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Authors: Richard A Zinke and Chris E Holmes, MD
Glanzmann's 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 Glanzmann's
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 Glanzmann's thrombasthenia patient
with active bleeding who had failed to respond to aggressive prior
management including recurrent rFVIIa dosing.
A 54-year-old woman with Glanzmann's 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 patient's 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 Glanzmann's thrombasthenia and may provide insight
into potential mechanistic functions of vWF in these patients.
February 2010 Issue of IMpact