演題詳細

ポスター / Poster

ポスター 51 (Poster 51) :小児血液 (Pediatric Blood)

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日程
2013年10月11日(金)
時間
16:50 - 17:50
会場
ポスター会場 / Poster (ロイトン札幌 3F エメラルドABCD)
座長・司会
遠藤 幹也 (Mikiya Endo):1
1:岩手医科大学 小児科学講座
 
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A case with refractory langerhans cell histiocytosis responded to combination of 2-CdA and Ara-C

演題番号 : PS-1-392

小林 千佳 (Chika Kobayashi):1、大川 哲平 (Teppei Okawa):1、富澤 大輔 (Daisuke Tomizawa):1、手束 真理 (Mari Tezuka):1、今井 耕輔 (Kohsuke Imai):1、高木 正稔 (Masatoshi Takagi):1、梶原 道子 (Michiko Kajiwara):1、長澤 正之 (Masayuki Nagasawa):1、森尾 友宏 (Tomohiro Morio):1、水谷 修紀 (Shuki Mizutani):1

1:Dept. Pediatrics, Tokyo Medical and Dental University. Japan

 

[Introduction] In recent years, some refractory cases of LCH have been treated successfully with a combination of cladribine (2-CdA) and cytarabine (Ara-C). However, indications and safety of such therapy has not been established so far. We report here an infant who was refractory to standard therapy and was steroid dependent, but could be treated safely with 2-CdA and Ara-C combination. [Case Report] A 7-month-old female presented with refractory eczema, thymoma, hepatosplenomegaly, pancytopenia, and DIC. The diagnosis of multisystem LCH with high-risk organ involvement (LCH MS-RO+) was made by biopsy of a skin lesion. Initially, the patient was treated according to the JLSG-02 protocol Induction A, but a mere 10 days later, the therapy had to be changed to Induction B2, which resulted in a slight decrease of hepatosplenomegaly (NR). However, reduction of PSL exacerbated her symptoms and the patient suffered from recurrent fevers while she was being treated with intermediate dose of MTX. Finally, conventional treatments were discontinued and a combination of 2-CdA and Ara-C was initiated. She completed 2 courses without any severe infections and the thymoma had markedly regressed. Now she is in remission, receiving maintenance therapy. [Discussion] Combination therapy of 2-CdA and Ara-C was effective for the current case with refractory LCH. Further studies need to be carried out in order to clarify indications for the use of this therapy, with regard to not only an efficacy but to a safety including late effects.

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