Hemolytic crisis in a patient treated with eculizumab for paroxysmal nocturnal hemoglobinuria possibly triggered by SARS
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LETTER TO THE EDITOR
Hemolytic crisis in a patient treated with eculizumab for paroxysmal nocturnal hemoglobinuria possibly triggered by SARS-CoV-2 (COVID-19): a case report Hannah Schüller 1 & Franziska Klein 1 & Michael Lübbert 2 & Eric Peter Prager 1 Received: 18 June 2020 / Accepted: 26 October 2020 # The Author(s) 2020
Dear Editor, COVID-19 caused by the recently discovered zoonotic severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) is characterized by high infectiousness and mild to severe respiratory symptoms, to some extent with life-threatening respiratory distress accompanied by multi-organ failure [1–3]. A 68-year-old Caucasian female patient was admitted to our hospital with confirmed COVID-19. She had been diagnosed with paroxysmal nocturnal hemoglobinuria (PNH) in 1977. Other than that, she has a past medical history significant for hypertension and chronic hepatitis C. For PNH, the patient was started on eculizumab (900 mg/14 days) in 2007. On March 19, 2020, 13 days after receiving her regular treatment with eculizumab, she presented with subfebrile temperatures, mild cough, a sore throat, and diarrhea. Because of her malaise, her eculizumab dose on March 20 was postponed. On March 21, 2020, she received the positive result for SARS-CoV-2 of the nasopharyngeal swab taken 2 days earlier. Five days later, a drop of hemoglobin levels was recognized, and the patient was admitted to our hospital for suspected recurrent hemolytic crisis. Vital signs on admission were within normal range. Physical examination showed no abnormalities. Laboratory parameters on admission on March 26 were suggestive for hemolytic anemia and mild inflammation
Hannah Schüller and Franziska Klein contributed equally to this work. * Eric Peter Prager [email protected] 1
Department of Nephrology, University Medical Center, Faculty of Medicine, University of Freiburg, Freiburg, Germany
2
Department of Hematology, Oncology & Stem Cell Transplantation, Faculty of Medicine, University of Freiburg, Freiburg, Germany
(hemoglobin 6.5 g/dl (last documented 10.6 g/dl on March 20)), serum LDH 457 U/l, haptoglobin 63 mg/dl (ref. val. 30–200 mg/dl), reticulocytes 276,000/μl (ref. val. 19,800–80,700/μl), free hemoglobin 5.6 mg/dl (ref. < 5 mg/ dl), total bilirubin 2.7 mg/dl, direct bilirubin 1.0 mg/dl, CRP 25 mg/l (normal range < 3 mg/l)). Whether her normal haptoglobin level and the only slightly elevated free hemoglobin level represents extravascular hemolysis or an already stabilized intravascular hemolysis situation at the time of admission remained unclear. Within several days of admission, she developed thrombocytopenia with a minimum of 102,000/μl, neutropenia with a minimum of 420/μl neutrophils, and lymphopenia with a minimum of 800/μl lymphocytes. Despite laboratory findings of beginning pancytopenia, she did not show any other clinical signs typical of PNH. We continued treatment with eculizumab on day 7 (March 26) and 20 (April 4) after positive SARS-CoV-2 testing at the usual dose. Additionall
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