Abstract B030: Intracerebral and intraventricular hemorrhage due to severe thrombocytopenia: A rare case of Evans syndrome
Authors: Patterson Kiersten R. , Khan Aliya M. , Patel Raj 
Published: December 2023

Introduction: Evans syndrome (ES) is a rare autoimmune disorder characterized by the development of autoimmune hemolytic anemia (AIHA), immune thrombocytopenia (ITP), and/or immune neutropenia (AIN) without an identifiable cause. It is believed to involve immune system dysregulation, leading to the destruction of red blood cells, platelets, and neutrophils. Case Presentation: A 68-year-old male with a history of coronary artery disease, hypertension, diabetes, and atrial fibrillation presented with abdominal pain and diarrhea. Laboratory tests revealed a platelet count of 3k, suggesting ITP. Initial treatment with dexamethasone and platelet transfusion provided limited improvement. A bone marrow biopsy showed mildly hypercellular bone marrow with trilineage hematopoiesis. However, the patient's condition rapidly deteriorated, and he returned to the emergency department with severe symptoms, including headache, gum bleeding, and lower extremity petechiae. A CT scan revealed a large intraparenchymal hematoma with intraventricular hemorrhage, highlighting the risk of life-threatening bleeding in ES patients. Discussion: The patient was managed with platelet transfusions, high-dose steroids, and intravenous immunoglobulin (IVIG), which provided temporary relief but failed to maintain stable platelet levels. Further investigations revealed a positive antinuclear antibody (ANA), suggesting an underlying rheumatological condition and confirming the diagnosis of secondary ES. This finding necessitated the exploration of treatment options. Of interest is Romiplostim (Promacta), a thrombopoietin receptor agonist, which has shown promise in increasing platelet counts and reducing bleeding episodes in refractory ITP. Considering the positive ANA and the suspected rheumatological etiology, Romiplostim may serve as a viable long-term treatment option to stabilize platelet levels and mitigate bleeding risks in this case. Conclusion: This case highlights the complexity of Evans syndrome and emphasizes the need to promptly recognize and manage life-threatening bleeding complications, particularly in older patients. The presence of a positive ANA suggests an underlying rheumatological condition, necessitating comprehensive evaluation, and tailored therapeutic approaches. Although further research is needed to understand the pathogenesis and optimal management strategies for ES, Romiplostim holds promise as a potential treatment for patients with secondary ES who are unresponsive to conventional therapies.    

Citation Format: Kiersten R. Patterson, Aliya M. Khan, Raj Patel. Intracerebral and intraventricular hemorrhage due to severe thrombocytopenia: A rare case of Evans syndrome [abstract]. In: Proceedings of the 16th AACR Conference on the Science of Cancer Health Disparities in Racial/Ethnic Minorities and the Medically Underserved; 2023 Sep 29-Oct 2;Orlando, FL. Philadelphia (PA): AACR; Cancer Epidemiol Biomarkers Prev 2023;32(12 Suppl):Abstract nr B030.