Winning Abstracts from the 2009 Medical Student Abstract Competition: Humate P for Treatment of Persistent, Life Threatening Epistaxis in a Patient with Glanzmannís Thrombasthenia: A Case Report
Authors: Richard A Zinke and Chris E Holmes, MD PhD
Glanzmannís thrombasthenia is a rare inherited disorder of platelet function caused by a deficiency or abnormality in the platelet glycoprotein (GP) IIb/IIIa complex. Treatment of Glanzmannís thrombasthenia with transfused blood products and desmopressin (DDAVP) is effective in many patients. Recombinant factor VIIa (rFVIIa) is often used in severe, refractory bleeds. We report on the successful use of Antihemophilic Factor VIII/von Willebrand Factor Complex (Humate P) in a Glanzmannís thrombasthenia patient with active bleeding who had failed to respond to aggressive prior management including recurrent rFVIIa dosing.
A 54-year-old woman with Glanzmannís thrombasthenia presented with persistent posterior epistaxis of three to four weeks duration unresponsive to platelet transfusions, multiple doses of rFVIIa, and local electrocautery and nasal packing at an outside institution. Despite aggressive therapy including repeated packed red blood cell transfusions, the patient was admitted to the ICU with a hemoglobin level of 4.2 mg/dl and active nasal bleeding leading to mild airway compromise. Treatment was continued with multiple platelet and packed red blood cell transfusions and two 90 mg/kg doses of rFVIIa were given. Epistaxis persisted and she remained hemodynamically unstable despite intranasal aminocaproic acid and phenylephrine four times daily. The patient periodically experienced severe transfusion reactions which were later found to likely be related to her development of GPIIb/IIIa alloantibodies. DDAVP therapy was contraindicated due to this patientís chronic hyponatremia (Sodium = 128 mEq/L). Factor VIII (434%, ref 61-192) and von Willebrand factor (352%, ref 50-200) levels were elevated; however, the decision was made to proceed with Humate P therapy given the tenuous clinical situation. Humate P (2450 ristocetin cofactor (RCoF) units, 25 units/kg) resulted in cessation of her epistaxis within four hours. A maintenance regimen of intranasal aminocaproic acid, estrogen nasal cream, and isoflavones has prevented recurrent hemorrhage.
Despite already elevated levels of factor VIII and von Willebrand factor (vWF), our patient had an abrupt response to Humate P infusion after more than five weeks of continuous, life threatening epistaxis. Humate P may offer an additional therapeutic option for patients with Glanzmannís thrombasthenia and may provide insight into potential mechanistic functions of vWF in these patients.
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