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This paper explores the management of ultra-early relapse of thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus.

May 20, 2026

B53-22 Ultra-Early Relapse of Systemic Lupus Erythematosus (SLE) -Associated Thrombotic Thrombocytopenic Purpura (TTP): A Call for Upfront Rituximab Therapy

Key Points

This paper explores the management of ultra-early relapse of thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus.
Case presentation of a 36-year-old female with SLE and class IV lupus nephritis exhibiting TTP symptoms.
Treatment included plasma exchange, corticosteroids, and upfront rituximab (375 mg/m² × 4 doses).
Evaluation of platelet recovery and laboratory outcomes post-treatment.
Initial treatment led to a platelet count of 170,000/μL after rituximab therapy.
Patient experienced rapid relapse within 5 days of discharge despite prior therapies.
Ultra-early relapse highlights refractoriness of plasma exchange and steroids in SLE-associated TTP.

Abstract

Abstract Introduction Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening thrombotic microangiopathy requiring prompt recognition and treatment. The incidence of acquired TTP ranges from 1.5 to 6 cases per million annually. Notably, 7-23% of TTP cases occur secondary to systemic lupus erythematosus (SLE). Here, we present a case of SLE-associated TTP with ultra-early relapse, highlighting the potential benefit of upfront B-cell depletion therapy in high-risk patients. Case Presentation A 36-year-old female with SLE and class IV lupus nephritis presented with pleuritic chest pain, abdominal pain, new-onset petechial rash, and heavy menstrual bleeding. Laboratory findings revealed severe thrombocytopenia (platelets 8,000/μL), hemolytic anemia (hemoglobin 9.4 g/dL), elevated PLASMIC score of 6, and severe ADAMTS13 deficiency (5%) with positive inhibitor. Notably, her SLE markers remained stable with normal complement levels. Despite initial response to seven plasma exchange sessions and corticosteroids, she experienced relapse within 5 days of discharge—among the earliest documented in literature. In response, treatment with rituximab (375 mg/m² × 4 doses) achieved sustained remission. The patient responded well to treatment with sustained platelet recovery to 170,000/μL. Conclusion This case illustrates an ultra-early relapse of SLE-associated TTP, supporting existing literature suggesting refractoriness of SLE-associated TTP to plasma exchange and steroids alone. Based on this experience, we propose that patients with SLE-associated TTP, particularly those with high-risk features including renal involvement (Class IV/V nephritis), very low ADAMTS13 activity (10%), or early relapse, should be considered for upfront rituximab therapy rather than reserving it for refractory cases. Early intervention in high-risk patients could help prevent relapse and achieve sustained remission. This abstract is funded by: None

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