Korean J Hematol.  2012 Jun;47(2):146-149. 10.5045/kjh.2012.47.2.146.

Cyclosporine A treatment for relapsed subcutaneous panniculitis-like T-cell lymphoma: a case with long-term follow-up

• Go SI
• Lee WS
• Kang MH
• Kim IS
• Kim DC
• Lee JH

• KMID: 2083610
• DOI: http://doi.org/10.5045/kjh.2012.47.2.146

Abstract

Subcutaneous panniculitis-like T-cell lymphoma (SPTL) is a distinctive lymphoma characterized by an infiltration of subcutaneous tissue by neoplastic cytotoxic T cells. There was no distinction between TCR alpha/beta phenotype and TCR gamma/delta phenotype, and anthracycline-based chemotherapy was usually used for both. Here, we report a patient with recurrent SPTL who achieved a second long-term complete remission by repeated cyclosporine A (CsA) treatment. From 2000 to 2001, the patient received anthracycline-based combination chemotherapy. However, the treatment did not induce long-term remission. In 2002, he received cyclosporine treatment for about 6 months. This resulted in a 5-year remission that ended in relapse in 2008. He received CsA treatment once again and attained a second long-term remission. This case suggests that re-treatment with CsA can be a good option for relapsed SPTL cases and can result in long-term remission.

Keyword

Subcutaneous panniculitis-like T-cell lymphoma; Cyclosporine; Treatment outcome

MeSH Terms

Cyclosporine
Drug Therapy, Combination
Follow-Up Studies
Humans
Lymphoma
Lymphoma, T-Cell
Panniculitis
Phenotype
Recurrence
Subcutaneous Tissue
T-Lymphocytes
Treatment Outcome
Cyclosporine
Lymphoma, T-Cell
Panniculitis

Figure

• 

  Fig. 1 Biopsy of a skin nodule. (A) The skin tissue showed dense lymphocytic infiltrate in lobular panniculitis-like pattern (arrowheads) with focal dermal infiltrations (arrow) (hematoxylin and eosin stain, ×40 magnification). (B) The infiltrated lymphocytes showed atypical features with hyperchromatic, irregular nuclei, and occasional nucleoli. There was fat rimming with atypical lymphocytes (hematoxylin and eosin stain, ×400 magnification). (C) In immunohistochemical stain, the atypical lymphocytes were positive for CD3 (CD3, ×400) and (D) CD8 (CD8, ×400 magnification).

• 

  Fig. 2 Whole body PET-CT (A) At the second relapse, multiple nodules with high signal intensities were noted in multiple subcutaneous and muscular areas. (B) After treatment with CsA, no active lesions were seen in the PET-CT scan. Abbreviations: PET-CT, positron emission tomography-computed tomography; CsA, cyclosporine A.

Reference

1. Gonzalez CL, Medeiros LJ, Braziel RM, Jaffe ES. T-cell lymphoma involving subcutaneous tissue. A clinicopathologic entity commonly associated with hemophagocytic syndrome. Am J Surg Pathol. 1991. 15:17–27.

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. 133:303–308.

3. Willemze R, Jansen PM, Cerroni L, et al. Subcutaneous panniculitis-like T-cell lymphoma: definition, classification, and prognostic factors: an EORTC Cutaneous Lymphoma Group Study of 83 cases. Blood. 2008. 111:838–845.

4. Foss FM, Edelson RL, Wilson LD. DeVita VT, Lawrence TS, Rosenberg SA, editors. Lymphomas: Cutaneous T-cell lymphomas. DeVita, Hellman, and Rosenberg's cancer: Principle & practice of oncology. 2005. 8th ed. Philadelphia, PA: Lippincott Williams & Wilkins;2143–2158.

5. 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. 101:1404–1413.

6. Huang JJ, Cai MY, Ye S, Li ZM, Huang HQ, Lin TY. Clinical analysis of 19 cases of subcutaneous panniculitis T-cell lymphoma with literature review. Ai Zheng. 2009. 28:1093–1099.

7. Henter JI, Horne A, Aricó M, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007. 48:124–131.

8. Rojnuckarin P, Nakorn TN, Assanasen T, Wannakrairot P, Intragumtornchai T. Cyclosporin in subcutaneous panniculitis-like T-cell lymphoma. Leuk Lymphoma. 2007. 48:560–563.