PB1332 - Neuropsychiatric Systemic Lupus Erythematosus with Concurrent Thrombotic Thrombocytopenic Purpura

Background: Concurrent presentation of thrombotic thrombocytopenic purpura (TTP) and systemic lupus erythematosus (SLE) is extremly rare. The incidence of TTP in SLE patients has been reported to be as low as 0.5%.

Aims: This case report described an adult male who presented with TTP-associated with neuropsychiatric SLE.

Methods: We report a case of 39-year-old male, previously well, presented with chills, dark colored urine, petechiae and easy bruising for 1 week duration's. His full blood count was Hb 8.2g/dL, WBC 7.2x109/L, Platelet 15x109/L, Retic 6.1% with elevated serum LDH 1149 U/L, indirect bilirubin 44umol/L, negative Coombs test. Subsequently in ward patient developed confusion which was diagnosed as meningoencephalitis. He was treated with broad spectrum antibiotics and methylprednisolone, and the patient responded well to the medication. However the patient's condition deteriorated in the second week, he became obtunded. Noted reducing Hb 6.5g/dL, WBC 6.5x109/L, Platelet 7x109/L, doubling of serum LDH 2749 U/L. The full blood picture showed features suggestive of microangiopathic hemolytic anemia and ADAMTS13 activity was 0.1%. The anti-nuclear antibody positive titer of 1:1000 with homogenous pattern. Thus, patient was diagnosed as neuropsychiatric SLE with TTP. Plasma exchange (PLEX) with IV Methylprednisolone was instigated. PLEX was stopped after the sixth cycle as patient condition improved and the platelet count recovered. Unfortunately, patient manifest as relapsed TTP with platelet drop to 38x109/L day 5 post PLEX . PLEX was re-initiate and Rituximab was added as part of management. Following treatment, the patient's platelet count and other hemolytic markers had returned to normal, indicating a clinical improvement.

Results: Diagnosing and managing TTP together with SLE can be challenging.

Conclusion(s): Crucial elements such as early diagnosis, searching for its association and aggressive treatment with PLEX and immunosuppression can be life saving.