What type of lymphoma has a high incidence in patients with X-linked lymphoproliferative syndrome (XLP)?

In patients with X-linked lymphoproliferative syndrome (XLP), there is an increased risk for developing certain types of lymphomas due to the underlying genetic defect that affects the immune response, particularly to viral infections such as Epstein-Barr Virus (EBV). One specific type of lymphoma that has a high incidence in these patients is ileocecal B-cell lymphoma. This particular lymphoma often arises due to the abnormal immune response and the resultant uncontrolled activation of lymphoid tissue, particularly in the gastrointestinal tract.

XLP is characterized by a predisposition not only to lymphoproliferative disorders but also to autoimmune conditions. The ileocecal region is a common site for the development of lymphomas in this scenario. In contrast, while other types of lymphomas, such as Burkitt lymphoma and non-Hodgkin lymphoma, may also be observed in various contexts of immunosuppression or viral associations, the ileocecal B-cell lymphoma is more specifically linked to the distinct pathology and clinical presentation associated with XLP.