P2.245: Stroke in the Setting of Acute Autoimmune Hemolytic Anemia as a Complication of Underlying, Undiagnosed Systemic Lupus Erythematosus and Antiphospholipid Syndrome in a Pediatric Patient

Introduction: Systemic Lupus Erythematosus (SLE) is a rare, chronic autoimmune disease with a prevalence of 3.3 to 8.8 per 100,000. It can present with nonspecific symptoms such as fever, fatigue, weight loss, malar rash, and multisystem involvement. Hematologic complications of SLE include hypercoagulability and autoimmune hemolytic anemia (AIHA), both of which increase the risk of thrombosis. We present a rare case of stroke in a pediatric patient due to AIHA, as a complication of underlying, undiagnosed SLE and antiphospholipid syndrome (APS).

Case Description: Our patient is a 17-year-old female with no significant past medical history who presented with symptomatic anemia and recurrent viral infections. She was diagnosed with mixed-type AIHA and treated with high-dose steroids and conservative transfusions. Differential diagnoses included viral-induced AIHA versus an underlying autoimmune process triggering the AIHA. The patient showed clinical and laboratory improvement and the decision was made to discharge on oral steroids with close hematology follow-up. On the day of expected discharge, she developed acute stroke-like symptoms. Imaging revealed a left internal carotid artery (ICA) and middle cerebral artery (MCA) occlusion, necessitating thrombectomy.

Approximately 12 hours post-thrombectomy, the patient developed seizures that progressed to status epilepticus, requiring intubation. Repeat imaging showed multiple hemorrhages and cerebral swelling in the stroke-affected area. Further laboratory workup confirmed a diagnosis of SLE and APS. High-dose steroid therapy was restarted, and the patient was transferred to a pediatric rheumatology center for exchange transfusion.

Discussion: This case highlights a rare and severe initial presentation of AIHA in the setting of undiagnosed SLE and APS, resulting in a life-threatening thrombotic event. AIHA promotes a hypercoagulable state through hemolysis and inflammation, further increasing thrombotic risk. Our patient had mixed-type AIHA, an uncommon form that exacerbated her prothrombotic state. This, combined with underlying autoimmune diseases, significantly heightened her risk for stroke.

Anticoagulation was initially considered but deferred due to multiple factors, including a downtrending D-dimer and a negative lower-extremity venous Doppler. However, this decision was made before confirming the SLE and APS diagnoses. We argue that in patients with high clinical suspicion for an autoimmune disorder, earlier consideration of anticoagulation may be warranted.

Conclusion: Pediatric stroke is rare, with a prevalence of 1.2 to 13 per 100,000. However, lupus patients face a 20- to 50-fold increased thrombosis risk. This case underscores the need to consider anticoagulation in patients with AIHA and suspected autoimmune disease, even in the absence of overt thrombosis markers. Despite reassuring initial labs and imaging, our patient suffered a massive stroke due to her AIHA, undiagnosed SLE, and APS. A more proactive approach to anticoagulation in such cases may help mitigate catastrophic outcomes.