Refractory esophageal stricture of esophageal mucous membrane pemphigoid after allogeneic hematopoietic stem cell transplantation

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  • FUJITA Nanako
    Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine
  • YAMASHITA Takaya
    Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine
  • ABE Fumito
    Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine
  • NARA Miho
    Division of Blood Transfusion, Akita University Hospital
  • YOSHIOKA Tomoko
    Department of Hematology and Nephrology, Akita City Hospital
  • KOGA Hiroshi
    Department of Dermatology, Kurume University School of Medicine
  • ISHII Norito
    Department of Dermatology, Kurume University School of Medicine
  • TAKAHASHI Naoto
    Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine

Bibliographic Information

Other Title
  • 難治性食道狭窄を来した同種造血幹細胞移植後の食道粘膜類天疱瘡
  • ナンチセイ ショクドウ キョウサク オ キタシタ ドウシュ ゾウケツ カンサイボウ イショク ゴ ノ ショクドウ ネンマクルイ テンポウソウ

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Abstract

<p>Haploidentical allogeneic hematopoietic stem cell transplantation from her brother was performed on a 41-year-old lady with no prior history of pemphigoid to treat recurrent AML. On day 59 following transplantation, she experienced esophageal stenosis. During immunosuppressive therapy for graft vs. host disease, this condition was controlled with periodic esophageal dilatation (GVHD). Her esophageal stricture, which required periodic dilatation, grew worse after she stopped immunosuppressive therapy because of recurrent AML. The esophageal mucosa was easily hemorrhagic and desquamative. Histologic analysis revealed that the squamous cell layers had been divided. Indirect immunofluorescence was negative for IgG and positive for IgA on the epidermal layers, while direct immunofluorescence showed a linear deposition of IgG on the basement membrane zone. It was determined through immunoblotting utilizing recombinant protein of BP180 C-terminal domain that both IgG and IgA antibodies were present, supporting the diagnosis of mucous membrane pemphigoid with anti-BP180. After allogeneic transplantation, basal epidermal cell destruction by GVHD may result in autoimmune blistering disorders, which expose basement membrane proteins and antigen presentation. A similar mechanism could apply to our situation. For rare GVHD cases, a thorough histological diagnosis is required.</p>

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 64 (2), 107-112, 2023

    The Japanese Society of Hematology

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