ABSTRACT
Introduction: Macrophage activation syndrome (MAS) resembling hemophagocytic lymphohistiocytosis (HLH) is a potentially life-threatening condition induced by a cytokine storm leading to inappropriate activation of macrophages. MAS and HLH, often observed in autoimmune or malignant disorders as well as infectious conditions have recently also been described in the course of SARS-CoV-2 infection. Here, we describe a patient (pt.) developing MAS after SARS-CoV-2 vaccination. Methods: Diagnosis of MAS and relationship to SARS-Cov-2 vaccination was suspected after thorough exclusion of other causes. Work-up included laboratory tests (infectious diseases screening, autoimmune antibodies, and analyses of cytokines), transthoracic echocardiography, CT and PET-CT scan, and bone marrow cytology Results: 5 days after the vaccination with ChAdOx1 nCoV-19 vaccine (AZD1222), the pt. experienced fever and dyspnea. Amoxicillin did not improve symptoms and the pt. was hospitalized three days later with persistent fever, dyspnea, and chest pain. Laboratory tests revealed leukocytosis, anemia, elevated inflammation parameters (CRP > 350 mg/L;PCT 5.7 ng/mL) increased ferritin (31766 ng/mL) and soluble IL2-receptor (4858 U/L) levels, elevated triglycerides (176 mg/dL) and the absence of any bacteria, virus or fungus in serology and cultures. Bone marrow cytology showed significant hemophagocytosis. Malignancies as a potential cause of MAS were excluded by FDG-PET CT, in which hepato-and splenomegaly were detected. Diagnosis of MAS was made;consequently, treatment with steroids and immunoglobulins was initiated but did not lead to clinical improvement or decrease of ferritin within 10 days. Thus, treatment with the interleukin-1-receptor antagonist anakinra was initiated 12 days after hospitalization and led to rapid clinical response as well as decrease of ferritin and other inflammation markers. Response is ongoing 6 weeks after discharge from hospital. Conclusion: We report a case of MAS developing 5 days after vaccination with the ChAdOx1 nCoV-19 vaccine (AZD1222). After exclusion of all potential other causes, we conclude that MAS in this patient is most likely related to the vaccination. Early diagnosis and immediate interdisciplinary care and treatment initiation were central to prevent organ failure. MAS is a life-threatening condition and should be suspected in any case with fever and hyperinflammatory markers after vaccination for SARS-CoV-2.