Acquired Glanzmann Thrombasthenia in a Pediatric Patient with Alagille Syndrome

Abstract

Abstract 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.