CD8-positive primary cutaneous peripheral T-cell lymphoma, unspecified

ARTICLE

ejd.2011.1564

Auteur(s) : Aristóteles Rosmaninho¹ arisrosmaninho@gmail.com, Madalena Sanches¹, Rosário Alves^1,2, Margarida Lima^2,3, Manuela Selores¹

¹ Department of Dermatology

² Multidisciplinary Consultation for Cutaneous Lymphomas

³ Department of Hematology, Centro Hospitalar do Porto, Rua D. Manuel II, 4099-001 Porto, Portugal

Primary cutaneous peripheral T-cell lymphoma, unspecified (PTLU) represents a rare and heterogeneous group of lymphomas. A CD8+ T-cell phenotype is found in a minority of the cases. The prognosis is generally poor and treatment with chemotherapy is recommended [1].

A 28 year-old female presented for evaluation of a 3 × 1 cm erythematous-violaceous, firm and non-painful nodule on the right thigh. On physical examination she also had a 1 × 1 cm erythematous-brownish nodule on the right arm, multiple violaceous maculopapular lesions on the anterior surface of the legs and a 1 × 0.5 cm erythematous papule on the periungual area of the left hallux (figure 1A, B, C). The lesions appeared almost simultaneously in the previous 2 years and only the thigh lesion progressively increased in size within the first 6 months. She denied fever, night sweats, anorexia or weight loss. A skin biopsy of the thigh nodule showed a neoplastic proliferation of cells infiltrating all the dermis and the subcutaneous fat. A Grenz zone was observed and there was no epidermotropism (figure 1D). Immunohistochemical studies showed the cells to be positive for CD45, CD3, CD5 and CD8 (figure 1E) and negative for CD4, CD30 and CD56. A cellular proliferation index of 20% was observed (figure 1F). The lymphocytes were large and atypical and some had a giant cell and horse shoe appearance (figure 1E). Flow cytometry of the lesion showed the cells to have a CD2+, CD3+, TCR alpha/beta+, CD4-, CD8+, CD16+, CD25-, CD26-, CD27+, CD28+, CD56- and CD57- phenotype. PCR-based molecular studies showed a monoclonal T-cell receptor-gamma gene rearrangement. There were no analytical abnormalities. Peripheral blood and bone marrow flow cytometry studies, bone biopsy and computed tomography scan of the thorax, abdomen and pelvis showed no evidence for systemic disease. We diagnosed a PTLU with a cytotoxic phenotype (CD3+, TCR alpha/beta+, CD8+, CD16+). The case was discussed at the multidisciplinary consultation for cutaneous lymphomas and clinical surveillance alone was decided.

PTLU comprises a rare but heterogeneous group of lymphomas in the WHO-EORTC classification with only 15% presenting a CD8+ phenotype [1, 2]. The diagnosis is of exclusion, since those lymphomas cannot fit into any of the other entities, including epidermotropic CD8+ cutaneous T-cell lymphoma, cutaneous γ/δ T-cell lymphoma, and primary cutaneous small-medium CD4+ T-cell lymphoma [1]. The disease occurs mainly in adults and lesions present as solitary, localized, or more frequently as generalized nodules or tumors [2]. The anatomopathological examination usually shows a mixed cellular composition with atypical lymphocytes, eosinophils, plasmocytes and epithelioid histiocytes. However, overlapping clinicopathological features within this broad group of lymphoproliferative disorders are frequently seen, constituting an additional challenge to its classification and treatment. PTLU are considered to have a poor prognosis, and in the series of Bekkenk MW et al., a median survival of 28 months was reported [2]. Recently, several cases of indolent CD8+ lymphoid proliferations of the ear and face have been reported and were ultimately regarded as a phenotypic variant of primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma [3-5]. In those reports no relapse was observed after surgery or radiotherapy and even spontaneous remission of the lesions occurred. In our report, the aggressive histological findings contrast with the indolent clinical behavior, since 38 months have passed since the appearance of the lesions with no evidence for disease progression. The patient is under clinical surveillance. Our case illustrates the need for a better characterization of this subset of lymphomas to better characterize their clinical behavior and define the best treatment approach. Despite the worrisome histology the disease had an indolent behavior and the over-treatment was avoided.

Disclosure

Financial support: none. Conflicts of interest: none.

References

1. Willemze R, Jaffe ES, Burg G, et al. WHO-EORTC classification for cutaneous lymphomas. Blood 2005 ; 105 : 3768-3785.

2. Bekkenk MW, Vermeer MH, Jansen PM, et al. Peripheral T-cell lymphomas unspecified presenting in the skin: analysis of prognostic factors in a group of 82 patients. Blood 2003 ; 102 : 2213-2219.

3. Petrella T, Maubec E, Cornillet-Lefebvre P, et al. Indolent CD8-positive lymphoid proliferation of the ear: a distinct primary cutaneous T-cell lymphoma?. Am J Surg Pathol 2007 ; 31 : 1887-1892.

4. Suchak R, O’Connor S, McNamara C, et al. Indolent CD8+ lymphoid proliferation on the face: part of the spectrum of primary cutaneous small-/medium-sized pleomorphic T-cell lymphoma or a distinct entity?. J Cutan Pathol 2009 ; 37 : 977.

5. Swick BL, Baum CL, Venkat AP, et al. Indolent CD8+ lymphoid proliferation of the ear: report of two cases and review of the literature. J Cutan Pathol 2011 ; 38 : 209-215.