Lymphomatoid papulosis after allogenic stem cell transplantation

ARTICLE

Auteur(s) : Sue Kyung Kim, You Chan Kim

Department of Dermatology, Ajou University School of Medicine, 5 Wonchon-Dong, Yeongtong-Gu, Suwon 443-721, South Korea

Post-transplantation lymphoproliferative disorders (PTLD) are relatively common lymphoid proliferation or lymphomas that develop in a recipient of a solid organ or bone marrow allograft. However, patients undergoing allogeneic stem cell transplantation have a PTLD incidence of about 1%. Moreover most examples are of B cell origin, and CD30+ T cell PTLDs are very rare [1].

We report a case of lymphomatoid papulosis (LyP) in an immunosuppressed patient who had received an allogenic stem cell transplantation. To our knowledge, only a few cases of posttransplantation LyP have been reported [2-4]. Our patient was a 49-year-old woman with a history of aplastic anemia who had received an allogenic stem cell transplantation six months before her visit to our clinic. The patient had received cyclosporine 75 mg for GVHD prophylaxis after stem cell transplantation for about six months. About one month before, the medication was changed to cyclosporine 50 mg with prednisolone 10 mg, because of elevated liver enzyme levels. She had prophylactic antiviral treatment just before and after she had stem cell transplantation. The patient had no history of previous malignancy. She presented with multiple zosteriform erythematous papules on her left arm, which had begun about one month previously (figure 1A). Because of her clinical presentation and immunosuppression state, we initially suspected herpes zoster; however, the lesions were not improved with sufficient oral antiviral treatment. We performed a biopsy and the specimen showed a wedge shaped perivascular lymphoid infiltrate with cytologic atypia (figure 1C). In the immunohistochemical study, the atypical lymphocytes expressed CD30 and CD3 antigens, but not CD20, CD34, CD68, and myeloperoxidase (figure 1D). Immunohistochemical staining to herpes simplex virus (HSV) and varicella-zoster virus were all negative. Polymerase chain reaction analysis for HSV DNA was also negative. Serologic tests for human immunodeficiency virus types 1 and 2, Epstein-Barr virus (EBV), and hepatitis viruses were all negative. The histopathological and immunophenotypical findings established the diagnosis of LyP. She was treated with topical steroids without discontinuation of the immunosuppressive agent and the cutaneous lesion showed significant improvement after four weeks. After six months, she had no recurrence, but showed post-inflammatory hyperpigmentation (figure 1B).

The pathogenesis of PTLD is complex and probably multifactorial. Drug-induced immunodeficiency and chronic antigenic stimulation exerted by the recipient’s tissue play an important role. Other risk factors include the type of transplanted organ, the recipient and donor EBV serological status, the type of disease leading to transplantation, and the type, length, and intensity of immunosuppressive drug treatments [1]. The incidence of PTLDs varies from 1% to 11% in solid organ transplant recipients. In bone marrow transplanted patients, the PTLD incidence is lower than 1% [1]. It is possible that patients with organ transplants as opposed to bone marrow transplants simply require prolonged immunosuppressive treatment and that this is a possible cause of an increasing risk of PTLD. Recently, the possibility of preventing transplant rejection has been radically improved by the introduction of new immunosuppressive drugs such as cyclosporine, tacrolimus, muromonab, and mycophenolate mofetil [1]. Ciancio et al. [5] investigated PTLD incidence in transplant recipients treated with different types of immunosuppressive regimens over 18 years and observed a greater prevalence of PTLDs in patients treated with new immunosuppressive drugs than in those treated only with corticosteroids. There are several CD 30+ lymphoproliferative disorder cases who received cyclosporine therapy. [6] Therefore, in our case, the immunocompromised status and cyclosporine treatment itself could be possible causes of PTLD.

In conclusion, we present a rare case of a PTLD presenting as LyP in an allogenic stem cell transplantation patient. Dermatologists should consider LyP in the differential diagnosis of cutaneous neoplastic and infectious conditions that arise in immunosuppressed patients after transplantation.

Acknowledgements

References

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