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IgG4‐related disease and lymphocyte‐variant hypereosinophilic syndrome: A comparative case series
Author(s) -
Carruthers Mollie N.,
Park Sujin,
Slack Graham W.,
Dalal Bakul I.,
Skinnider Brian F.,
Schaeffer David F.,
Dutz Jan P.,
Law Joanna K.,
Donnellan Fergal,
Marquez Vladimir,
Seidman Michael,
Wong Patrick C.,
Mattman Andre,
Chen Luke Y.C.
Publication year - 2017
Publication title -
european journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0902-4441
DOI - 10.1111/ejh.12842
Subject(s) - medicine , hypereosinophilic syndrome , eosinophilia , igg4 related disease , immunoglobulin e , gastroenterology , rituximab , immunology , antibody , disease , lymphoma , pathology
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.