and substrate reduction therapy. Recently, discussion has escalated around the association of Gaucher disease and cancer, with conflicting reports as to whether Gaucher patients have an increased risk of malignancy. In this review, we present both the concept and controversy surrounding the association of Gaucher disease with cancer.http://dx.doi.org/10.1155/2011/150450 |
| spellingShingle | Francis Y. M. Choy Tessa N. Campbell Gaucher Disease and Cancer: Concept and Controversy International Journal of Cell Biology |
| title | Gaucher Disease and Cancer: Concept and Controversy |
| title_full | Gaucher Disease and Cancer: Concept and Controversy |
| title_fullStr | Gaucher Disease and Cancer: Concept and Controversy |
| title_full_unstemmed | Gaucher Disease and Cancer: Concept and Controversy |
| title_short | Gaucher Disease and Cancer: Concept and Controversy |
| title_sort | gaucher disease and cancer concept and controversy |
| url | http://dx.doi.org/10.1155/2011/150450 |
| work_keys_str_mv | AT francisymchoy gaucherdiseaseandcancerconceptandcontroversy AT tessancampbell gaucherdiseaseandcancerconceptandcontroversy |