Funding sources: none.
A case of CD8+ small/medium-sized pleomorphic T-cell lymphoma: clinical and histopathological differential diagnosis
Article first published online: 13 JAN 2014
© 2013 British Association of Dermatologists
British Journal of Dermatology
Volume 170, Issue 1, pages 204–206, January 2014
How to Cite
Kutlubay, Z., Engin, B., Kote, E., Aydin, O., Demirkesen, C. and Oguz, O. (2014), A case of CD8+ small/medium-sized pleomorphic T-cell lymphoma: clinical and histopathological differential diagnosis. British Journal of Dermatology, 170: 204–206. doi: 10.1111/bjd.12549
Conflicts of interest: none declared.
- Issue published online: 13 JAN 2014
- Article first published online: 13 JAN 2014
- Accepted manuscript online: 2 AUG 2013 01:37AM EST
Dear Editor, CD8+ lymphoid proliferation is a rare type of cutaneous T-cell lymphoma. Recently, CD8+ lymphoid proliferations that usually present with solitary, slow-growing nodules on the face or ear, and which resolve after treatment, have raised the possibility of a distinct entity, although the prognostic influence of the CD8 phenotype is still unclear. Irrespective of the histopathological appearance suggesting a high-grade lymphoma in some cases, clinical behaviour should be the main basis for the determination of treatment.[2, 3]
A 60-year-old female patient was admitted with the complaint of progressively increasing red and swollen lesions on her face. Histopathological examination of the initial biopsy, performed 6 years previously, had been compatible with rosacea; a partial regression occurred with rosacea treatments. However, more prominent lesions recurred at almost the same sites after nearly 1 year. A second biopsy exhibited ‘perifollicular lymphoid infiltration’. The patient had no drug history. A partial regression has been noticed with intralesional steroid injections.
Dome-shaped, discrete nodules, bright pink in colour, involved the forehead, nasolabial folds and cheeks – prominently confined to sun-exposed areas (Fig. 1). The main characteristics of the clinical picture suggested a photoinduced dermatosis.
Our first skin biopsy was interpreted as ‘cutaneous lymphoproliferative lesion with CD8+ cytotoxic phenotype'. Peripheral T-cell lymphoma and actinic reticuloid were proposed in the differential diagnosis. We did not detect the characteristic photosensitivity when applying photopatch test using ultraviolet (UV) A and UVB. There was partly diffuse or patchy infiltration within the dermis and subcutaneous fatty tissue, composed of small-to-medium-sized lymphoid cells with oval or round nuclei, dark chromatin and scant cytoplasm. The epidermis was spared and a subepidermal Grenz zone was prominent in the upper dermis. All T cells were CD3+, CD2+, CD5+ and CD8+. While immunoreactivity with granzyme-B and TIA-1 was identified in most of the lymphoid cells, they were negative for CD4, CD7 and CD56. The proliferative activity with Ki67 was 5% (Fig. 2). A clonal T-cell population was not encountered using BIOMED 2 (http://cordis.europa.eu/biomed/).
The patient was investigated for systemic involvement. β2-Microglobulin was 1·749 mg L−1; human immunodeficiency virus was negative; other biochemical tests, and thoracal and neck tomography scans were normal. There was no association with Epstein–Barr virus. Using flow cytometry, the cell count was very low; in CD45/side scatter graphs, the percentages of lymphocyte, granulocyte, monocyte and CD45– cells were 23%, 69%, 5% and 2%, respectively; in forward scatter/side scatter graphs, the percentage of T lymphocytes was 66%. There was an increase (36–37%) of natural killer cells and the ratio of CD4+/CD8+ T lymphocytes was normal.
Topical steroid and retinoid–psoralen plus UVA (PUVA) provided a remarkable resolution of lesions after 4 weeks. Because new formation of infiltrated papular lesions was detected, the retinoid was stopped and a combination therapy of PUVA plus methotrexate 25 mg weekly and subcutaneous interferon alfa-2b, 3 × 4·5 mU weekly was started. After the application of 30 sessions of PUVA and five weekly doses of methotrexate, and 15 doses of interferon alfa-2b, we did not observe enough regression. Therefore, all the treatment modalities were stopped and the application of 18 sessions of radiotherapy alone using 6 mV energy with 17 × 180 Gy provided complete healing, and the patient was in full recovery after 1 year.
The most prominent clinical feature of the present case is the localization of the lesions, suggesting a UV-induced skin disorder. But the clinical features did not indicate actinic reticuloid, granulomatous rosacea or sarcoidosis, which were proposed as the preliminary diagnoses. Mycosis fungoides was also considered because lesions were slowly progressing and histopathological examination showed T-cell phenotypes. However, the absence of epidermotropism or folliculotropism, the presence of CD8+ T cells and the clinical findings excluded the probability of mycosis fungoides.
Certain cases do not meet the enrolment criteria for the general classification of primary cutaneous T-cell lymphomas. Some of these have been termed ‘peripheral T-cell lymphoma, unspecified type’. This group generally follows an aggressive course and primary cutaneous CD8+ T-cell lymphomas localized to the ear, nose and, rarely, the face are included in this group.[4-8] The cases termed CD8+ T-cell lymphoma of the ear by Petrella et al. have been reported to follow an indolent course.
While most cases present with solitary nodules on the head or neck, the present case had multiple nodules involving the whole face. Khamaysi et al. described a case with a pruritic nodule on the patient's right foot as pleomorphic CD8+ small/medium size cutaneous T-cell lymphoma. Similarly, Kempf et al. published three cases with the same lymphoproliferations in the skin at extrafacial sites. These cases were similar to our case in clinical progress but with different localizations; the lesions were totally cured with radiotherapy.
The primary cutaneous cytotoxic T-cell lymphoma with small-to-medium-sized cells, and its slowly progressive course with the absence of epidermotropism should constitute a different group of T-cell lymphomas from a prognostic point of view. There are some primary cutaneous lymphomas with interesting features that do not completely fit into the classification from the aspect of clinical presentation, course, histopathological and immunophenotypic features. We suggest nonaggressive treatment strategies for such lymphoproliferative lesions.