Mollie N Carruthers1, Sujin Park2, Graham W Slack3,4, Bakul I Dalal5, Brian F Skinnider3,4, David F Schaeffer3, Jan P Dutz6, Joanna K Law7, Fergal Donnellan8, Vladimir Marquez8, Michael Seidman9, Patrick C Wong10, Andre Mattman11, Luke Y C Chen2. 1. Division of Rheumatology, Department of Medicine, University of British Columbia, Vancouver, BC, Canada. 2. Division of Hematology, Department of Medicine, University of British Columbia, Vancouver, BC, Canada. 3. Department of Pathology and Laboratory Medicine, University of British Columbia, Vancouver, BC, Canada. 4. Department of Pathology, Centre for Lymphoid Cancer, British Columbia Cancer Agency, Vancouver, BC, Canada. 5. Division of Laboratory Hematology, Vancouver General Hospital, Vancouver, BC, Canada. 6. Department of Dermatology and Skin Science, University of British Columbia, Vancouver, BC, Canada. 7. Division of Gastroenterology and Hepatology, Department of Medicine, Johns Hopkins Hospital, Baltimore, MD, USA. 8. Division of Gastroenterology, Department of Medicine, University of British Columbia, Vancouver, BC, Canada. 9. Department of Pathology and Laboratory Medicine, Providence Healthcare, Vancouver, BC, Canada. 10. Department of Pathology and Laboratory Medicine, Richmond Hospital, Richmond, BC, Canada. 11. Adult Metabolic Disease Clinic, Vancouver General Hospital, Vancouver, BC, Canada.
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.
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.
Authors: Jiachen Liu; Wei Yin; Lisa S Westerberg; Pamela Lee; Quan Gong; Yan Chen; Lingli Dong; Chaohong Liu Journal: Front Immunol Date: 2021-09-01 Impact factor: 7.561
Authors: Nivaz Brar; Michael A Spinner; Matthew C Baker; Ranjana H Advani; Yasodha Natkunam; David B Lewis; Oscar Silva Journal: Haematologica Date: 2022-01-01 Impact factor: 9.941