Treatment with Plasma Exchange Followed By Plasma Infusion for Thrombotic Thrombocytopenic Purpura Associated with SjöGren's Syndrome

Abstract: Thrombotic thrombocytopenic purpura (TTP) is rarely associated with Sjögren's syndrome (SS) with only 12 previously reported cases in the literature. All of them were women. A case of a previously healthy 64-year-old Chinese male presenting with microangiopathic hemolytic anemia, otherwise unexplained thrombocytopenia, and central nervous system involvement is reported. At the beginning the patient's family refused plasma exchange (PE) therapy due to cost consideration, later plasma infusion (PI) was initiated along with methylprednisolone pulse therapy. Significant clinical and laboratory response was achieved. However, recurrence happened after discontinuing the treatment for only three weeks without consulting his doctor. Then the patient received another two cycles of therapy of PE with again an excellent clinical and laboratory response. The patient was discharged in a state of remission on methylprednisolone and thymopolypeptides. In order to prevent further complications, prophylactic infusions of plasma was performed per month. The patient remained asymptomatic thereafter. Autoimmune workup revealed positive antinuclear antibodies and Anti-SSA. Lip biopsy revealed findings consistent with SS. TTP with SS tends to be recurrence. PE along with immunosuppressive therapy followed by prophylactic PI can prevent long-term organ complications in these cases.