Surgery-Associated Bleeding Risk in Patients with Platelet Function Disorders - a Cross Sectional Study with the American Thrombosis and Hemostasis Network Dataset (ATHNdataset)

Introduction: Platelet function disorders (PFDs) are a group of heterogenous bleeding disorders with varying bleeding phenotype. Intraoperative and post-operative bleeding are serious complications among patients with PFDs undergoing surgery. There are very few studies in literature that have specifically investigated surgery associated bleeding complications in PFDs. The aim of this study was to utilize a large national dataset to describe surgeries performed in patients with PFD, characterize the bleeding associated with these surgical procedures and outline the therapeutic approaches adopted. Methods: In this retrospective study, the ATHNdataset was queried for demographic data, PFD diagnosis, surgeries among patients with PFD, intraoperative and post-operative bleeding episodes and treatment. Descriptive statistics were used. The ATHNdataset captures information from patients with bleeding and clotting disorders from over 140 federally funded hemophilia and thrombosis treatment centers (HTCs) in the US. Patients authorize inclusion of their demographic and clinical information in this de-identified Health Insurance Portability and Accountability Act (HIPAA)-compliant data set. Results: From January 2010 to March 2020, the ATHNdataset captured 2767 patients with PFDs, of which 1769 (63.93%) were female and 998 (36.1%) were male, with 1393 patients between 0-18 years (50%) and 1374 (50%) adults >18 years. PFDs identified include 32 patients with Bernard Soulier syndrome (1.16%), 131 patients with Glanzmann thrombasthenia (4.7%), 4 patients with Gray platelet syndrome (0.14%), 29 patients with Hermansky Pudlak syndrome (1%), 1548 patients with storage pool deficiency (55.9%), and 1023 patients diagnosed as PFD (36.9%). A total of 3252 procedures were reported between 2010 and 2020; 1271 patients (46%) patients with at least one documented procedure. Figure 1 shows common procedures among patients with PFDs. Surgery-associated bleeding episodes (includes intraoperative and post-operative bleeds) were reported with 69 procedures (2.1%), which included intraoperative bleeds reported for 18 procedures (0.5%) and post-operative bleeds reported for 51 procedures (1.6%). Of the 60 procedures in patients with Glanzmann thrombasthenia, surgery-associated bleeding episodes were reported after 9 dental procedures (41%), 1 circumcision (25%) and 11 other surgeries/procedures (18.3%). Of the 6 procedures in patients with Bernard Soulier syndrome, no intraoperative or post-operative bleeding episodes were reported. Of 1688 procedures in patients with storage pool deficiency, surgery-associated bleeding episodes were reported after 26 dental procedures (1.5%) and 62 other surgeries/procedures (3.67%). No intraoperative or post-operative mortality was reported among these patients. Of 1272 patients who underwent at least 1 procedure, 646 patients (50.7%) received some form of treatment before/during/after a procedure. Among these 646 patients, 2794 exposure days of hemostasis medications were used before/during/after procedures. Among these, 49% were prior to the procedure, 0.7 % during the procedure and 49.5% after the procedure. Treatments used are shown in figure 2. Conclusion: Our study shows that patients with PFDs have a substantial risk of bleeding associated with surgery. Identifying the risk of bleeding by type and providing appropriate pre-surgical prophylaxis can decrease rates of surgery-associated bleeding in PFDs. Figure 1 Figure 1. Disclosures Sidonio: Sanofi, Takeda, Octapharma, Bayer, Biomain, Grifols, Kedrion, Genentech. Catalyst, Guardian Therapeutics, Novo Nordisk, Hema Biologics, Uniqure.: Consultancy, Honoraria. Ahuja: Genentech: Membership on an entity's Board of Directors or advisory committees; Takeda: Other: DSMB member ; XaTek, Inc: Patents & Royalties; Sanofi: Membership on an entity's Board of Directors or advisory committees.

Acquired Glanzmann Thrombasthenia in a Pediatric Patient with Alagille Syndrome

Introduction/Objective Acquired Glanzmann Thrombasthenia is a rare bleeding disorder that is characterized by inhibition of glycoprotein IIb/IIIa signaling, usually by an autoantibody, leading to an interference in platelet aggregation. Clinically, this disorder presents with spontaneous mucocutaneous bleeding in the setting of a normal platelet count. Acquired Glanzmann Thrombasthenia has been associated with primary immune thrombocytopenic purpura (ITP), several types of hematologic and solid malignancies, solid organ transplants, and other autoimmune disorders. Methods/Case Report A 4-year-old female patient with a history of Alagille Syndrome requiring liver transplant at age 3 was admitted to the hospital after presenting to the emergency department with complaints of bruising, nosebleeds, and a petechial rash. The patient was found to have a platelet count of 11 K/mm3 and was diagnosed with ITP. The patient received a single dose of IVIG at 1g/kg with subsequent resolution of bleeding and a recovering platelet count of 27 K/mm3 12 hours after administration. However, two months later, the patient presented again with worsening bruising, multiple nosebleeds per day, and worsening petechiae. Lab studies revealed the patient’s platelet count was within normal limits. A platelet antibody screen was positive with a subsequent Platelet Antibody Bead Array revealing anti-Gp IIb/IIIa HPA-1 and HPA-3 positivity. Results (if a Case Study enter NA) N/A Conclusion Acquired Glanzmann Thrombasthenia is a rare bleeding disorder that is the result of interference with platelet aggregation. Antibodies that may be associated with any of several underlying conditions lead to impaired platelet function and subsequent mucocutaneous bleeding. The present case represents an occurrence of Acquired Glanzmann Thrombasthenia in a patient with multiple risk factors for development of the disorder.

Glanzmann Thrombasthenia: A Case Report of a Rare Inherited Coagulation Disorder Presenting with Traumatic Head Injury

This case study deals with a 32-year-old Indian male patient who presented with a traumatic head injury in the hospital, experienced uncontrolled bleeding after conducting surgery, and was eventually diagnosed with Glanzmann thrombasthenia. Glanzmann thrombasthenia is a rare hereditary blood clotting disorder characterised by a lack of platelet aggregation due to the absence of platelet glycoprotein IIb/IIIa. This occurrence is generally triggered by consanguineous marriages and is apparent in approximately one in one million people. Education and raising awareness about consanguinity in communities may help to reduce challenging, unusual genetic diseases.

Optimized outcome of pregnancy in a woman with Glanzmann thrombasthenia with multidisciplinary approach

Glanzmann thrombasthenia (GT), an autosomal recessive disorder of defective platelet aggregation due to abnormalities of platelet receptor GPIIb/IIIa, can result in complication of bleeding during pregnancy. We report multidisciplinary management of a case with optimized outcome. 23 years old short statured (142 cm) primigravida was referred for contracted pelvis at term in labour. She was diagnosed with GT at 12 years of age during evaluation of gum bleeding, easy bruising, and prolonged post traumatic bleeding. She was born of a second-degree consanguineous marriage. At admission she was hemodynamically stable but moderately pale. Her hemoglobin was 8.7 g%, with 4.6 lacs platelets and clotting time of 15 minutes. Multidisciplinary team of hematologist, transfusionist and anesthetist was activated. Arranging single donor platelets in a short notice was challenging. Emergency cesarean section under general anesthesia was performed after transfusing two units packed red blood cell and 2 single donor platelets to deliver an alive male baby of 3.3 kg. Hemostasis was secured with cautery and ligatures. Tranexamic acid infusion and sublingual misoprostol was given prophylactically. she was transfused another 4 random donor platelets during operation and postoperatively. The case details will be presented. GT have normal to high platelet counts though they are normal morphologically, they are dysfunctional. This fact needs to be recognized and referred early to equipped centres. We used component transfusion, active management of third stage and tranexamic acid for optimizing outcome due to haemorrhage. At present, there is lack of consensus regarding optimum treatment of post-partum hemorrhage in patients with GT.