Cutaneous T-cell lymphoma: clinicopathological relationships, therapy and survival in ninety-two patients

Abstract

Clinicopathological findings, methods of treatment and survival in ninety-two patients with cutaneous T-cell lymphoma (CTCL) (eighty-five mycosis fungoides and seven Sézary syndrome), seen in the Leiden University Hospital between 1974 and 1980, are reported. All patients were staged and treatment was given accordingly. Patients without signs of extracutaneous disease (74%) were treated with eigher total-skin electron-beam irradiation (E beam), topical mechlorethamine (HN2) or PUVA. Topical HN2 and E beam had an equal effect on survival. Electron-beam induced a higher number of initial complete remissions than HN2, but fewer patients relapsed while on maintenance treatment with HN2. In the patients with lymph node and/or visceral involvement, E beam followed by systemic chemotherapy--cyclophosphamide, vincristin (Oncovin) and prednisone (COP)--gave better results than topical therapy or COP alone. Survival was influenced most by the type of skin lesion, the presence or absence of lymph node and/or visceral involvement, and the ability to induce a complete remission initially.

Mycosis Fungoides

“…For the survival rates for the different stages of MF, the overall and disease-specific survival rates at 5 and 10 years for patients with stage Ia and Ib MF (Table 2) were similar to those reported in previous studies. 4,5,[10][11][12][13][14] In this study, we did find a difference in survival between those with stage Ia and those with stage Ib MF, but this did not reach statistical significance. The fact that disease progression was much more frequent in patients with stage Ib MF than in those with stage Ia MF suggests that with longer follow-up, the difference in survival might become statistically significant.…”

“…Whether patients with enlarged, but histologically uninvolved, lymph nodes (stage II) have a more unfavorable prognosis compared with patients without clinically enlarged lymph nodes is a matter of controversy. 4,11,13 In the present study, patients with stage II disease had a significantly lower survival rate than patients with stage I MF (PϽ.001). Previous studies 27 suggested that T-cell receptor gene rearrangement analysis is a more accurate and objective method of diagnosing early lymph node involvement in patients with MF than routine histological features.…”

“…This 4 Translation into the TNM classification 18 is as follows; Ia, T1 N0 M0; Ib, T2 N0 M0; Ic, T3 N0 M0; Id, T4 N0 M0; IIa through IId, T1 through T4 N1 M0; IIIa through IIId, T1 through T4 N3 M0; and IVa through IVd, T1 through T4 N0 through N3 M1. MF indicates mycosis fungoides.…”

“…Comparison of published series is often difficult, because of different inclusion criteria (eg, inclusion or exclusion of patients with large-plaque parapsoriasis) and an inconsistent use of the terms MF and CTCL. Several studies, including one of the largest European studies of 92 Dutch patients published by Hamminga et al 4 in 1982, included not only patients with classic MF but also patients with Sézary syndrome and other types of CTCL defined more recently. For instance, patients with CTCL presenting with tumors with the histological appearance of a diffuse, large, T-cell lymphoma and without prior or concurrent patches or plaques were designated previously as having "MF d'emblé e," whereas such patients are now classified as having either CD30 + or CD30 − large-cell CTCL, which are considered distinct disease entities separate from MF.…”

“…There is, therefore, no evidence that the lymph nodes of these patients with stage II MF were actually involved, which leaves the more unfavorable prognosis of this group unexplained. In the literature, the following prognostic variables have been described: stage of disease, [3][4][5][11][12][13][14] age, 2,10,12,13 race, 2 response to initial treatment, 3,10,13 and prior malignant neoplasm. 2 In the present study, stage at diagnosis (ie, the presence of extracutaneous disease and the type of skin involvement), complete remission after initial treatment, and the presence of follicular mucinosis proved independently predictive of disease-specific survival and disease progression.…”