Concurrent Diagnosis of Sjogren’s Syndrome and Polycthemia Vera: a Rare Co-Existence

Abstract

This case report explores the unprecedented concurrent diagnosis of Sjogren’s syndrome (pSS) and polycythemia vera (PV) in a 62-year-old woman presenting with erythrocytosis, classic pSS symptoms, and a positive JAK2-V617F mutation. While autoimmune diseases and hematological malignancies frequently coexist, the combination of PV and pSS has not been previously documented. Despite recurrent phlebotomies for PV, the patient experienced persistent symptoms and elevated inflammatory markers. The case underscores the complexity of managing rare coexisting conditions, emphasizing the importance of considering a broad spectrum of hematological malignancies in primary SS patients. The patient is currently receiving hydroxyurea and hydroxychloroquine with regular follow-ups, shedding light on the challenges associated with the treatment of such unique clinical scenarios. This report contributes to the medical literature by expanding our understanding of potential associations between autoimmune disorders and hematological malignancies beyond conventional patterns.