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An unusual presentation of idiopathic thrombocytopenic purpura in pregnancy
Journal of Clinical Anesthesia (2005) 17, 66 – 68
Case report
An unusual presentation of idiopathic thrombocytopenic purpura in pregnancy J. Sudharma Ranasinghe MD, FFARCSI, Evert W. Tjin-A-Tsoi MD, Michael C. Lewis MD* Department of Anesthesiology, University of Miami School of Medicine, Miami, FL 33101, USA Received 1 November 2003; accepted 16 March 2004
Keywords: Anesthesia, regional; Cesarean delivery, emergent; Idiopathic thrombocytopenic purpura; Pregnancy; Thrombocytopenia
Abstract A 35-year-old, 39-week pregnant woman underwent an uneventful emergent cesarean delivery for suspected placental abruption or uterine dehiscence. Given the urgency of the situation and the unremarkable airway anatomy, general anesthesia was the chosen technique. Four hours after her surgery, she returned to the operating room for persistent vaginal bleeding. Hematology tests performed before the cesarean delivery revealed severe thrombocytopenia. This was later diagnosed as idiopathic thrombocytopenia, which was treated successfully with steroid therapy. D 2005 Elsevier Inc. All rights reserved.
1. Introduction
2. Case report
Thrombocytopenia affects up to 10% of all pregnancies [1]. The commonest pathological cause is idiopathic thrombocytopenic purpura (ITP). This autoimmune disorder is associated with antiplatelet immunoglobulins. Usually, it occurs in the first and second trimesters of pregnancy and affects 1 to 2 per 10 000 pregnancies [2]. Here we present a case where this diagnosis was made only after the patient underwent an emergent cesarean section (C-section) complicated by postpartum bleeding. The anesthetic management of this patient is presented. In addition, the common causes of thrombocytopenia in an otherwise healthy pregnant patient are discussed.
A 35-year-old Haitian woman was admitted to the labor and delivery unit complaining of abdominal pain and vaginal bleeding. She had received no prenatal care and was 39 weeks pregnant (G5P1031). Previous history was significant for an uneventful C-section (unknown uterine scar) in Haiti for which she received general anesthesia. The patient admitted to having gingival bleeding during this pregnancy. Physical examination revealed a patient weighing 67 kg and 167 cm in height, blood pressure (BP) 120/76 mm Hg, heart rate 96 beats per minute, and urine examination showed trace of proteinuria. Airway evaluation revealed a class II Mallampati score, with the ability to subluxate the mandible. There was adequate mouth opening with excellent range of motion of the atlantooccipital joint. The patient was taken emergently to the operating room because of the suspicion of placental abruption or dehis-
* Corresponding author. Fax: +1 305 545 6753. E-mail address: [email protected] (M.C. Lewis). 0952-8180/$ – see front matter D 2005 Elsevier Inc. All rights reserved. doi:10.1016/j.jclinane.2004.03.016
Thrombocytopenia complicating pregnancy cence of the previous uterine scar. The patient’s vital signs and fetal HR were within normal limits. Laboratory results were still pending. Because of the history of bleeding, the unavailability of the laboratory results, and the unremarkable airway anatomy, a decision was made to proceed with general anesthesia. The anesthetic course was uneventful. On uterine incision, the surgeon noted marginal abruption. The baby was extracted without complications, with Apgar scores of 8 and 9 at 1 and 5 minutes, respectively. Blood loss was estimated to be about 1200 mL, and 2200 mL of Ringer’s lactate solution was infused during surgery. Four hours postoperatively, the patient was noted to have a significant postpartum vaginal hemorrhage approximating 1500 mL. An oxytocin infusion was initiated and methylergonovine 0.2 mg was administered intramuscularly (Im). Because of continued bleeding despite these interventions, the patient was brought back to the operating room for examination during anesthesia. Laboratory tests drawn before C-section were now available and revealed a platelet count of 3000, with normal prothrombin time (pT) and partial thromboplastin (pTT) times. Examination during anesthesia revealed no obvious cause of bleeding. The patient was transported to the postanesthesia recovery unit and was transfused with 12 units of platelets and 2 units of packed red blood cells were given because of the continued significant hemorrhage. A primary differential diagnosis of hemolytic anemia-elevated liver function tests and low platelets (HELLP) syndrome vs ITP was made. Posttransfusion laboratory results showed hemoglobin/hematocrit 8.4/24, pT/pTT 12.9/29, fibrinogen 237, platelet 5000 lL, and normal liver function tests. The blood smear revealed giant platelets without evidence of fragmentation and was positive for spherocytes and occasional target cells. A diagnosis of ITP was then confirmed and therapy was initiated with prednisone 2 mg/kg per day for 5 days followed by prednisone 20 mg 3 times a day. The platelet count in subsequent laboratorytests increased from 6 to 23 to 77 1000/lL, and the patient was discharged home 5 days later.
3. Discussion Idiopathic thrombocytopenic purpura is the most common cause of thrombocytopenia in the first trimester, whereas gestational thrombocytopenia is more commonly seen in the second or third trimester. The latter is a benign disorder that affects approximately 5% of all pregnant women. It may reflect the effects of hemodilution or accelerated platelet clearance. Although most patients show platelet counts in the range of 110 to 150 000/lL, counts less than 70 000/lL are unlikely with this disorder. The pathophysiology of ITP involves the development of antibodies against platelet glycoproteins, primarily GpIIIIIa and GpIb-IX, as well as the clearance of these immunoglobulin G (IgG)–coated platelets by the reticulo-
67 endothelial system. The diagnosis is not difficult in the setting of moderate to severe thrombocytopenia (ie, b50 000/lL). Although asymptomatic patients with platelet counts N30 000 lL do not generally require treatment, more aggressive measures are required as pregnancy approaches term, to minimize hemorrhagic complications of delivery and facilitate administration of regional anesthesia. Because of transplacental passage of maternal IgG, particularly during the third trimester, fetal thrombocytopenia may also occur. Currently, there is no proven maternal treatment to decrease the incidence of fetal thrombocytopenia. Moreover, because of the increased risk of neonatal intracranial hemorrhage, the route of delivery is still debatable. The goal of treatment for patients with ITP is remission, not cure [3]. Corticosteroids are considered the first line of therapy because of their efficacy and low cost. High-dose intravenous g -globulins may be used as initial therapy in some cases because of their low toxicity profile and relatively rapid onset of action. Splenectomy, immunosuppressive therapy, or plasmapheresis is reserved for refractory cases. Thrombocytopenia may also be an early sign of preeclampsia. Occasionally, thrombocytopenia may even precede other manifestations of preeclampsia. This thrombocytopenia occurs because of accelerated platelet clearance and/or destruction, adhesion of circulating platelets to damaged or activated endothelium, activation and consumption of platelets as a consequence of activation of the hemostatic mechanisms, and clearance of IgG-coated platelets by the reticuloendothelial system. The HELLP syndrome is a variant of preeclampsia that occurs primarily in white multiparous women older than 25 years. Criteria for this disorder include (1) microangiopathic hemolytic anemia, (2) level of serum aspartate aminotransferase and serum glutamine-O-transferase N70 lL, and (3) thrombocytopenia (b100 000 lL). Other causes of thrombocytopenia to be considered in pregnancy include HIV infection and systemic lupus erythematosus. A low platelet count may occur in up to 25% of patients with systemic lupus erythematosus secondary to platelet destruction due to antiplatelet antibodies, circulating immune complexes, or other causes. In this case, thrombocytopenia was not detected until after the C-section, partly because of lack of prenatal care. The diagnosis of ITP was easy to make in the setting of severe thrombocytopenia and otherwise normal coagulation parameters, and the patient responded well to the steroid therapy. Mortality from complications of general anesthesia was shown to be 16.7 times that of regional anesthesia [4]. Inability to intubate or ventilate the lungs and pulmonary aspiration are the leading causes of maternal death related to anesthesia. Therefore, spinal and epidural anesthesia have become the anesthetic techniques of choice in obstetric practice whereas general anesthesia is generally reserved for emergency situations.
68 Because of the urgency of the situation, the history of bleeding, and the unremarkable airway evaluation, we chose to perform general anesthesia. Had the scenario been one of an anticipated difficult airway, regional anesthesia would have been considered. In this case, a transfusion of platelets should have been given immediately before the procedure to minimize the risk of development of an epidural hematoma. The absolute number of platelets considered safe for the performance of a neuroaxial block is still debatable. Regional anesthesia has been administered safely to patients with mild thrombocytopenia (platelet count N69 000/lL) [5]. Although unusual bleeding was not noticed during the cesarean delivery, the safety of regional anesthesia would still be questionable in this case. This case reflects the importance of an appropriate preoperative evaluation before any anesthetic encounter. Even during an emergency, the anesthesia provider has to take a thorough history to detect any abnormalities on the physical or the laboratory examination that would be crucial to determine the best anesthetic plan for that patient. The
J.S. Ranasinghe et al. choice of anesthesia is dictated by many factors, and in controversial situations, it is for the anesthesia consultant to weigh the risks vs benefits of each technique.
References [1] McCrae KR, Samuels P, Schreiber AD. Pregnancy-associated thrombocytopenia: pathogenesis and management. Blood 1992;80: 2697 - 714. [2] Rath FA. Differential diagnosis of thrombocytopenia in pregnancy. Zentralbl Gynakol 2001;123:80 - 90. [3] Laros Jr RK. Coagulation disorders and hemoglobinopathies in the obstetric and surgical patient. In: Hughes SC, Levinson G, Rosen MA, editors. Shnider and Levinson’s Anesthesia for Obstetrics. 4th ed. Philadelphia7 Lippincott Williams and Wilkins; 2001. p. 347. [4] Hawkins JL, Koonin LM, Palmer SK, Gibbs CP. Anesthesiarelated deaths during obstetric delivery in the United States, 19791990. Anesthesiology 1997;86:277 - 84. [5] Beilin Y, Zahn J, Comerford M. Safe epidural analgesia in thirty parturients with platelet counts between 69,000 and 98,000 mm3. Anesth Analg 1997;85:385 - 8.
Case report
An unusual presentation of idiopathic thrombocytopenic purpura in pregnancy J. Sudharma Ranasinghe MD, FFARCSI, Evert W. Tjin-A-Tsoi MD, Michael C. Lewis MD* Department of Anesthesiology, University of Miami School of Medicine, Miami, FL 33101, USA Received 1 November 2003; accepted 16 March 2004
Keywords: Anesthesia, regional; Cesarean delivery, emergent; Idiopathic thrombocytopenic purpura; Pregnancy; Thrombocytopenia
Abstract A 35-year-old, 39-week pregnant woman underwent an uneventful emergent cesarean delivery for suspected placental abruption or uterine dehiscence. Given the urgency of the situation and the unremarkable airway anatomy, general anesthesia was the chosen technique. Four hours after her surgery, she returned to the operating room for persistent vaginal bleeding. Hematology tests performed before the cesarean delivery revealed severe thrombocytopenia. This was later diagnosed as idiopathic thrombocytopenia, which was treated successfully with steroid therapy. D 2005 Elsevier Inc. All rights reserved.
1. Introduction
2. Case report
Thrombocytopenia affects up to 10% of all pregnancies [1]. The commonest pathological cause is idiopathic thrombocytopenic purpura (ITP). This autoimmune disorder is associated with antiplatelet immunoglobulins. Usually, it occurs in the first and second trimesters of pregnancy and affects 1 to 2 per 10 000 pregnancies [2]. Here we present a case where this diagnosis was made only after the patient underwent an emergent cesarean section (C-section) complicated by postpartum bleeding. The anesthetic management of this patient is presented. In addition, the common causes of thrombocytopenia in an otherwise healthy pregnant patient are discussed.
A 35-year-old Haitian woman was admitted to the labor and delivery unit complaining of abdominal pain and vaginal bleeding. She had received no prenatal care and was 39 weeks pregnant (G5P1031). Previous history was significant for an uneventful C-section (unknown uterine scar) in Haiti for which she received general anesthesia. The patient admitted to having gingival bleeding during this pregnancy. Physical examination revealed a patient weighing 67 kg and 167 cm in height, blood pressure (BP) 120/76 mm Hg, heart rate 96 beats per minute, and urine examination showed trace of proteinuria. Airway evaluation revealed a class II Mallampati score, with the ability to subluxate the mandible. There was adequate mouth opening with excellent range of motion of the atlantooccipital joint. The patient was taken emergently to the operating room because of the suspicion of placental abruption or dehis-
* Corresponding author. Fax: +1 305 545 6753. E-mail address: [email protected] (M.C. Lewis). 0952-8180/$ – see front matter D 2005 Elsevier Inc. All rights reserved. doi:10.1016/j.jclinane.2004.03.016
Thrombocytopenia complicating pregnancy cence of the previous uterine scar. The patient’s vital signs and fetal HR were within normal limits. Laboratory results were still pending. Because of the history of bleeding, the unavailability of the laboratory results, and the unremarkable airway anatomy, a decision was made to proceed with general anesthesia. The anesthetic course was uneventful. On uterine incision, the surgeon noted marginal abruption. The baby was extracted without complications, with Apgar scores of 8 and 9 at 1 and 5 minutes, respectively. Blood loss was estimated to be about 1200 mL, and 2200 mL of Ringer’s lactate solution was infused during surgery. Four hours postoperatively, the patient was noted to have a significant postpartum vaginal hemorrhage approximating 1500 mL. An oxytocin infusion was initiated and methylergonovine 0.2 mg was administered intramuscularly (Im). Because of continued bleeding despite these interventions, the patient was brought back to the operating room for examination during anesthesia. Laboratory tests drawn before C-section were now available and revealed a platelet count of 3000, with normal prothrombin time (pT) and partial thromboplastin (pTT) times. Examination during anesthesia revealed no obvious cause of bleeding. The patient was transported to the postanesthesia recovery unit and was transfused with 12 units of platelets and 2 units of packed red blood cells were given because of the continued significant hemorrhage. A primary differential diagnosis of hemolytic anemia-elevated liver function tests and low platelets (HELLP) syndrome vs ITP was made. Posttransfusion laboratory results showed hemoglobin/hematocrit 8.4/24, pT/pTT 12.9/29, fibrinogen 237, platelet 5000 lL, and normal liver function tests. The blood smear revealed giant platelets without evidence of fragmentation and was positive for spherocytes and occasional target cells. A diagnosis of ITP was then confirmed and therapy was initiated with prednisone 2 mg/kg per day for 5 days followed by prednisone 20 mg 3 times a day. The platelet count in subsequent laboratorytests increased from 6 to 23 to 77 1000/lL, and the patient was discharged home 5 days later.
3. Discussion Idiopathic thrombocytopenic purpura is the most common cause of thrombocytopenia in the first trimester, whereas gestational thrombocytopenia is more commonly seen in the second or third trimester. The latter is a benign disorder that affects approximately 5% of all pregnant women. It may reflect the effects of hemodilution or accelerated platelet clearance. Although most patients show platelet counts in the range of 110 to 150 000/lL, counts less than 70 000/lL are unlikely with this disorder. The pathophysiology of ITP involves the development of antibodies against platelet glycoproteins, primarily GpIIIIIa and GpIb-IX, as well as the clearance of these immunoglobulin G (IgG)–coated platelets by the reticulo-
67 endothelial system. The diagnosis is not difficult in the setting of moderate to severe thrombocytopenia (ie, b50 000/lL). Although asymptomatic patients with platelet counts N30 000 lL do not generally require treatment, more aggressive measures are required as pregnancy approaches term, to minimize hemorrhagic complications of delivery and facilitate administration of regional anesthesia. Because of transplacental passage of maternal IgG, particularly during the third trimester, fetal thrombocytopenia may also occur. Currently, there is no proven maternal treatment to decrease the incidence of fetal thrombocytopenia. Moreover, because of the increased risk of neonatal intracranial hemorrhage, the route of delivery is still debatable. The goal of treatment for patients with ITP is remission, not cure [3]. Corticosteroids are considered the first line of therapy because of their efficacy and low cost. High-dose intravenous g -globulins may be used as initial therapy in some cases because of their low toxicity profile and relatively rapid onset of action. Splenectomy, immunosuppressive therapy, or plasmapheresis is reserved for refractory cases. Thrombocytopenia may also be an early sign of preeclampsia. Occasionally, thrombocytopenia may even precede other manifestations of preeclampsia. This thrombocytopenia occurs because of accelerated platelet clearance and/or destruction, adhesion of circulating platelets to damaged or activated endothelium, activation and consumption of platelets as a consequence of activation of the hemostatic mechanisms, and clearance of IgG-coated platelets by the reticuloendothelial system. The HELLP syndrome is a variant of preeclampsia that occurs primarily in white multiparous women older than 25 years. Criteria for this disorder include (1) microangiopathic hemolytic anemia, (2) level of serum aspartate aminotransferase and serum glutamine-O-transferase N70 lL, and (3) thrombocytopenia (b100 000 lL). Other causes of thrombocytopenia to be considered in pregnancy include HIV infection and systemic lupus erythematosus. A low platelet count may occur in up to 25% of patients with systemic lupus erythematosus secondary to platelet destruction due to antiplatelet antibodies, circulating immune complexes, or other causes. In this case, thrombocytopenia was not detected until after the C-section, partly because of lack of prenatal care. The diagnosis of ITP was easy to make in the setting of severe thrombocytopenia and otherwise normal coagulation parameters, and the patient responded well to the steroid therapy. Mortality from complications of general anesthesia was shown to be 16.7 times that of regional anesthesia [4]. Inability to intubate or ventilate the lungs and pulmonary aspiration are the leading causes of maternal death related to anesthesia. Therefore, spinal and epidural anesthesia have become the anesthetic techniques of choice in obstetric practice whereas general anesthesia is generally reserved for emergency situations.
68 Because of the urgency of the situation, the history of bleeding, and the unremarkable airway evaluation, we chose to perform general anesthesia. Had the scenario been one of an anticipated difficult airway, regional anesthesia would have been considered. In this case, a transfusion of platelets should have been given immediately before the procedure to minimize the risk of development of an epidural hematoma. The absolute number of platelets considered safe for the performance of a neuroaxial block is still debatable. Regional anesthesia has been administered safely to patients with mild thrombocytopenia (platelet count N69 000/lL) [5]. Although unusual bleeding was not noticed during the cesarean delivery, the safety of regional anesthesia would still be questionable in this case. This case reflects the importance of an appropriate preoperative evaluation before any anesthetic encounter. Even during an emergency, the anesthesia provider has to take a thorough history to detect any abnormalities on the physical or the laboratory examination that would be crucial to determine the best anesthetic plan for that patient. The
J.S. Ranasinghe et al. choice of anesthesia is dictated by many factors, and in controversial situations, it is for the anesthesia consultant to weigh the risks vs benefits of each technique.
References [1] McCrae KR, Samuels P, Schreiber AD. Pregnancy-associated thrombocytopenia: pathogenesis and management. Blood 1992;80: 2697 - 714. [2] Rath FA. Differential diagnosis of thrombocytopenia in pregnancy. Zentralbl Gynakol 2001;123:80 - 90. [3] Laros Jr RK. Coagulation disorders and hemoglobinopathies in the obstetric and surgical patient. In: Hughes SC, Levinson G, Rosen MA, editors. Shnider and Levinson’s Anesthesia for Obstetrics. 4th ed. Philadelphia7 Lippincott Williams and Wilkins; 2001. p. 347. [4] Hawkins JL, Koonin LM, Palmer SK, Gibbs CP. Anesthesiarelated deaths during obstetric delivery in the United States, 19791990. Anesthesiology 1997;86:277 - 84. [5] Beilin Y, Zahn J, Comerford M. Safe epidural analgesia in thirty parturients with platelet counts between 69,000 and 98,000 mm3. Anesth Analg 1997;85:385 - 8.