Incidence of malignancies among patients with type I Gaucher disease from a single referral clinic.
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.


RESULTS
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.
Zimran
Ari
A
Gaucher Clinic, Shaare Zedek Medical Center, P.O. Box 3235, Jerusalem 91031, Israel.
Liphshitz
Irena
I
Barchana
Micha
M
Abrahamov
Aya
A
Elstein
Deborah
D
D000293:Adolescent; D000328:Adult; D005260:Female; D005776:Gaucher Disease / Q000150:complications* / Q000453:epidemiology; D006801:Humans; D015994:Incidence; D007557:Israel / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D009101:Multiple Myeloma / Q000209:etiology; D009369:Neoplasms / Q000453:epidemiology / Q000209:etiology*; D012189:Retrospective Studies; D017678:Sex Distribution; D012737:Sex Factors