TY - JOUR
T1 - Incidence of malignancies among patients with type I Gaucher disease from a single referral clinic
AU - Zimran, Ari
AU - Liphshitz, Irena
AU - Barchana, Micha
AU - Abrahamov, Aya
AU - Elstein, Deborah
PY - 2005/5
Y1 - 2005/5
N2 - Background. It has been implied that the incidence of malignant disorders is increased in patients with non-neuronopathic (type I) Gaucher disease. The purpose of the study was to ascertain numbers of cancers in type I Gaucher disease since this is of considerable concern to patients and physicians. Methods. Records of 505 patients with type I Gaucher disease seen at a large referral clinic since 1990 were culled in December 2004 to ascertain diagnosis of a cancer identified during follow-up. Age-matched data from the Israeli National Cancer Registry (INCR) database were used for comparison. Findings. Patients diagnosed with cancer before 1990 were not included. Of the remaining 500 patients, 227 (45.4%) were male, mean age = 38.7 years; and 273 (54.6%) were female, mean age = 37.0 years (SD = 21.0 years for both). Twenty patients (4.0%) had developed a cancer through December 31, 2003: 6 were male and 14 were female. The most common were three cases each of lymphoma and myelodysplastic syndrome and two cases of multiple myeloma. There was no statistically significant excess of cancer rate among patients relative to age-matched rates reported in national Jewish Israeli and Ashkenazi Jewish Israeli registry records. Conclusions. There appears to be no excess risk for hematological or other cancers among patients with type I Gaucher disease relative to the overall Jewish population matched for age. This study confirms recent international studies of patients with Gaucher disease for no excess risk for all cancers but multiple myeloma where these latter studies implicate a significantly higher incidence.
AB - Background. It has been implied that the incidence of malignant disorders is increased in patients with non-neuronopathic (type I) Gaucher disease. The purpose of the study was to ascertain numbers of cancers in type I Gaucher disease since this is of considerable concern to patients and physicians. Methods. Records of 505 patients with type I Gaucher disease seen at a large referral clinic since 1990 were culled in December 2004 to ascertain diagnosis of a cancer identified during follow-up. Age-matched data from the Israeli National Cancer Registry (INCR) database were used for comparison. Findings. Patients diagnosed with cancer before 1990 were not included. Of the remaining 500 patients, 227 (45.4%) were male, mean age = 38.7 years; and 273 (54.6%) were female, mean age = 37.0 years (SD = 21.0 years for both). Twenty patients (4.0%) had developed a cancer through December 31, 2003: 6 were male and 14 were female. The most common were three cases each of lymphoma and myelodysplastic syndrome and two cases of multiple myeloma. There was no statistically significant excess of cancer rate among patients relative to age-matched rates reported in national Jewish Israeli and Ashkenazi Jewish Israeli registry records. Conclusions. There appears to be no excess risk for hematological or other cancers among patients with type I Gaucher disease relative to the overall Jewish population matched for age. This study confirms recent international studies of patients with Gaucher disease for no excess risk for all cancers but multiple myeloma where these latter studies implicate a significantly higher incidence.
KW - Cancer
KW - Gaucher disease
KW - Hematological cancers
KW - MGUS
KW - Multiple myeloma
UR - http://www.scopus.com/inward/record.url?scp=18844449947&partnerID=8YFLogxK
U2 - 10.1016/j.bcmd.2005.03.004
DO - 10.1016/j.bcmd.2005.03.004
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C2 - 15885601
AN - SCOPUS:18844449947
SN - 1079-9796
VL - 34
SP - 197
EP - 200
JO - Blood Cells, Molecules, and Diseases
JF - Blood Cells, Molecules, and Diseases
IS - 3
ER -