2017 Volume 54 Issue 2 Pages 143-148
We report a case of refractory secondary hemophagocytic lymphohistiocytosis (HLH) manifesting severe liver dysfunction following Kawasaki disease (KD-HLH). A 1-year-old girl admitted for her first KD episode experienced remission after initial treatment with intravenous immunoglobulin (IVIG) and prednisolone (PSL). Symptoms similar to recurrent KD, including severe liver dysfunction, anemia, thrombocytopenia, and elevated soluble interleukin 2 receptor (sIL-2R), occurred 28 days after the initial treatment, fulfilling five of the eight HLH criteria. Her condition deteriorated rapidly. She required intensive care for multiorgan failure. Steroid pulse therapy was followed by dexamethasone, and IVIG was readministered. Cyclosporine A was also administered owing to worsening symptoms and increased sIL-2R levels. She is currently well without sequelae.
Some previous reports of cases of KD-HLH mentioned severe manifestations and acute exacerbation such as those in our case. Early recognition and treatment are important when atypical KD manifestations occur or when the patient fails to respond to conventional KD treatment.
