[Therapeutic effect of cyclosporin A combined with methylprednisolone pulse therapy on hemophagocytic syndrome with the central nervous system involvement].

Two pediatric cases of hemophagocytic syndrome (HPS) with the central nervous system (CNS) involvement are reported. Case 1, a two-month-old girl with primary hemophagocytic lymphohistiocytosis, showed high fever, skin rash and hemorrhagic diathesis followed by convulsion, impairment of consciousness and involuntary movements. Case 2, an eight-year-old boy, complained of visual hallucination in the course of measles complicated with interstitial pneumonitis. MRI of the two cases disclosed multiple T2-hyperintense areas in the CNS. Both clinical symptoms and MRI findings of these cases significantly improved in response to cyclosporin A (CsA) combined with methylprednisolone (mPSL) pulse therapy. Although the pathogenesis of CNS lesions in HPS remains to be elucidated, the etiologic role of hypercytokininemia is obviously important. Because CsA and mPSL have a potent inhibitory effect on the action of cytokines, it is rational and worthwhile to use these agents in the treatment of HPS with CNS involvement.