Cancer Res Treat.  2014 Jul;46(3):312-316.

Cyclosporine A as a Primary Treatment for Panniculitis-like T Cell Lymphoma: A Case with a Long-Term Remission

• Lee WS
• Hwang JH
• Kim MJ
• Go SI
• Lee A
• Song HN
• Lee MJ
• Kang MH
• Kim HG
• Lee JH

Abstract

Subcutaneous panniculitis-like T cell lymphoma (SPTL) is a distinctive cutaneous lymphoma characterized by an infiltration of subcutaneous tissue by neoplastic T cells, similar to panniculitis. It is well-established that patients who are diagnosed with SPTL usually respond poorly to chemotherapy, showing fatal outcome. As a first line treatment for SPTL, anthracycline-based chemotherapy was most frequently used. For the treatment of SPTL, the efficacy of cyclosporine A has been recently reported in relapsed SPTL after anthracycline-based chemotherapy. However, it is still not clear whether cyclosporine A can be used as a first-line treatment against SPTL. Here, we report a case of SPTL, which achieved complete remission for nine years after first-line cyclosporine A therapy. This study suggests that cyclosporine A can induce a complete long-term remission as a first-line treatment.

Keyword

Subcutaneous panniculitis-like T cell lymphoma; Cyclosporine A; Remission

MeSH Terms

Cyclosporine*
Drug Therapy
Fatal Outcome
Humans
Lymphoma
Lymphoma, T-Cell*
Panniculitis
Subcutaneous Tissue
T-Lymphocytes
Cyclosporine

Figure

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  Fig. 1. Changes in laboratory findings and body temperature during the cyclosporine A (CsA) treatment. (A) After 5 weeks of treatment initiation with CsA, biochemical test (lactate dehydrogenase, alkaline phosphatase, aspartate aminotransferase, and alanine aminotransferase) was normalized. (B) After 14 days of the CsA therapy, the body temperature returned to normal.

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  Fig. 2. (A) The skin tissue showed diffuse atypical lymphocytic infiltration in the subcutaneous tissue similar to lobular panniculitis. Infiltration to the subcutaneous tissue without involvement of the overlying dermis or epidermis was confirmed (H&E staining, ×40). (A, inlet) The infiltrated lymphocytes had irregular, hyperchromic nuclei and indistinct nucleoli. The atypical lymphocytes were rimmed individual fat cells and some of them showed nuclear karyorrhexis (H&E staining, ×200). (B) Immunohistochemical stain, the atypical lymphocytes were positive for CD3 (×200). (C) CD20 (×200). (D) CD56 (×200). The atypical lymphocytes were negative for both CD20 and CD56.

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  Fig. 3. The summary of previous results of cyclosporine A treatment for subcutaneous panniculitis-like T cell lymphoma (SPTL). (A) Treatment response. (B) Remission duration for each patient from previous reports.

Reference

References

1. Go RS, Wester SM. Immunophenotypic and molecular features, clinical outcomes, treatments, and prognostic factors associated with subcutaneous panniculitis-like T-cell lymphoma: a systematic analysis of 156 patients reported in the literature. Cancer. 2004; 101:1404–13.

2. Parveen Z, Thompson K. Subcutaneous panniculitis-like T-cell lymphoma: redefinition of diagnostic criteria in the recent World Health Organization-European Organization for Research and Treatment of Cancer classification for cutaneous lymphomas. Arch Pathol Lab Med. 2009; 133:303–8.

3. Willemze R, Jansen PM, Cerroni L, Berti E, Santucci M, Assaf C, et al. Subcutaneous panniculitis-like T-cell lymphoma: definition, classification, and prognostic factors: an EORTC Cutaneous Lymphoma Group Study of 83 cases. Blood. 2008; 111:838–45.

4. Foss FM, Edelson RL, Wilson LD. Lymphomas: cutaneous T-cell lymphomas. In : DeVita VT, Hellman S, Rosenberg SA, editors. DeVita, Hellman, and Rosenberg`s cancer: principles and practice of oncology. Philadelphia: Lippincott Williams & Wilkins;2005. p. 2143–58.

5. Rojnuckarin P, Nakorn TN, Assanasen T, Wannakrairot P, Intragumtornchai T. Cyclosporin in subcutaneous panniculitislike T-cell lymphoma. Leuk Lymphoma. 2007; 48:560–3.

6. Go SI, Lee WS, Kang MH, Kim IS, Kim DC, Lee JH. Cyclosporine A treatment for relapsed subcutaneous panniculitis- like T-cell lymphoma: a case with long-term follow-up. Korean J Hematol. 2012; 47:146–9.

7. Mizutani S, Kuroda J, Shimura Y, Kobayashi T, Tsutsumi Y, Yamashita M, et al. Cyclosporine A for chemotherapy-resistant subcutaneous panniculitis-like T cell lymphoma with hemophagocytic syndrome. Acta Haematol. 2011; 126:8–12.

8. Jung HR, Yun SY, Choi JH, Bae SH, Ryoo HM, Kum YS. Cyclosporine in relapsed subcutaneous panniculitis-like T-cell lymphoma after autologous hematopoietic stem cell transplantation. Cancer Res Treat. 2011; 43:255–9.

9. Shani-Adir A, Lucky AW, Prendiville J, Murphy S, Passo M, Huang FS, et al. Subcutaneous panniculitic T-cell lymphoma in children: response to combination therapy with cyclosporine and chemotherapy. J Am Acad Dermatol. 2004; 50(2 Suppl):S18–22.

10. Al Zolibani AA, Al Robaee AA, Qureshi MG, Al Nosian H. Subcutaneous panniculitis-like T-cell lymphoma with hemophagocytic syndrome successfully treated with cyclosporin A. Skinmed. 2006; 5:195–7.

11. Chen R, Liu L, Liang YM. Treatment relapsed subcutaneous panniculitis-like T-cell lymphoma together HPS by cyclosporin A. Hematol Rep. 2010; 2:e9.

12. Tsukamoto Y, Katsunobu Y, Omura Y, Maeda I, Hirai M, Teshima H, et al. Subcutaneous panniculitis-like T-cell lymphoma: successful initial treatment with prednisolone and cyclosporin A. Intern Med. 2006; 45:21–4.

13. Marzano AV, Berti E, Paulli M, Caputo R. Cytophagic histiocytic panniculitis and subcutaneous panniculitis-like T-cell lymphoma: report of 7 cases. Arch Dermatol. 2000; 136:889–96.

14. von den Driesch P, Staib G, Simon M Jr, Sterry W. Subcutaneous T-cell lymphoma. J Am Acad Dermatol. 1997; 36(2 Pt 2):285–9.