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Transfusion-Transmitted Plasmodium falciparum in a Patient With Sickle Cell Hemoglobinopathy*

Background: Sickle cell disease is a genetic hemoglobinopathy that often presents as a vaso-occlusive crisis. Moyamoya disease, which is a complication of sickle cell disease, is a progressive, occlusive cerebrovascular disorder that affects the circle of Willis and the arteries that feed it (1). Objective: To describe a case of transfusion-transmitted Plasmodium falciparum infection resulting in multiorgan failure in a patient with sickle cell anemia. Case Report: An 18-year-old African American man with a history of essential hypertension, moyamoya disease, and homozygous sickle cell disease presented with fever, chills, and generalized body pain of 1 week's duration. He had been receiving monthly transfusions of packed red blood cells for conservative management of his sickle cell disease for 2 years. He was born in the United States and moved from Maryland to Georgia 1 year ago. He had no history of travel outside the United States or any sick contacts. The patient was hospitalized for suspected vaso-occlusive crisis. On initial examination, he was drowsy and in moderate distress, febrile, and tachycardic and tachypneic but otherwise hemodynamically stable. The chest and legs were diffusely tender on palpation. Blood testing showed a hemoglobin level of 93 g/L, leukocyte count of 11.1109 cells/L with 84% neutrophils, and a reticulocyte count of 0.073. Sickle hemoglobin levels were 35% on electrophoresis. Findings on chest radiography were normal, and results of blood and urine cultures were negative. We administered hypotonic fluids and opioid analgesic therapy as well as vancomycin and piperacillintazobactam to provide broad-spectrum coverage for presumed infection. The patient remained febrile and developed increasing somnolence and hypotension. He was intubated for airway protection, and vasopressor therapy was initiated for septic shock. Computed tomography of the head showed no acute findings. Antibiotic coverage was changed to meropenem, linezolid, and doxycycline. Somnolence was attributed to hepatic encephalopathy because the patient developed substantially elevated serum aminotransferase levels and hyperbilirubinemia (levels of aspartate aminotransferase, 1979 U/L; alanine aminotransferase, 668 U/L; alkaline phosphate, 0.8 kat/L; total bilirubin, 205.2 mol/L [12.0 mg/dL]; direct bilirubin, 143.7 mol/L [8.4 mg/dL]; and serum ammonia, 111 mol/L [155 g/dL]; international normalized ratio was 2.2). He also developed hemolytic anemia (hemoglobin level, 66 g/L, and lactate dehydrogenase level, 45.7 kat/L), progressive thrombocytopenia with the platelet count decreasing from 399109 cells/L at admission to 48109 cells/L, and disseminated intravascular coagulopathy (prothrombin time, 25.8 s; activated partial thromboplastin time, 45.4 s; fibrinogen level, 6.6 mol/L; and D-dimer level, 124.8 nmol/L). The creatinine level increased to 398 mol/L (4.5 mg/dL), and findings on urine microscopy were consistent with acute tubular necrosis. Results of serologic testing for hepatitis, HIV, cytomegalovirus, Epstein Barr virus, Mycoplasma, and Legionella were negative. We believed that end-organ damage was caused by the vaso-occlusive crisis, and the patient had exchange transfusion of 7 units of packed red blood cells and hemodialysis. On hospital day 6, intraerythrocyte inclusions were incidentally seen on peripheral blood smear (Figure). A malaria thick smear and polymerase chain reaction assay confirmed the diagnosis of P falciparum infection. With no history of travel and inclusions of mostly nonsickled cells on the peripheral smear, transfusion-transmitted malaria was considered probable. This case was reported to the Centers for Disease Control and Prevention (CDC). Figure. Peripheral smear showing intraerythrocyte inclusions ( arrows ). Although CDC guidelines recommend intravenous quinidine as first-line treatment of severe malaria (2), this therapy was avoided because of a prolonged rate-corrected QT interval on electrocardiography. Instead, we administered intravenous artesunate for 7 days. The patient improved, was extubated on hospital day 10, and was transferred out of the intensive care unit. He was discharged on hospital day 42. The CDC conducted a retrospective review of transfusion records, including the exchange transfusion received during the hospital stay, and determined that the patient received a transfusion contaminated with P falciparum 3 weeks before admission. Discussion: Transfusion-transmitted malaria is uncommon in the United States (3, 4). However, persons who require many blood transfusions, such this patient, are at increased risk and may have severe complications that can be falsely attributed to the underlying disease.