Intestinal malignant lymphoma in a child with familial celiac disease

A case of intestinal malignant lymphoma occurring in a 10-year-old North African girl with celiac disease is reported. Celiac disease was diagnosed at age 4. A gluten-free diet was started and was maintained for the next 6 years. Seven months after the patient returned to a normal diet, clinical symptoms and subtotal villous atrophy relapsed. Within the next month an abdominal malignant lymphoma was diagnosed. After chemotherapy the tumor disappeared but recurred 6 months later, involving the colon and the liver. Death occurred shortly thereafter. A brother, two years older, also with proven celiac disease, is presently on a strict gluten-free diet. Both children shared the same histocompatibility antigens (HLA A2-B17/A29-B12, identity at the D locus). Whether or not relapse of celiac disease played a role in the appearance of the tumor remains highly speculative; however, the case of our patient suggests that familial involvement and, in some instances, ethnic origin may constitute risk factors predisposing to malignancy.