Cladribine treatment of repeatedly-relapsed Langerhans cell histiocytosis: a case report and literature review / 中华儿科杂志

ABSTRACT

<p><b>OBJECTIVE</b>To investigate the salvage therapy for a child with refractory and ( or) repeatedly-relapsed Langerhans cell histiocytosis.</p><p><b>METHOD</b>Data of a patient with Langerhans cell histiocytosis whose disease relapsed repeatedly treated with cladribine was collected and analyzed and the related literature was reviewed.</p><p><b>RESULT</b>The initial symptoms developed 3 months after his birth, multiple systems (skin, skeleton, lung, liver) were involved; he was sequentially treated with LCH-III-Group I, JLSG-96, DAL-HX90 chemotherapeutic regimens. The patient got relapses for more than 3 times, but the disease got completely controlled after being treated with cladribine when the patient was 6 years old. The dosage was 10 mg/(m2 · d) for 4 days, and one course lasted for 28 days, the third to fifth courses of treatment used Arac in combination, the whole treating time lasted for 5 months. The patient remained in persistent remission for 8 months since discontinuation of treatment. "Langerhans cell histiocytosis" "refractory" "cladribine" were used as the key words to search in the data bases CNKI, Wanfangdata and Pubmed, 11 articles were picked. According to the literature, the effective rate of cladribine in treatment of repeatedly relapsing Langerhans cell histiocytosis was 44%-100%, with a good response of 22%-86%, the dose was 5-13 mg/(m2 · d). The main side effects were hematological system damages and infection.</p><p><b>CONCLUSION</b>The effect of commonly used chemotherapeutic regimens is limited for children with refractory and (or) repeatedly-relapsed Langerhans cell histiocytosis and cladribine can be used as an alternative therapeutic option of the salvage therapy.</p>