Multicentric angiofollicular lymph node hyperplasia in children: a clinicopathologic study of eight patients.

Important note: Information in this article was accurate in 1997. The state of the art may have changed since the publication date.

Multicentric angiofollicular lymph node hyperplasia in children: a clinicopathologic study of eight patients.

Mod Pathol. 1996 Dec;9(12):1135-42. Unique Identifier : AIDSLINE MED/97127721
Smir BN; Greiner TC; Weisenburger DD; Department of Pathology and Microbiology, University of Nebraska; Medical Center, Omaha 68198-3135, USA.

Abstract: Multicentric angiofollicular lymph node hyperplasia (MAFH) is an idiopathic systemic disorder that has been reported only rarely in children. Therefore, we reviewed the clinical and pathologic features of eight patients listed in the Angiofollicular Lymph Node Hyperplasia Registry at our institution. The ages of the patients ranged from two to 17 years (median, 10 yr), and the male-to-female ratio was 1:3. The patients presented with constitutional symptoms, multifocal lymphadenopathy, hepatomegaly, and/or splenomegaly. The laboratory findings included peripheral blood cytopenias, polyclonal hypergammaglobulinemia, and renal and hepatic dysfunction. Histologically, we observed the plasma cell variant of MAFH in five patients (62.5%) and the hyaline-vascular variant in three 37.5%). Immunohistochemical stains revealed a polyclonal plasma cell population in all cases. Two of six specimens were positive for Epstein-Barr virus by RNA in situ hybridization. A clonal immunoglobulin heavy gene rearrangement was identified in one of the five specimens studied, but this had no apparent impact on the clinical course of the disease. None of the four specimens analyzed for the presence of Kaposi's sarcoma-associated herpesvirus was positive. Most patients were stable or free of disease after treatment, which included corticosteroids in six of the eight patients. We concluded that the clinical and pathologic features of MAFH in children are similar to those of adults, but MAFH seems to have a more favorable clinical course, i.e., low morbidity and mortality, in children.

Keywords: *Giant Lymph Node Hyperplasia/PATHOLOGY
970530
M9751376

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