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Achievement of the longest survival of paraneoplastic pemphigus with bronchiolitis obliterans associated with follicular lymphoma using R-CHOP chemotherapy

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Abstract

Paraneoplastic pemphigus (PNP) is a rare, fatal, paraneoplastic autoimmune mucocutaneous blistering disease, commonly associated with lymphoproliferative disorders, including malignant lymphomas. Lymphoproliferative disorders associated with PNP are sometimes associated with a serious lung complication, bronchiolitis obliterans (BO). Due to its rarity, guidelines for the management of PNP have not been established. Furthermore, most patients die within 1 year. Here we report the successful treatment of lymphoma-associated PNP and BO using R-CHOP chemotherapy. A 53-year-old Japanese man was admitted to our hospital for severe erosive stomatitis. Computed tomography and positron emission tomography showed multiple lymphadenopathies. He was diagnosed with follicular lymphoma (Ann Arbor stage IVA) and PNP-related BO. The patient underwent six cycles of R-CHOP and an additional cycle of rituximab. Both the erosive stomatitis and the obstructive lung disease persisted, but complete response of the follicular lymphoma was achieved. The patient survived 27 months after diagnosis. Although he died from progressive respiratory failure due to BO, we note that this patient achieved the longest survival of any reported case of PNP-related BO associated with a lymphoproliferative disorder. The present case suggests that intensive immunochemotherapy for underlying lymphoma may improve the prognosis in patients with PNP-related BO associated with lymphoma.

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Acknowledgements

The authors wish to acknowledge Dr. K. Takeuchi and Dr. M. Ohta for their scientific advice. The first author would like to thank her husband, Dr. T. Morishita, for useful discussion.

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Authors and Affiliations

  1. Department of Hematology, Japan Red Cross Fukui Hospital, 2-4-1 Tsukimi, Fukui, Fukui, 918-8501, Japan

    Shin Lee, Keiichi Kinoshita, Shin Imamura & Kenichi Kamiya

  2. Department of Hematology and Oncology, Faculty of Medical Sciences, University of Fukui, Fukui, Japan

    Takahiro Yamauchi

  3. Department of Dermatology, School of Medicine, Kurume University, Fukuoka, Japan

    Norito Ishii

  4. Kurume University Institute of Cutaneous Cell Biology, Fukuoka, Japan

    Takashi Hashimoto

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  1. Shin Lee
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  2. Takahiro Yamauchi
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  4. Takashi Hashimoto
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  5. Keiichi Kinoshita
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  6. Shin Imamura
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Correspondence to Shin Lee.

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Lee, S., Yamauchi, T., Ishii, N. et al. Achievement of the longest survival of paraneoplastic pemphigus with bronchiolitis obliterans associated with follicular lymphoma using R-CHOP chemotherapy. Int J Hematol 106, 852–859 (2017). https://doi.org/10.1007/s12185-017-2305-2

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  • DOI: https://doi.org/10.1007/s12185-017-2305-2

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