Hemophagocytic lymphohistiocytosis single-center experience


Toret E. , Ay Y., Hilkay Karapinar T., Oymak Y., Bulut M., Vergin C.

IZMIR DR BEHCET UZ COCUK HASTANESI DERGISI, cilt.8, sa.1, ss.64-68, 2018 (ESCI İndekslerine Giren Dergi) identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 8 Konu: 1
  • Basım Tarihi: 2018
  • Doi Numarası: 10.5222/buchd.2018.064
  • Dergi Adı: IZMIR DR BEHCET UZ COCUK HASTANESI DERGISI
  • Sayfa Sayıları: ss.64-68

Özet

Objective: Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder which develops as a result of 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) where an underlying disease is present. Among acquired causes infections, malignancies, autoimmune disorders and some metabolic disorders may be enumerated. The basis of treatment for HLH is immunosuppression and apoptotic chemotherapy. The curative treatment alternative for familial or non-familial persistent HLH is stem cell transplantation.& para;& para;Methods: We retrospectively analyzed 46 HLH cases diagnosed and treated in our clinic in terms of diagnostic criteria and treatment plans.& para;& para;Results: We identified the most frequently recorded diagnostic criteria as 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.& para;& para;Conclusion: As a result, we think that search for HLH in children who presented with hectic-persistent fever is important for the early diagnosis and treatment of HLH.