Immunoglobulin G4 (IgG4)-related Mikulicz's disease (MD) is an immune-mediated fibroinflammatory condition of unknown etiology characterized by the swelling of multiple exocrine glands including lacrimal, salivary, and parotid glands. 1,2 Immune thrombocytopenia is common in many autoimmune diseases. However, IgG4-related MD is rarely associated with immune thrombocytopenia and its pathogenic mechanism remains largely unclear. To our knowledge, only one case has been reported to date in the literature. In that case, the patient developed severe thrombocytopenia after autologous peripheral blood stem cell transplantation (PBSCT) for multiple myeloma (MM). 3 This novel study aims to report the case of a 48-year-old male patient suffering from MD with severe immune thrombocytopenia without hematological malignancies and review all reported cases of IgG4-related disease (IgG4-RD) with thrombocytopenia. CASE REPORT A 48-year-old male patient was admitted to our department with enlarged lacrimal and parotid glands associated with thrombocytopenia. He underwent resection for bilateral swelling of submandibular glands three years ago. The pathological examination revealed no tumor cells or focal lymphocytic infiltration. However, he repeatedly had enlarged lacrimal and parotid glands and dry mouth, without dry eye or arthralgia. He was then diagnosed with Sjögren syndrome (SS) and received anti-rheumatic treatment with hydroxychloroquine and Tripterygium wilfordii (a traditional Chinese medicine having definite effects on autoimmune and inflammatory diseases) for two years. However, the patient suffered from repeated recurrences of enlarged glands and presented with thrombocytopenia after two years. Physical examination revealed nontender bilateral swelling of lacrimal and ABSTRACT Mikulicz's disease (MD), an immunoglobulin G4 (IgG4)-related disease, is a fibrosis-associated inflammatory disease characterized by painless bilateral swelling of lacrimal and salivary glands. Although thrombocytopenia is associated with many rheumatologic disorders, it is rare in IgG4-related MD. This novel study aims to report a rare case of IgG4-related MD complicated with severe autoimmune thrombocytopenia without any hematological malignancies and review all cases of IgG4-related disease associated with thrombocytopenia in the literature.
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