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

Bassam N. Smir, Timothy Charles Greiner, Dennis D. Weisenburger

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Abstract

Multicentric angiofollicular lymph node hyperplsia (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. Histlogically, we observed the plasma cell variant of MAFH in five patients (62.5%) and the hyaline-vascular variant in three (31.5%). 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.

Original languageEnglish (US)
Pages (from-to)1135-1142
Number of pages8
JournalModern Pathology
Volume9
Issue number12
StatePublished - Dec 1 1996

Fingerprint

Giant Lymph Node Hyperplasia
Lymph Nodes
Plasma Cells
Hypergammaglobulinemia
Human Herpesvirus 8
Child Mortality
Immunoglobulin Genes
Hyalin
Hepatomegaly
Gene Rearrangement
Splenomegaly
Human Herpesvirus 4
In Situ Hybridization
Blood Vessels
Registries
Adrenal Cortex Hormones
Coloring Agents
RNA
Morbidity
Kidney

Keywords

  • Angiofollicular hyperplasia
  • Castle-man's disease
  • Children
  • Lymph node

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

Cite this

Multicentric angiofollicular lymph node hyperplasia in children : A clinicopathologic study of eight patients. / Smir, Bassam N.; Greiner, Timothy Charles; Weisenburger, Dennis D.

In: Modern Pathology, Vol. 9, No. 12, 01.12.1996, p. 1135-1142.

Research output: Contribution to journalArticle

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