CASE 1
A four-year-old previously healthy boy referred with the diagnosis of the Kawasaki Disease. He had no known exposure to SARS-CoV-2. He was hospitalized with the complaint of fever for more than seven days and severe abdominal pain. On admission he had rashes, conjunctival injection, edema on the extremities, abdominal tenderness and hepatosplenomegaly. On abdominal ultrasonography (USG), hepatosplenomegaly, hydrops of gallbladder and free fluid in the peri splenic area were reported. He had normal echocardiographic findings. His SARS-CoV-2 RT-PCR test was negative for two times during 7-day course of illness. Intravenous immunoglobulin (IVIg) (2gr/kg/day) and acetylsalicylic acid were started. On the second day of hospitalization, he had headache and nuchal rigidity. Lumbar puncture (LP) was performed. Cerebrospinal fluid (CSF) biochemistry was within normal ranges for age (CSF protein:40 mg/dL, CSF glucose: 58mg/dL, blood glucose level:72 mg/dL. No cell was detected on the microscopy and culture was negative. Bone marrow aspiration (BMA) revealed hemophagocytosis with all normal lineages. Later on, he was hypotensive and had respiratory distress. Intensive Care Unit (ICU) admission was required, so he was referred to our hospital.
Laboratory work-up was notable for thrombocytopenia and lymphopenia (Table 1). Elevated cardiac enzymes; brain natriuretic peptide (BNP) 1405.5 pg/ml, troponin -I 109.5 ng/L, creatinine kinase myocardial band (CK-MB) 5.2 µg/l were present. High C reactive protein (CRP) and procalcitonin level were present. He had hypoalbuminemia, hypofibrinogenemia, high D-dimer and ferritin level (Table 1) (Figure 1 a).
Echocardiographic findings were normal [Ejection fraction (EF): 70%] with no coronary artery abnormalities. Bone marrow aspiration smears from referring hospital were re-evaluated and revealed hemophagocytic cells with no malignant cells (Figure 2 A&B). His SARS-CoV-2 IgG level was high; 8.9.
These features were compatible with MIS-C, methylprednisolone (2mg/kg/day) and anakinra (4mg/kg/day) were administered. He was started on antibiotics (vancomycin, ceftriaxone, and clarithromycin), favipiravir and enoxaparin (1mg/kg/day). On the fifth day of the treatment abnormal physical findings were resolved. He was well with improved laboratory findings.