Ruskoné-Fourmestraux A, Delmer A, Lavergne A, Molina T, Brousse N, Audouin J, Rambaud J C
Department of Gastroenterology, Hôtel-Dieu, Paris, France.
Gastroenterology. 1997 Jan;112(1):7-16. doi: 10.1016/s0016-5085(97)70212-9.
BACKGROUND & AIMS: Multiple lymphomatous polyposis (MLP) is an uncommon type of gastrointestinal lymphoma. The aim of this study was to analyze the clinicopathologic features and outcome of a large series of patients.
From 1984 to 1995, 31 patients were enrolled prospectively. Exhaustive staging and immunohistochemical analysis were performed. The first 10 patients were treated with cyclophosphamide-vincristine-prednisolone (COP) and the subsequent 21 with doxorubicin-teniposide-cyclophosphamide-prednisolone (AVmCP). Eleven patients were treated with high-dose radiochemotherapy and stem cell autotransplantation because of partial response or relapse.
Advanced age and multiple polyps involving several gastrointestinal segments characterized initial clinical presentation. The typical tumor cell phenotype (pan-B+, CD5+, and CD10-) along with Bcl-1 rearrangement indicated that MLP is the gastrointestinal counterpart of mantle cell lymphoma. After COP treatment, only 3 patients achieved partial remissions, whereas 80% of patients after AVmCP treatment achieved complete or good partial remission. High-dose radiochemotherapy changed partial into complete remission in 80% of cases. None of the initial 10 patients survived for 3 years, but the expected 5-year survival rate was 59% in patients receiving the anthracycline-containing regimen (P < 0.0001).
MLP is a distinct entity among gastrointestinal lymphomas. An anthracycline-containing multidrug regimen and high-dose radiochemotherapy supported by autotransplantation seem to improve its poor prognosis.
