Death from Thrombotic Thrombocytopenic Purpura in a Patient Initially Diagnosed With HELLP Syndrome
Abstract Number: 149
Abstract Type: Case Report/Case Series
Introduction: Thrombotic Thrombocytopenic Purpura (TTP) is a rare, life-threatening disease caused by inherited or acquired loss of the von Willebrand factor-cleaving protease ADAMTS-13 (1). It is characterized by thrombocytopenia, altered mental status, renal failure, fever, and microangiopathic hemolytic anemia. TTP resembles the HELLP (Hemolysis, Elevated Liver enzymes and Low Platelets) syndrome (1,2). Proper diagnosis is critical as treatments for these conditions (plasma exchange for TTP and delivery for HELLP syndrome) differ significantly. Here, we report a death from TTP in a patient initially diagnosed with HELLP syndrome.
Case: A 33 year old G2P1 patient with chronic hypertension presented at 37 weeks gestation with tachycardia and elevated blood pressure. Labs were notable for 2+ proteinuria, uric acid 6.2 mg/dl, AST 121 IU/L, and LDH 517 IU/L. She was diagnosed with HELLP syndrome and an urgent repeat cesarean section was performed. Her platelet count continued to trend down and she remained hypertensive and tachycardic. On POD 5, the patient developed left upper quadrant pain and altered mental status. Ten minutes after receiving 50 mg IV meperidine, the patient developed respiratory distress followed by cardiac arrest unresponsive to CPR. Autopsy revealed microthrombi in the vasculature of the heart, lungs, and kidneys and multiple schistocytes on peripheral blood smear. Serum obtained previously that day had undetectable (<5%) ADAMTS-13 activity and the presence of an ADAMTS-13 inhibitor consistent with a diagnosis of TTP.
Discussion: Diagnosing TTP in parturients is difficult. Patients with HELLP, a more common disease, have similar symptoms and lab values, including moderately decreased ADAMTS-13 activity (3). However, platelet counts as low as those seen on POD 3-5 are unusual in HELLP. The lack of a rapid, reliable test for TTP complicated management in this patient.
References: 1. Martin JN, et al. AJOG 2008;199:98-104. 2. Proia A, et al. Ann Hematol 2002;81:210-214.
3. Hulstein JJ, et al. J Thromb Haemost 2006;4:2569-75.