La maladie de Gaucher est associée à un risque accru de myélome multiple

Il est communément suggéré que les patients atteints de la maladie de Gaucher présentent un risque plus élevé de développer des cancers, probablement en raison d’une stimulation chronique du système immunitaire et d’une lymphoprolifération associée au stockage des glucocérébrosides dans les macrophages. Cependant, un nombre insuffisant de patients et plusieurs biais ont faussé les études antérieures évaluant cette hypothèse. Une équipe américaine a donc étudié l’incidence des cancers chez 2742 malades issus du registre international de la maladie de Gaucher. Le nombre de cancers observé chez ces patients était comparable à celui de la population américaine (risque relatif global de 0,79), excepté pour le myélome multiple pour lequel le risque relatif était significativement plus élevé (5,9). Les médecins en charge de patients atteints de la maladie de Gaucher devraient connaître ces données et les prendre compte dans leurs suivis, en concluent Rosenbloom et coll. 

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Blood ; 105(12) : 4569-4572 ; 15 juin 2005

http://www.orpha.net/actor/Orphanews/2005/050621.html#3249

Blood. 2005 Jun 15;105(12):4569-72. Epub 2005 Feb 17
Gaucher disease and cancer incidence: a study from the Gaucher Registry.

Rosenbloom BE,

Weinreb

NJ

, Zimran A, Kacena KA, Charrow J, Ward E.

Tower Oncology,

9090 Wilshire Blvd, Ste 200

,

Beverly Hills

,

CA

90211

. rosenbloom@toweroncology.com.

Patients with Gaucher disease (GD) are alleged to be at an increased risk of malignant disorders, possibly due to potential chronic stimulation of the immune system and lymphoproliferation associated with storage of glucocerebroside in tissue macrophages. Because previous reports of increased risk of malignancy in GD may have been affected by small patient numbers and ascertainment bias, 2742 patients with GD from the International Gaucher Registry were studied. The number of cancers identified among patients in the registry was compared with that expected in the

US

population of similar attained age and sex. The majority of patients were young or middle-aged adults at the time of last follow-up, with only 14% older than age 60. There were 10 patients with multiple myeloma, yielding an estimated relative risk of 5.9 (95% confidence interval [95% CI]: 2.8, 10.8). The relative risk of cancer overall was 0.79 (95% CI: 0.67, 0.94), and the subgroups for cancers of the breast, prostate, colon and rectum, lung, and hematologic malignancies other than myeloma did not yield statistically significant higher risks. This study suggests that, in general, patients with Gaucher disease are not at highly increased risk of cancer, at least during early and middle age. However, there appears to be a significantly higher risk of multiple myeloma of which physicians should be aware when caring for these patients. (Blood. 2005;105:4569-4572).

PMID: 15718419 [PubMed - in process]

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