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.

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Clinical and laboratory investigations of 8 patients with Sézary's syndrome are presented. Sézary cells were found in peripheral blood as well as in bone marrow and lymph nodes. The overall follow-up period was 8 years. Different therapeutic regimens were given. The best results were obtained with chlorambucil and prednisone orally, combined with topical application of nitrogen mustard. Three patients died of pneumonia and septicaemia.

Red cells of 3 patients with the painful bruising syndrome showed morphological abnormalities. In the fraction not sedimenting in Ficoll/Isopaque gradient centrifugation, some of the cells had club-shaped processes, mitochondria, nuclear remnants, and vacuoles. In freeze-etch preparations, 90% of the red cells showed membrane elevations at pH 7.4 and 25% at pH 6.4, while in freeze-etch preparations of controls these values were 55 and 0 respectively. In addition, rouleaux formation was markedly enhanced in the preparations of blood of patients with the painful bruising syndrome.

Extracutaneous involvement in mycosis fungoides (MF) is associated with a poor prognosis. Therefore, a pretreatment staging procedure is used to determine the extent of the disease. As part of the staging procedure lymphography was performed in 36 patients with MF. The radiographic findings and the correlation of these findings with the histopathologic changes in lymph nodes and the clinical course are described. Lymphography in MF shows a variety of abnormalities. However, these abnormalities correlate in only 61% of the patients with the extent and the clinical course of the disease. It is concluded that lymphography is not a useful clinical tool in the staging of MF.