Fatal Dengue in Patients with Sickle Cell Disease or Sickle Cell Anemia in Curaçao: Two Case Reports

Abstract

Presentation of Cases Case 1 A ten-year-old girl with sickle cell disease (SCD) (combined heterozygosity for the abnormal hemoglobin S and abnormal HbC; HbSC) presented to our clinic with a history of four days fever, malaise, and a generalized tonic-clonic convulsion. At presentation she was alert, nauseous, and complained of headache and retro-orbital pain. Temperature was 38.0uC, heart rate (HR) 135/min, blood pressure (BP) 94/ 62 mmHg, respiratory rate (RR) 20/min; minor hepatosplenomegaly and upperquadrant abdominal tenderness were present. No further abnormalities were reported on physical examination. Initial lab results revealed a moderate increase of CRP, anemia, thrombocytopenia, prolonged activated partial thromboplastin time (aPTT) and prothrombin time (PT), increased liver enzymes, and decreased albumin and hematocrit (Ht) (Table S1). On day two, during defervescence she became hypotensive and was treated with fluid resuscitation and inotropic agents. Blood products were transfused to correct anemia and thrombocytopenia. Hemolysis and acute hemorrhage with gastrointestinal, vaginal, and later on venipuncture sites bleeding developed. Splenic sequestration was also considered as the cause of anemia, but could not be confirmed with abdominal ultrasound. Pleural effusion, gallbladder wall thickening, and ascites were seen. On day three, she lost vision. A brain CT scan revealed no abnormalities. Lumbar puncture was deferred and antibiotic treatment was adjusted to cover possible meningitis. Hereafter she developed acute respiratory distress syndrome (ARDS) requiring mechanical ventilation. Dengue serology was positive for IgM (titer: 3.26) and IgG (3.26) (Focus Diagnostics), and RT-PCR showed dengue virus serotype 2 (DENV-2). Her clinical situation deteriorated with an increased hemorrhagic tendency, and blood, clotting products, and clotting factors were administered. In time multiorgan dysfunction syndrome (MODS) developed. Blood cultures remained negative for bacteria, but antibiotics were administered anyway. Candida was cultured and antifungals were initiated. Despite intensive treatment the patient did not recover, disseminated intravascular coagulation (DIC) progressed and kidney function did not return. Consequently further treatment was discontinued and the patient died, 28 days after admission.