International Journal of Hematology

, Volume 106, Issue 6, pp 852–859 | Cite as

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

  • Shin Lee
  • Takahiro Yamauchi
  • Norito Ishii
  • Takashi Hashimoto
  • Keiichi Kinoshita
  • Shin Imamura
  • Kenichi Kamiya
Case Report


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.


R-CHOP Follicular lymphoma Paraneoplastic pemphigus Bronchiolitis obliterans 



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.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.


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Copyright information

© The Japanese Society of Hematology 2017

Authors and Affiliations

  • Shin Lee
    • 1
  • Takahiro Yamauchi
    • 2
  • Norito Ishii
    • 3
  • Takashi Hashimoto
    • 4
  • Keiichi Kinoshita
    • 1
  • Shin Imamura
    • 1
  • Kenichi Kamiya
    • 1
  1. 1.Department of HematologyJapan Red Cross Fukui HospitalFukuiJapan
  2. 2.Department of Hematology and Oncology, Faculty of Medical SciencesUniversity of FukuiFukuiJapan
  3. 3.Department of Dermatology, School of MedicineKurume UniversityFukuokaJapan
  4. 4.Kurume University Institute of Cutaneous Cell BiologyFukuokaJapan

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