SHM Converge Scientific Abstract ePoster Gallery-Navigating a Rare Complication: Thrombotic Thrombocytopenic Purpura in Systemic Lupus Erythematosus

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This case describes a 28-year-old woman with systemic lupus erythematosus, lupus nephritis, stage 4 chronic kidney disease, and chronic anemia who presented with worsening exertional dyspnea, easy bruising, and a presyncope episode. Although she was initially hemodynamically stable and lacked classic TTP features such as fever or neurologic symptoms, laboratory findings strongly suggested thrombotic microangiopathy: severe anemia (hemoglobin 6.7), profound thrombocytopenia (platelets 7), elevated reticulocyte count, bilirubin, and LDH, low haptoglobin, worsening creatinine, negative Coombs test, schistocytes on peripheral smear, and a negative DIC workup.<br /><br />Given concern for thrombotic thrombocytopenic purpura (TTP), treatment was started promptly with intravenous steroids and plasma exchange for five days. Hemolysis improved, and platelet counts stabilized. Subsequent testing showed low ADAMTS13 activity with elevated inhibitor levels, confirming TTP. Rituximab and caplacizumab were then initiated, and the patient improved enough to be discharged.<br /><br />The report emphasizes that TTP can occur as a rare complication of SLE, affecting fewer than 0.5% of patients, and may present without the classic pentad. Early recognition based on careful history, prior records, and laboratory interpretation is critical, because delayed treatment can be life-threatening.

Asset Subtitle

Navanita Biswas

Meta Tag

Author List Navanita Biswas, Pallab Sarker, Prakash Adhikari

Category Clinical Vignettes

Concept Thrombotic Thrombocytopenic Purpura

Concept Total Plasma Exchange

Concept Microangiopathic Hemolytic Anemia

Concept Thrombocytopenia

Concept ADAMTS13

Distinguished Non-Finalist

Presenter Organization Tower Health Reading Hospital

Presenting Author Navanita Biswas

Track Adult

Keywords

systemic lupus erythematosus

thrombotic thrombocytopenic purpura

lupus nephritis

thrombotic microangiopathy

ADAMTS13 deficiency

plasma exchange

rituximab

caplacizumab

hemolytic anemia

thrombocytopenia

Thrombotic Thrombocytopenic Purpura

Total Plasma Exchange

Microangiopathic Hemolytic Anemia

Thrombocytopenia

ADAMTS13