Severe Thrombocytopenia: An Uncommon Presentation of Primary Sjögren’s Syndrome

Background: Primary Sjögren's syndrome (pSS) is a chronic autoimmune disorder typically presenting with dry eyes and mouth. Hematological abnormalities are common, but severe thrombocytopenia is rare and poses diagnostic challenges. Case Report: A 50-year-old female with chronic eye dryness post-LASIK presented with acute petechial rash, ecchymosis, minimal epistaxis, and persistent menorrhagia. She also reported occasional dysphagia, chronic heartburn, and pain in her neck, back, and knees. Examination showed no significant findings apart from peticheal rash. Laboratory tests revealed microcytic, hypochromic anemia and severe thrombocytopenia (3,000/mm³). Immunological tests were positive for ANA, ENAs, anti-SSA, and anti-SSB, suggesting pSS. A Schirmer test and conjunctival fluorescein staining supported this. Bone marrow biopsy and flow cytometry ruled out other hematological disorders. Treatment with methylprednisolone and IVIg, followed by oral prednisolone, improved hemoglobin and platelet counts. At discharge, platelet count was 160,000/mm³, maintaining around 200,000/mm³ after three months. Conclusion: Severe thrombocytopenia can be an initial manifestation of pSS, even without typical exocrine gland symptoms. Recognizing this can lead to earlier diagnosis and treatment, improving outcomes in pSS-related thrombocytopenia.