MULTICENTRIC ANGIOFOLLICULAR LYMPH-NODE HYPERPLASIA IN CHILDREN - A CLINICOPATHOLOGICAL STUDY OF 8 PATIENTS

Multicentric angiofollicular lymph node hyperplasia (MAFH) is an idiop athic 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 Registr y at our institution. The ages of the patients ranged from two to 17 y ears (median, 10 yr), and the male-to-female ratio was 1:3. The patien ts presented with constitutional symptoms, multifocal lymphadenopathy, hepatomegaly, and/or splenomegaly, The laboratory findings included p eripheral blood cytopenias, polyclonal hypergammaglobulinemia, and ren al and hepatic dysfunction, Histologically, we observed the plasma cel l variant of MAFH in five patients (62.5%) and the hyaline-vascular va riant in three (37.5%). Immunohistochemical stains revealed a polyclon al plasma cell population in all cases. Two of six specimens were posi tive for Epstein-Barr virus by RNA in situ hybridization. A clonal imm unoglobulin heavy gene rearrangement was identified in one of the five specimens studied, but this had no apparent impact on the clinical co urse of the disease. None of the four specimens analyzed for the prese nce of Kaposi's sarcoma-associated herpesvirus was positive. Most pati ents were stable or free of disease after treatment, which included co rticosteroids in six of the eight patients. We concluded that the clin ical and pathologic features of MAFH in children are similar to those of adults, but MAFH seems to have a more favorable clinical course, Le ., low morbidity and mortality, in children.