IgG4-related disease and lymphocyte-variant hypereosinophilic syndrome: A comparative case series

Mollie N. Carruthers, Sujin Park, Graham W. Slack, Bakul I. Dalal, Brian F. Skinnider, David F. Schaeffer, Jan P. Dutz, Joanna K. Law, Fergal Donnellan, Vladimir Marquez, Michael Seidman, Patrick C. Wong, Andre Mattman, Luke Y.C. Chen

Research output: Contribution to journalArticlepeer-review

Abstract

Objective: To compare the clinical and laboratory features of IgG4-related disease (IgG4-RD) and lymphocyte-variant hypereosinophilic syndrome (L-HES), two rare diseases that often present with lymphadenopathy, gastrointestinal symptoms, eosinophilia, and elevated immunoglobulins/IgE. Method: Comparative case series of 31 patients with IgG4-RD and 13 patients with L-HES. Results: Peripheral blood eosinophilia was present in eight of 31 patients with IgG4-RD compared to 13 of 13 patients with L-HES (median eosinophils 0.4 vs 7.0 giga/L, P=.001) and 12 of 20 patients with IgG4-RD had increased serum IgE compared to eight of 13 patients with L-HES, P=.930. Twenty-seven of 30 patients with IgG4-RD had elevated serum IgG4 compared to five of 12 patients with L-HES (median IgG4 9.6 g/L vs 0.80 g/L, P=.002). Flow cytometry demonstrated an aberrant T-cell phenotype in 7 of 23 patients with IgG4-RD and 13 of 13 patients with L-HES (P<.001). T-cell clonality by PCR was positive in 12 of 23 patients with IgG4-RD vs 10 of 13 patients with L-HES (P=.143). Patients in both groups received corticosteroids as first-line therapy. For refractory disease in IgG4-RD, rituximab was the most common steroid-sparing agent, whereas in L-HES, it was pegylated interferon-α-2a. Conclusion: The overlapping features of these two diseases with divergent treatment options demonstrate the importance of familiarity with both entities to optimize diagnosis and treatment.

Original languageEnglish (US)
Pages (from-to)378-387
Number of pages10
JournalEuropean Journal of Haematology
Volume98
Issue number4
DOIs
StatePublished - Apr 1 2017

Keywords

  • IgG4
  • IgG4-related disease
  • eosinophil
  • hypereosinophilic syndrome
  • hypergammaglobulinemia

ASJC Scopus subject areas

  • Hematology

Fingerprint Dive into the research topics of 'IgG4-related disease and lymphocyte-variant hypereosinophilic syndrome: A comparative case series'. Together they form a unique fingerprint.

Cite this