Since the beginning of the severe SARS-CoV-2 pandemic, an increasing number of countries reported cases of a systemic hyperinflammatory condition defined as multi-system inflammatory syndrome in children (MIS-C). The clinical features of MIS-C can be an overlap of Kawasaki Disease (KD), Toxic Shock Syndrome (TSS), Macrophage Activation Syndrome (MAS), or have often an acute abdominal presentation. Intravenous immunoglobulin (IVIG) is recommended as first line therapy in KD. Recent evidence suggests intravenous immunoglobulins (IVIG) resistance in some cases of SARS-CoV-2 related MIS-C, thereby questioning the benefit of immunomodulators such as IL-1 or IL-6 blocking agents. We report on a cohort of 6 Swiss children with SARS-CoV2 related MIS-C presenting with clinical features compatible with Incomplete KD and Toxic Shock Syndrome associated to a cytokine storm. Serum cytokine profile investigations showed increased IL1RA levels (8 to 22-fold) in 5 of the 6 patients (one patient had not been tested), whereas, IL-6 serum levels were increased only in the 3 patients of the 6 who were tested. With exception of one patient who had only benefited by Anakinra, all patients received at least one dose of IVIG. One patient has only received Anakinra with favorable evolution, and three patients had also a steroid treatment. In addition to all this anti-inflammatory medication two patients have also received one dose of anti-IL6. In conclusion, our case series reports on clinical and laboratory findings of most of Swiss cases with MIS-C and suggests the use of Anakinra as an alternative to steroids in these children, most of whom presented with high IL-1RA levels.

A 73-days old infant of 34 weeks' gestation was hospitalized with a co-infection of respiratory syncytial virus (RSV) and Bordetella pertussis (BP). She required invasive ventilation for 9 days in the context of malignant pertussis with persistent hypoxemia and hypercapnia secondary to a leukemoid reaction. Despite an increase of white blood cell (WBC) count up to 70 G/L and ensuing pulmonary hypertension, no hemodynamic compromise occurred. Without clear indication for leukapheresis nor exchange transfusion, an off-label treatment with hydroxyurea was given for 5 days with gradual decrease of WBC count, without any complication and hospital discharge on day 29. To our knowledge, no effective therapy for malignant pertussis has been described in the literature and complications are frequent with leukoreduction procedures. We discuss an alternative to invasive procedures in young infants to fulfill the need to decrease rapidly leukocyte counts in a leukemoid reaction associated with Bordetella pertussis infection. To our knowledge, hydroxyurea has never been used in malignant pertussis but is a well-known medication for oncologic and hematologic diseases such as acute myeloid leukemia or sickle cell anemia. Its effects in this setting are not well understood but the positive outcome in our patient supports the need for further studies.

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