Abstract

INTRODUCTION

Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder which is one of the cytokine storm syndrome due to uncontrolled hemophagocytosis. Fever, splenomegaly, hyperferritinemia and cytopenias are well-known clinical manifestations of the disease. Hemophagocytic lymphohistiocytosis is classified as familial (primary/genetic) and secondary (acquired) HLH according to mostly infections, malignancies, autoimmune disorders and some metabolic disoreders. The basis of treatment for HLH is immunosuppression and apoptotic chemotherapy. The curative treatment of familial or non-familial persistan HLH are stem cell transplantation.

METHODS

We retrospectively analyzed 46 HLH cases diagnosed and treated in our clinic in terms of diagnostic criteria and treatment plans.

RESULTS

We identified the most frequent recorded diagnostic criterias fever, hemophagocytosis and hyperferritinemia. We found that 46% of the cases needed treatment consisting of dexamethasone, cyclosporine (CsA) and etoposide according to HLH-2004 treatment guideline.

DISCUSSION AND CONCLUSION

As a result; the consideration of HLH in children hectic-peristant fever for early diagnosis and treatment is thought important.