Treatment strategies and outcomes in pediatric patients with immune thrombocytopenia (ITP) undergoing surgical procedures: A multicenter study

Abstract

Abstract Introduction: Children with immune thrombocytopenia (ITP) may have an increased risk of perioperative bleeding. However, current ITP guidelines do not address this risk, and studies of surgical management of suspected bleeding disorders often exclude ITP patients. In this multicenter study, we examined perioperative management and outcomes in a cohort of children with ITP who underwent four common surgeries. We aimed to describe perioperative management and outcomes by platelet count, type of surgery, and ITP-directed treatment strategies. Methods: In conjunction with the ITP Consortium of North America (ICON), we conducted an IRB-approved retrospective chart review of patients with ITP aged 0-24 years who underwent tooth extraction, tonsillectomy and adenoidectomy (T&A), appendectomy, and/or splenectomy at six centers in the United States and Canada between 2019-2024. Patients were excluded if ITP was resolved at the time of surgical planning (defined as platelet count >150 x 109/L on two occasions or on one occasion with a normal mean platelet volume, in the absence of ITP-directed therapy). Results:Fifty-six patients underwent 61 surgical procedures. Median age at time of surgery was 14 years (range 3-24). Most patients (84%; 47/56) had chronic ITP, and 44% (27/61) of patients were receiving baseline ITP-directed treatments (range: 0-6 treatments) at the time surgery was recommended. Additional perioperative ITP-directed treatment was recommended for 52% (32/61) of surgeries, typically 1 (44%), 2 (47%), or rarely ≥3 treatments (9%). The most common surgery was tooth extraction(s), with 50% (18/36) of patients receiving perioperative treatment, including antifibrinolytics (n=12), corticosteroids (n=8), intravenous immunoglobulin (IVIG) (n=4), and/or thrombopoietin receptor agonists (TPO-RA) (n=4). Most patients undergoing splenectomy (80%; 8/10) received treatment, including corticosteroids (n=5), IVIG (n=5), TPO-RA (n=1), and/or platelet transfusion (n=1). A third (3/9) of patients undergoing appendectomy received treatment, including IVIG (n=3), corticosteroids (n=1), and/or platelet transfusion (n=1). Half of patients (3/6) undergoing T&A received treatment, including IVIG (n=2), corticosteroid (n=1), antifibrinolytic (n=1), and/or platelet transfusion (n=1). Across all surgeries, 83% (15/18) of patients with platelet counts <50 x 109/L received perioperative treatment, compared to 53% (9/17) with platelet counts 50-99 x 109/L, and 33% (8/24) with platelet counts ≥100 x 109/L. At the time of surgery, 93% of patients had platelet counts ≥50 x 109/L (53/57); one patient had a platelet count of 1 x 109/L, and three had platelet counts 30-49 x 109/L. There were two perioperative bleeding events (3%; 2/58), both related to tooth extractions. In the first case, the patient with a platelet count of 1 x 109/L had severe bleeding at the site of extraction requiring emergent intervention. Hematology was not aware of the procedure, and no additional perioperative ITP treatment was provided. The second patient had a platelet count of 69 x 109/L and had self-limited bleeding at the site of extraction; this patient had received an antifibrinolytic for dual management of perioperative bleeding risk and heavy menses. Hematology was consulted perioperatively in 89% (49/55) of cases. Hospital admission was reported for 30% (18/61) of surgeries, and 10% (6/59) of patients had unanticipated hospital admissions (n=1) or emergency room visits (n=5; of which only 1 was likely ITP-related) within 28 days of surgery. Medication side effects were reported in 7% of patients receiving ITP-directed therapies (3/43); all three patients experienced headaches. Conclusions: In this study of 61 surgeries, 52% of pediatric patients with ITP were given perioperative ITP-directed treatments, most commonly antifibrinolytics, IVIG, and corticosteroids. Treatment was prescribed for almost half of patients with a platelet count ≥50 x 109/L at the time surgery was recommended. Notably, only two patients had bleeding events, of which one had a platelet count ≥50 x 109/L. Our findings suggest that children with ITP with higher platelet counts are generally at low risk of procedural bleeding. Variable management strategies were used, which may represent the heterogeneity of the disease and variable responses to emergent therapies. Future validation studies should examine perioperative ITP management and resource utilization in larger cohorts.