P1.016: Beyond Platelets: A Case of Severe ITP Complicated by Intracranial Hemorrhage and Autoimmune Red Flags

Introduction: Immune Thrombocytopenic Purpura (ITP) is an acquired autoimmune disorder causing isolated thrombocytopenia. Most pediatric cases are self-limiting. Severe complications like intracranial hemorrhage (ICH) occur rarely, though are potentially fatal. We present a case of a 12-year-old with severe ITP complicated by ICH, hypocomplementemia, and positive ANA, underscoring the need for early autoimmune screening in rare presentations with prompt neurological and airway management.

Case Description: A previously healthy 12-year-old girl presented with severe ITP, manifesting as prolonged epistaxis and petechial rash. Shortly after IVIG treatment, she developed acute neurological deterioration. Imaging revealed a cerebellar hemorrhage with hydrocephalus, requiring emergent intubation, EVD placement, and hematoma evacuation. Further workup showed hypocomplementemia and positive ANA, raising concerns for an underlying autoimmune disorder. High-dose steroids and romiplostim were initiated, along with hydroxychloroquine, per rheumatology recommendations. Her course was complicated by vocal cord paralysis and stridor, necessitating re-intubation. She also developed sepsis from Acinetobacter, which was successfully treated. During recovery, the patient received seizure prophylaxis, nutritional support, and multidisciplinary rehabilitation for motor and swallowing deficits. She later developed a pseudo meningocele, managed conservatively. At discharge, the patient had normalized platelet counts on romiplostim with long-term monitoring for hematologic, neurologic, and autoimmune sequelae.

Discussion: ICH is a rare but devastating complication of ITP, primarily caused by severe thrombocytopenia and impaired platelet-mediated hemostasis. Additional factors, including endothelial dysfunction and coexisting autoimmune disorders, may further elevate bleeding risk. In this case, hypocomplementemia and positive ANA raised suspicion for SLE, a known autoimmune condition that can overlap with or initially present as ITP, particularly in adolescents. Studies have highlighted ITP as a potential early manifestation of SLE, reinforcing the importance of early autoimmune screening in pediatric patients with atypical or severe presentations. The development of vocal cord paralysis and stridor can occur secondary to autoimmune neuropathy or post-hemorrhagic complications, contributing to significant airway compromise. Timely airway management, including intubation and respiratory support, was critical throughout this patient's hospitalization.
The therapeutic approach to severe ITP with ICH involves rapid platelet count elevation using high-dose corticosteroids, IVIG, and platelet transfusions, as needed. In refractory cases, thrombopoietin receptor agonists such as romiplostim may benefit long-term hematologic control. Neurocritical care, including ICP monitoring, seizure prophylaxis, and neurosurgical intervention, is essential in managing ICH. Multidisciplinary coordination across hematology, neurology, critical care, and rehabilitation services is vital for addressing acute complications and long-term functional recovery. This case underscores the need for heightened vigilance in pediatric ITP patients with atypical features. Early rheumatologic workup and tailored immunosuppressive therapy may help prevent complications and improve long-term outcomes.

Conclusion: This case highlights the potential for severe, life-threatening rare complications in pediatric ITP, underscoring the importance of a comprehensive autoimmune workup and multidisciplinary management approach.