Granulocyte-colony-stimulating Factor-resistant Neutropenia and Polyneuropathy Presenting as Severe Complications of Sjögren's Syndrome

Autor: Katsuyuki, Tomita, Shinnosuke, Tokugawa, Genki, Inui, Shotaro, Moriyasu, Tomoyuki, Ikeuchi, Hirokazu, Touge, Fuminari, Tajima
Rok vydání: 2022
Předmět:
Zdroj: Internal Medicine. 61:3287-3291
ISSN: 1349-7235
0918-2918
DOI: 10.2169/internalmedicine.9092-21
Popis: Primary Sjögren's syndrome (pSS) has multi-dimensional manifestations, including neutropenia and polyneuropathy. We herein report a 76-year-old woman with pSS initially presenting as severe granulocyte-colony-stimulating factor (G-CSF)-refractory neutropenia and axonal sensorimotor polyneuropathies (SMP). Systemic glucocorticoid administration had reduced neutrophil-associated immunoglobulin G (NAIgG) on the neutrophil surface as detected using flow cytometry, resulting in the development of neutropenia. A patient with pSS concomitant with axonal SMP might show severe neutropenia as aggressive autoimmune disease. Neutropenia can be treated with systemic glucocorticoids based on the assessment of NAIgG on the neutrophil surface.
Databáze: OpenAIRE