Successful treatment with intravenous immunoglobulin of severe thrombocytopenia complicated in primary Sjögren's syndrome.
Sjogren's syndrome (SS) is a systemic autoimmune disease characterized by xerophthalmia, xerostomia and extraglandular manifestations. Anemia, leukopenia, thrombocytopenia and lymphoproliferative disorders, including lymphoma are well-known extraglandular, hematological complications of SS. We report here a rare case of patient with primary SS who developed pancytopenia with severe thrombocytopenia as an initial manifestation and successfully treated with IV immunoglobulin (IVIG). The present case suggests that pancytopenia with severe thrombocytopenia can be a difficult-to-treat abnormality, and initial manifestation of primary SS and IVIG might be an effective treatment for severe thrombocytopenia refractory to high-dose steroid in primary SS.
Division of Rheumatology, Department of Internal Medicine, Chonbuk National University Medical School and Research Institute of Clinical Medicine, San 2-20 Geumam-dong, Deokjin-gu, Jeonju, Jeonbuk 561-756, South Korea.
Bum-Su
BS
Choung
Wan-Hee
WH
Yoo
D000328:Adult; D004351:Drug Resistance; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007155:Immunologic Factors / Q000627:therapeutic use*; D010198:Pancytopenia / Q000209:etiology / Q000628:therapy; D012720:Severity of Illness Index; D012859:Sjogren's Syndrome / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology; D013921:Thrombocytopenia / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology / Q000276:immunology; D016896:Treatment Outcome
D005938:Glucocorticoids; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors