Any digestive tract lymphoma in persons with Down syndrome? About a
theoretical risk and practical observation
Daniel Satgé, Laboratoire d'Anatomie pathologique, Centre
Hospitalier, 19 000 Tulle, France.
Annie J. Sasco, INSERM, U 593, team of Epidemiology for Cancer Prevention,
Bordeaux, F-33076 France ; Université Victor Segalen Bordeaux II,
Bordeaux, F-33076, France.
Marie-Odile Réthoré, Institut Jérôme Lejeune, 50 avenue Foch 75116 Paris,
France.
A recent article of Nancy L. Swigonski et al in Pediatrics (1)
evaluates the cost effectiveness of screening asymptomatic children with
Down syndrome (DS) for celiac disease (CD) in order to prevent lymphoma.
The authors conclude that this screening should not be recommended since
it is not cost effective and results in fewer quality adjusted life-years.
The study was based on the facts that 1) there is a clearly increased
prevalence of CD in persons with DS, and that 2) digestive non-Hodgkin's
lymphoma is a well known complication of celiac disease (2). In addition
it should be noted that helicobacter pylori infection is more commun in
the DS population than in the general one and is a risk factor for gastric
lymphoma.
Another reason strongly stands against this screening: to our knowledge no
case of digestive tract lymphoma in a person with DS has so far ever been
reported. It is suspected, but not well accepted, that globally lymphoma
could be slightly over-represented in DS. Barber and Spiers in 1964 and
Scholl et al in 1982 (cited in 3) found an excess of solid hematopoietic
tumors in children and adults with DS. Furthermore, the two studies of
Hermon et al in 2001 and of Hill et al in 2003 (cited in 1) indicate a
slightly elevated, but not statistically significant, standardized
mortality odds ratio (SMRO) at 2.08 and relative risk (RR) at 1.5 for non-
Hodgkin's lymphoma incidence in persons with DS. However, we could not
find a single case of digestive lymphoma in a review of lymphoma in DS
(3), and in a recent extensive review of 140 digestive tract neoplasms in
children and adults with DS (4). Moreover, we are not aware of any
unpublished case. Finally, one of us (M-O R) who has a 53-year clinical
experience of persons with DS, and who followed hundreds of such patients
has never seen such an occurrence. Thus, although there is a clear
theoretical risk for digestive tract lymphoma in DS, no case has been
documented.
We would be very interested to know if a reader of this letter would know
such a case. Waiting for this, it is clear that such a lack, although life
expectancy in DS has greatly progressed (5) allowing the onset of cancers
occurring mainly after 50 years of age, do not currently support the
screening of CD in persons with DS to prevent digestive lymphoma. Finally,
if no case is reported, we should consider the possibility that some
unknown biological mechanism could protect persons with DS against
digestive lymphoma.
Acknowledgments: A grant from the Fondation Jérôme Lejeune supports
the study of neoplasms in persons with Down syndrome.
References:
1. Swigonski NL, Kuhlenschmidt HL, Bull MJ, Corkins MR, Downs SM.
Screening for celiac disease in asymptomatic children with Down syndrome:
cost-effectiveness of preventing lymphoma. Pediatrics. 2006;118:594-602
2. Brousse N, Meijer JW. Malignant complications of coeliac disease.
Best Pract Res Clin Gastroenterol. 2005;19:401-12
3. Satgé D, Le Tourneau A, Verger JP et al. A case report of Down
syndrome and centroblastic lymphoma. Pathol Res Pract. 1996;192:1266-9
4. Satgé D, Sasco AJ, Vekemans MJJ, Portal M-L, Fléjou J-F. Aspects
of digestive tract tumors in Down syndrome: a literature review. Dig Dis
Sci (in press)
5. Yang Q, Rasmussen SA, Friedman JM. Mortality associated with
Down's syndrome in the USA from 1983 to 1997: a population-based study.
Lancet. 2002;359:1019-25
Conflict of Interest:
None declared
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