Abstract

The definition of refractory Langerhans cells histiocytosis(LCH)is risk organs(bone marrow, liver, spleen)involvement and the first-line treatment failure.It has a very poor prognosis.The survival depends on timely and effective salvage treatment.Currently the treatment for refractory LCH includes isolated 2-chlorodeoxyadenosine, 2-chlorodeoxyadenosine combined with high-dose cytarabine, indomethacin, hematopoietic stem cell transplantation and targeted therapy.In this paper, the advances in treatment of the refractory LCH in children were reviewed. Key words: Refractory Langerhans cell histiocytosis; Therapy; 2-chlorodeoxyadenosine; Indomethacin; Children

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