Sinus histiocytosis with massive lymphadenopathy: A spectrum of disease associated with immune dysfunction

Diane L. Maennle, Helen L. Grierson, David G. Gnarra, Dennis D. Weisenburger

Research output: Contribution to journalArticle

25 Citations (Scopus)

Abstract

A detailed immunologic study of three cases of sinus histiocytosis with massive lymphadenopathy (SHML) was performed to better characterize this rare disorder. One patient had prominent cervical lymphadenopathy that regressed spontaneously, whereas the other two patients had persistent cervical lymphadenopathy and recurrent infections. The first patient was otherwise healthy and had normal immunologic studies. One of the latter patients had a relative increase in blood B cells, a decreased level of serum immunoglobulin A (IgA), decreased blood lymphocyte mitogenic responses to multiple mitogens (37-42% of controls), and cutaneous anergy. The other patient with persistent disease also had a relative increase in blood B cells, polyclonal hypergammaglobulinemia, and circulating immune complexes, as well as decreased blood T cells and markedly decreased blood lymphocyte responses to mitogens (12-37% of controls). Immunohistochemical stains of the lymph nodes of the three patients revealed a characteristic phenotype for the sinus histiocytes: S-100 protein, 3/3; CD14 (Leu M3), 3/3; CD11c (Leu M5), 1/1; CD71 (OKT9), 3/3; CD4 (Leu 3a), 2/3; CD1a (OKT6), 1/3; alpha-1-antitrypsin, 3/3; alpha-1-antichymotrypsin, 3/3; CD35 (C3b), 1/1; CD11b (Mo1), 0/3; CD15 (Leu M1), 0/3; HLA-DR, 0/3; and lysozyme, 0/3. This phenotype suggests that the cells of SHML have features of both the Langerhans/interdigitating cell and mononuclear phagocyte lineages. Emperipolesis by the histiocytes of B cells, T cells, and natural killer cells was demonstrated by a double-staining technique. Our findings indicate that patients with SHML may have a variably expressed immunodeficiency that predisposes them to recurrent infections..

Original languageEnglish (US)
Pages (from-to)399-412
Number of pages14
JournalFetal and Pediatric Pathology
Volume11
Issue number3
DOIs
StatePublished - Jan 1 1991

Fingerprint

Sinus Histiocytosis
Blood Cells
B-Lymphocytes
Histiocytes
Mitogens
Emperipolesis
alpha 1-Antichymotrypsin
Lymphocytes
Hypergammaglobulinemia
T-Lymphocytes
Phenotype
alpha 1-Antitrypsin
Langerhans Cells
S100 Proteins
HLA-DR Antigens
Phagocytes
Muramidase
Infection
Antigen-Antibody Complex
Natural Killer Cells

Keywords

  • Emperipolesis
  • Histiocyte
  • Immune dysfunction
  • Lymphadenopathy
  • S-100 protein
  • Sinus histiocytosis

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Pathology and Forensic Medicine

Cite this

Sinus histiocytosis with massive lymphadenopathy : A spectrum of disease associated with immune dysfunction. / Maennle, Diane L.; Grierson, Helen L.; Gnarra, David G.; Weisenburger, Dennis D.

In: Fetal and Pediatric Pathology, Vol. 11, No. 3, 01.01.1991, p. 399-412.

Research output: Contribution to journalArticle

Maennle, Diane L. ; Grierson, Helen L. ; Gnarra, David G. ; Weisenburger, Dennis D. / Sinus histiocytosis with massive lymphadenopathy : A spectrum of disease associated with immune dysfunction. In: Fetal and Pediatric Pathology. 1991 ; Vol. 11, No. 3. pp. 399-412.
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