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Successful treatment with 2-chlorodeoxyadenosine of refractory pediatric Langerhans cell histiocytosis with initial involvement of the gastrointestinal tract

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Abstract

Gastrointestinal (GI) tract involvement in Langerhans cell histiocytosis (LCH) is extremely rare. Langerhans cell histiocytosis with GI tract involvement (GI-LCH) is frequently associated with multi-system disease, and usually presents with severe systemic symptoms, such as protein-losing enteropathy (PLE). Although the GI tract is not included among the organs at risk, the prognosis of GI-LCH is poor, and no effective chemotherapeutic regimen has been identified. Here, we report an infant case of primary refractory GI-LCH with PLE that showed marked improvement in response to 2-chlorodeoxyadenosine (2-CdA) therapy with no severe adverse events, even under conditions of deteriorating general health. The present findings indicate that 2-CdA may be effective for refractory GI-LCH with PLE. Further studies are warranted to determine the optimal therapeutic strategies for GI-LCH with PLE.

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Authors and Affiliations

  1. Department of Pediatrics, Kyoto Prefectural University of Medicine, 465, Kajii-cho, Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto, 602-8566, Japan

    Azusa Mayumi, Toshihiko Imamura, Kenichi Sakamoto, Takeshi Ota, Shinya Osone & Hajime Hosoi

  2. Department of Pediatric Hematology and Oncology, Hyogo Prefectural Amagasaki General Medical Center, Amagasaki, Japan

    Ikuya Usami

Authors
  1. Azusa Mayumi
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  2. Toshihiko Imamura
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  3. Kenichi Sakamoto
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  4. Takeshi Ota
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  5. Shinya Osone
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  6. Ikuya Usami
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  7. Hajime Hosoi
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Correspondence to Azusa Mayumi.

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Mayumi, A., Imamura, T., Sakamoto, K. et al. Successful treatment with 2-chlorodeoxyadenosine of refractory pediatric Langerhans cell histiocytosis with initial involvement of the gastrointestinal tract. Int J Hematol 110, 756–762 (2019). https://doi.org/10.1007/s12185-019-02711-0

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  • DOI: https://doi.org/10.1007/s12185-019-02711-0

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