Clinical characteristics and course of CD8+ cytotoxic variant of mycosis fungoides: a case series of seven patients

Abstract

Summary Background Fewer than 5% of cases of mycosis fungoides (MF) present with a cytotoxic/suppressor CD8+ phenotype which, despite immunophenotypic similarities with CD8+ aggressive lymphomas, is regarded as a phenotypic variant of MF. Poikilodermatous MF showing a CD8+ phenotype has been reported to have a nonaggressive clinical behaviour and a good response to psoralen plus ultraviolet A treatment. Objectives To perform a retrospective study of CD8+ MF cases diagnosed in the skin lymphoma clinic of Andreas Sygros Hospital. Methods We analysed the clinical characteristics, the immunophenotypic and molecular indices, as well as the clinical course of these patients. Results Seven cases of CD8+ MF (6·5% of all cases of cutaneous T‐cell lymphoma) were diagnosed during 2002–2007. One of seven patients had stage IA, five stage IB and one stage IIB disease. Clinical characteristics were variable: four of seven patients presented with poikilodermatous plaques (in one of them lesions of lymphomatoid papulosis with CD8+ phenotype coexisted), one patient with classic MF, one with plantar MF and one with follicular MF. The time period between disease onset and diagnosis was long for most patients (up to 33 years). All patients received the recommended treatment according to TNM staging. Five of seven patients had complete remission, one partial response and one stable disease. Conclusions Special clinical characteristics, such as hyperpigmentation and poikiloderma, are often noted in CD8+ MF cases. In our series CD8+ MF presented with a long‐standing disease and indolent course suggesting that CD8+ cytotoxic immunophenotype may represent a marker of mild biological behaviour.

Hyperpigmented mycosis fungoides: An unusual variant of cutaneous T-cell lymphoma with a frequent CD8+ phenotype

“…1 In our 3 patients with other unusual coexistent MF variants, all lesions in each case had the same immunophenotype. This was not unexpected, as earlier studies reported an overrepresentation of the CD8 1 phenotype and, less often, the CD4 À CD8 À phenotype, in hypopigmented MF, 7,36 and others described the CD8 1 immunophenotype in poikilodermatous MF 28,37 and in chronic pigmented purpuraelike MF. 38 In addition, the CD8 1 phenotype in lesions of PLEVA/PLC in the affected patient agrees with similar findings in some cases of PLEVA and PLC reported previously.…”

“…15 Nevertheless, in 2 previous series describing the clinical features of patients with MF with a CD8 1 immunophenotype, hyperpigmented lesions were mentioned. 17,28 Our present study demonstrates that this relation is bidirectional, namely, that most cases of hyperpigmented MF bear the CD8 1 immunophenotype. This observation can be explained by the suppressor or cytotoxic nature of CD8 1 T cells and their ability to affect neighboring cells, such as melanocytes or basal keratinocytes, thereby causing interface changes, marked melanin incontinence leading to the clinical appearance of hyperpigmentation.…”

Poikilodermatous mycosis fungoides: A study of its clinicopathological, immunophenotypic, and prognostic features

“…26 There was a predominance of a CD8 1 CD4 e phenotype detected on immunohistochemistry (15/40; 38%), which suggests that there may be an overrepresentation of CD8 1 cases relative to classic MF with previous studies reporting the CD8 1 phenotype in just 6.5% of MF. 23 The association of poikiloderma with a CD8 1 phenotype has been previously noted in the literature: there is one case report of poikilodermatous MF with a CD8 1 phenotype and 4 similar cases within a series of 7 patients with MF all with CD8 1 phenotype. 23,24 The recent literature suggests that a CD8 1 phenotype may be seen in early stage MF but has a similar prognosis to the classic CD4 1 CD8 e phenotype.…”

“…21 We found that the duration of clinical symptoms before diagnosis was longer in our study with a median duration of 10 years compared with an average of 6 to 7 years for classic MF. 22 A long disease history (14,15, and 33 years) has also been reported in 3 of 4 patients who presented with poikilodermatous plaques and CD8 1 CD4 e immunophenotype 23 and in a single case report of a patient with poikilodermatous MF and CD8 1 immunophenotype who presented with a 17-year history of disease. 24 As in classic MF, most (88%) patients had early stage disease (IA-IIA) at diagnosis.…”

CD8-positive mycosis fungoides: a case report

“…Szisztematizáció, agresszív terjedés nem észlel-hetô. Egy hét beteg adatait feldolgozó esetközlés alapján a CD8-pozitív mycosis fungoides miatt vizsgált betegek többségében poikilodermatosus tüneteket lehetett látni, PUVA illetve NB-UVB terápiára, valamint lokális kortikoszteroid és PUVA kezelésre jól reagáltak a tünetek, bexarotene-re váltás TNM beosztásnak megfelelôen egy betegnél volt szükséges (10).…”