[Multicentric abdominal angiofollicular hyperplasia: a clinical variant with exclusive involvement of the liver and...

Three patients with a non-malignant lymphoproliferative disease involving liver and abdominal lymph nodes, without peripheral lymphadenopathy, are presented. They had systemic symptoms, fever and/or arthralgia, a biochemical pattern of liver obstruction and signs of B-cell immune hyperactivity (hypergammaglobulinaemia and different autoantibodies). Abdominal lymph node enlargement was found on laparotomy; the lymph node histologic pattern was that of angiofollicular hyperplasia (AFH), malignant lymphoma being discarded. Several stages of involvement were present in all cases, made up of small lymphocytes, plasma cells, and centrofollicular cells forming portal nodules with cholangiole infiltrates in one case which gave rise to a lymphoepithelial lesion. Steroid therapy was effective in all cases, but the symptoms and the liver obstruction relapsed in two patients after steroid suppression. Persistent functional anomalies of B and T lymphocytes were present in all cases. No evidence of viral disease was seen. These patients form a clinicopathological variant of multicentric AFH on whom malignant lymphoma could be excluded only by the histological study of abdominal lymph nodes. The persistence of lymphocytosis (although polyclonal and lacking cytogenetic alterations) may suggest the possible evolution into true and malignant lymphoma in any case.