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Retroperitoneal fibrosis as manifestation of chronic GVHD after allogeneic hematopoietic SCT

Bone Marrow Transplantation volume 48, pages 467–468 (2013)Cite this article

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GVHD comprises a spectrum of diseases following hematopoietic SCT (HSCT), caused by the graft recognition of allogeneic Ag on host tissue cells with subsequent inflammation and structural tissue damage. The host target cells are mostly epithelial in nature, with only rare cases of manifestations in mesenchymal tissues. GVHD may, however, mimic any and even rare forms of autoimmune disease. Here we report two cases of retroperitoneal fibrosis (RPF) in allogeneic transplant recipients, in both cases associated with extensive chronic GVHD, suggesting that RPF may be considered as a manifestation of GVHD.

The second patient was a 36-year-old man who underwent PBSCT from a matched unrelated donor for blast-phase CML after conditioning with BU, CY and anti-thymocyte globulin. The patient also experienced severe chronic GvHD with mucocutaneous, ocular and hepatic manifestations, starting 6 months after transplantation. Systemic immunosuppressive therapy with systemic prednisolone and tacrolimus at trough-levels of 10 ng/mL resulted in normalization of liver function tests, and topical treatment of keratoconjunctivitis sicca and of the sclerotic skin involvement of the lower legs prevented progression. Four years after transplant, the patient described sacral back pain; at that time the patient exhibited distinct manifestations of chronic GVHD, such as nail dystrophy, dry eyes and sclerotic lesions of both lids, and received continued therapy with low-dose systemic prednisolone and tacrolimus at trough-levels. Radiographic diagnosis showed a diffuse retroperitoneal tissue mass covering the iliac vessels and right ureter, which was associated with hydronephrosis. Magnetic resonance imaging (MRI) confirmed the diagnosis of RPF (Figure 1b). Ureteral stenting was placed and immunosuppressive therapy was re-escalated to a dose of 1 mg/kg body weight, leading to disease stabilization. Repeated imaging studies documented a slight regress of the RPF over the next 3 years. Serum levels of IgG4 were repeatedly within normal range, and pancreas functions were not impaired. Systemic steroid treatment was gradually tapered to a dose of 10 mg daily and tacrolimus was continued, with stable sclerotic manifestations of chronic GVHD at the skin and buccal mucosa.

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Authors and Affiliations

  1. Department of Hematology and Oncology, Comprehensive Cancer Center G-CCC, Georg-August-University of Goettingen, Goettingen, Germany

    C Dicke, J Hasenkamp, W Jung, L Trümper & G G Wulf

  2. Department of Radiology, Georg-August-University of Goettingen, Goettingen, Germany

    A Kertész

  3. Institute of Pathology Oldenburg, Oldenburg, Germany

    R P Henke

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  1. C Dicke
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  2. A Kertész
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  3. R P Henke
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  5. W Jung
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  7. G G Wulf
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Correspondence to C Dicke.

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Dicke, C., Kertész, A., Henke, R. et al. Retroperitoneal fibrosis as manifestation of chronic GVHD after allogeneic hematopoietic SCT. Bone Marrow Transplant 48, 467–468 (2013). https://doi.org/10.1038/bmt.2012.165

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