• Yoshitaka Shimada, Yasushi Nagaba, Hiroyuki Okawa, Kaori Ehara, Shinya Okada, and Hiroaki Yokomori.
• Department of Internal Medicine, Kitasato University Medical Center, Saitama, Japan.
• Medicine (Baltimore). 2022 Oct 28; 101 (43): e31304e31304.

RationaleCoronavirus disease (COVID-19), an infectious disease caused by the severe acute respiratory syndrome coronavirus 2 virus, was reported in Wuhan of China in December 2019. The world is still in a state of pandemic owing to COVID-19. COVID-19 vaccines help our bodies develop immunity against the virus that causes COVID-19 without having to get the illness. Herein, we describe a rare case of a critical disorder, hemophagocytic lymphohistiocytosis (HLH), in a patient with nephritic sclerosis associated with hypertension, following mRNA COVID-19 vaccination. HLH is a life-threatening hyperinflammatory syndrome caused by aberrantly activated macrophages and cytotoxic T cells that may rapidly progress to terminal multiple organ failure.Patient ConcernsAn 85-year-old Japanese woman with chronic renal failure and hypertension was included in this study. Routine laboratory investigations provided the following results: white blood cell (WBC) count, 4.6 × 109/L; hemoglobin (Hb), 8.1 g/dL; platelet count, 27 × 109/L; blood urea nitrogen 48.9 mg/dL, and serum creatinine 3.95 mg/dL. The patient developed malaise, vomiting, and persistent high fever (up to 39.7°C) on the 12th day after receiving the second dose of the vaccine. Initial evaluation revealed neutropenia. The total WBC count was 0.40 × 109/L (Neutrophils 0, Lymphocytes 240/μ, blast 0%); Hb 9.0 g/dL, platelet count 27 × 109/L; and, C Reactive Protein 9.64 mg/dL.DiagnosisFurther tests showed hyperferritinemia (serum ferritin 2284.4 μg/L). Bone marrow examination revealed haemophagocytosis. A provisional diagnosis of HLH associated with the Comirnaty® vaccination was made based on the HLH-2004 diagnostic criteria.InterventionsThe patient was treated with granulocyte colony-stimulating factor and 500 mg methylprednisolone.OutcomesA significant improvement was observed in the patient's condition; the abnormal laboratory results resolved gradually, and the patient was discharged.LessonsThis case serves to create awareness among clinicians that HLH is a rare complication of COVID-19 vaccination and should be considered, especially in patients with a history of chronic renal failure and hypertension.Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.

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