Anesth Analg 2004;99:1258-1260
© 2004 International Anesthesia Research Society
doi: 10.1213/01.ANE.0000131726.09685.BF
GENERAL ARTICLES
A Patient with Glanzmann’s Thrombasthenia for Emergent Abdominal Surgery
Halil Ibrahim Uzunlar, MD,
Ahmet Eroglu, MD,
Ahmet Can Senel, MD,
Habib Bostan, MD, and
Nesrin Erciyes, MD
Department of Anesthesiology and Reanimation, Karadeniz Technical University Faculty of Medicine, Trabzon, Turkey
Address correspondence and reprint requests to Halil Ibrahim Uzunlar, MD, Karadeniz Technical University Tip Fakültesi, Anesteziyoloji 61080, Trabzon, Turkey. Address e-mail to uzunlar{at}gmx.com.tr
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Abstract
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Glanzmann’s thrombasthenia is a rare autosomal recessive disease characterized by potentially major mucocutaneous complications and nose bleeds. It is considered hazardous for these surgical patients to conceive, with a high risk of urgent surgery. The treatment of bleeding or prevention of hemorrhage for surgery or invasive procedures is based on platelet transfusion. However, platelet transfusions may be responsible for the development of alloimmunization, with a high risk of future platelet refractoriness. We report a surgical case of Glanzmann’s thrombasthenia complicated by nasopharyngeal bleeding and managed with platelet transfusions, recombinant activated factor VII, and postoperative airway management in the intensive care unit.
IMPLICATIONS: We describe the management of an emergent surgical patient with Glanzmann’s thrombasthenia and nasopharyngeal bleeding requiring platelets and recombinant activated factor VII infusions. These patients may need postoperative intensive care.
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Introduction
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Glanzmann’s thrombasthenia (GT), a rare inherited platelet disorder, is characterized by a complete lack of platelet aggregation due to a defect in the platelet membrane receptor complex (
IIb/ßIIIa) (1). Epistaxis, purpuric-type bleeding, gum bleeding, and menorrhagia are the common clinical manifestations of GT. Spontaneous bleeding (2) is uncommon, but posttraumatic and postoperative hemorrhage may be particularly serious. There is no specific treatment. Prophylactic and therapeutic platelet transfusions are supportive. In many patients, the efficacy of this approach is diminished by allo-antiplatelet antibodies. As a current therapeutic management, recombinant activated factor VII (rFVIIa) is proposed for hemorrhage associated with GT (1,2). We present and discuss the perioperative special requirements of a patient with GT who required emergent exploratory laparotomy under general anesthesia for a gangrenous appendix.
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Case Report
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A 21-yr-old man with GT presented to the emergency department with abdominal pain. The patient was diagnosed with GT in early childhood subsequent to the development of multiple nose and cutaneous bleeds. His disease was determined to be the result of prolonged bleeding time, with a normal platelet count and absence of aggregation of platelets with the agonist adenosine diphosphate. Absence of the glycoprotein (GP)IIb/IIIa receptor was confirmed by flow cytometry. His condition required multiple whole blood and platelet transfusions throughout childhood and adolescence. Two years previously, he had had gastrointestinal bleeding and required multiple transfusions of whole blood and platelets, resulting in a prolonged stay in the hospital. Fortunately, he did not have any surgery or invasive procedures at this time. On examination, acute appendicitis was suspected. As shown in Table 1, laboratory results, except for the bleeding time, were normal. Bleeding time measured by the Ivy method (normal, 2–7 min) was prolonged (>30 min), but prothrombin and activated partial thromboplastin times, platelet count, and morphology were all normal. Urgent exploratory laparotomy was planned.
Before surgery, three ABO-identical plateletpheresis units were administered over 30 min in combination with rFVIIa 90 µg/kg (NovoSeven®; Novo Nordisk, Denmark). After transfusion of platelets, the bleeding time decreased to 18 min, and his platelet count did not change. A regimen for perioperative platelet management was planned. Four units of whole blood were cross-matched, and good venous access was achieved by the placement of 2 16-gauge cannulas in his forearm veins in case of probable intraoperative bleeding. A rapid induction of anesthesia and oral tracheal intubation were performed. Anesthesia was maintained during surgery with oxygen, nitrous oxide, and sevoflurane via a circle system. A right-sided triple-lumen central line was sited via the external jugular vein after the induction of anesthesia in case of excessive hemorrhage. A nasogastric tube (NG) was placed during surgery because of extensive abdominal distention. The patient had a gangrenous appendix. Because of intraabdominal adherence, the operation was prolonged to 2.5 h. The procedure progressed uneventfully, with an estimated blood loss of 100–200 mL. In total, 3 U of platelets were given during surgery, in addition to the 3 U given before surgery. Meticulous hemostasis was achieved before wound closure, but bleeding at the NG insertion site continued even after completion of the surgical procedure. For this reason, the patient was not tracheally extubated and was transferred to the intensive care unit (ICU). In the ICU, under sedation/analgesia, mechanical ventilation was maintained.
Because the nasopharyngeal bleeding continued despite packing, and because the bleeding time was measured as 30 min, the patient received rFVIIa every 2 h for the first 12 h, every 3 h for the second 12 h, and then every 4 h on Day 1 in doses of 90 µg/kg. He also received 6 U of platelets for the first 24 h. On the second day after surgery, the patient remained free from nasopharyngeal bleeding, with a bleeding time of 10 min, and was tracheally extubated. He was discharged from the ICU on the second postoperative day and had no further problems.
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Discussion
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Bleeding in patients with GT may be extremely difficult to stop. These patients have conventionally been managed with blood and blood component (platelet-rich plasma and platelet concentrates) transfusions to control hemorrhage resulting from trauma or surgical procedures. Life-threatening hemorrhage may occur in these individuals, usually secondary to surgical procedures or trauma. Familiarity with this disorder is necessary to intervene early, because transfusion of single-donor platelets may be lifesaving (2–4).
Human platelet antigen (HPA)-1 and HPA-3 are localized on GPIIb and GPIIIa, respectively, which are defective in GT, and refractoriness due to alloimmunization may develop with recurrent platelet transfusions (1). The development of anti-HPA 1a antibodies, requiring the selection of donors lacking this antigen (5), may result in a variable response to subsequent platelet transfusions (4,5). The perioperative evaluation of the hematological state in these patients is very important. In this case, an acceptable response to platelet transfusion was first demonstrated because the bleeding time decreased from 25 to 18 minutes, but repeated platelet transfusions administered later were not effective to decrease bleeding time. Because of this unresponsiveness, development of anti-HPA 1a antibodies was considered. After demonstration of these antibodies on the patient’s platelets by using fluorescein-conjugated monoclonal antibodies to GPIIb/IIIa with flow cytometry, rFVIIa was administered again.
Because there is no universally available monitor of platelet function, its clinical course is difficult to monitor. Techniques include measurement of bleeding time, platelet aggregometry, and thromboelastography. Platelet aggregometry and thromboelastography are not widely available (6). The latter has been reported in only one case of GT (4). We used bleeding time only to monitor platelet function for the response to treatment, because these tests are not available in our hospital. In our case, 3 U of platelets with a single preoperative dose of rFVIIa decreased the bleeding time at first. However, satisfactorily decreased bleeding and bleeding time in the postoperative period were achieved only after the rFVIIa infusion.
Failure to obtain a shorter bleeding time after repeated platelet transfusion was intriguing, because circulating homologous platelets were clearly evident. This report shows that such assumptions regarding failure to achieve platelet increments posttransfusion in this patient or others may need reappraisal and that rFVIIa may be useful used in tandem or with platelet transfusions in GT. The treatment of bleeds in GT is a challenging issue, especially when repeated platelet transfusions have induced anti-GPIIb/IIIa. In such conditions, rFVIIa is the most important alternative treatment for bleeding (7,8). We used rFVIIa as first-line therapy in our patient, who had anti-GPIIb/IIIa antibodies. In this case, the reason for the use of a prophylactic preoperative single dose of rFVIIa with platelets was the risk of excessive surgical bleeding. Platelet transfusion remains the first-line therapy for GT in case of bleeding or prophylaxis for surgery unless there are antibodies to platelets. In thrombasthenic patients with antiplatelet antibodies, rFVIIa is a potent alternative (7,9). Because platelet transfusions were not effective later, we administered rFVIIa to stop nasopharyngeal bleeding.
In patients with hemorrhagic diathesis, surgical or invasive interventions must be performed much more cautiously and gently. In such patients with a history of nosebleeds, either NG should not be inserted or, if it is absolutely necessary, as in our case, the most suitable one should be inserted with meticulous care. The best way of placing tube orally with a guide (through the airway) may cause much less trauma to the mucosa. However, in our case, because postoperative decompression of the stomach was needed, we placed the tube nasally. Probably, if the more careful and gentle technique had been used, less mucosal trauma and bleeding would have occurred.
In these patients, intubation and tube insertion may cause persistent bleeding, resulting in difficulty of airway management and even requiring intensive care. In these conditions, the bleeding from invasive manipulations and perioperative surgical hemorrhage may be serious. This case report describes some of the uncommon features of the disorders and the currently available options, which anesthesiologists and intensivists should be aware of during the perioperative management of these patients.
Only one case with GT has been reported related to an elective cesarean delivery (4). Our patient is the first GT case in the literature with emergent surgery for acute appendicitis.
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References
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Accepted for publication April 23, 2004.
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Abstract
Introduction
