A modified staging classification for cutaneous T-cell lymphoma.

BACKGROUND: Despite refinements in the diagnosis of cutaneous T-cell lymphoma (CTCL), since 1979 there have been no changes to the staging of CTCL used to classify mycosis fungoides and Sézary syndrome. OBJECTIVE: We reviewed the current staging of CTCL and examined the usefulness of a new staging scheme for mycosis fungoides and Sézary syndrome. METHODS: We determined overall survival of 450 patients with mycosis fungoides and Sézary syndrome using the current and modified staging classifications. RESULTS: There were no significant differences between survival of patients with stage IB (patches/plaques involving greater than 10% body surface area) and IIA (peripheral adenopathy) disease and of patients with stage IIB (tumor) and III (erythroderma) disease. There was a significant difference in survival between patients with extensive patch versus extensive plaque stage disease. Modification of the current classification by splitting T2 into patch versus plaque stage disease and incorporating tumors and erythroderma into stage III proved superior to the current scheme in predicting overall survival. CONCLUSION: Modification of the current staging classification for CTCL yields subgroups useful in the prognostic assessment of CTCL.

Relative frequency of various forms of primary cutaneous lymphomas.

BACKGROUND: Data on the relative frequency of the various forms of primary cutaneous lymphomas (PCLs) are largely limited to European institutions. OBJECTIVE: Our purpose was to document the relative frequencies of various PCLs seen at 3 US institutions with active cutaneous lymphoma programs and to compare those with the European data. METHODS: Included in this study are newly registered patients seen at MCP Hahnemann University, New York University, and the University of California, San Francisco from July 1, 1995 to June 30, 1998. RESULTS: A total of 755 patients were seen. The frequency distribution of the major diagnostic groups was as follows: mycosis fungoides/Sézary syndrome, 82.3%; lymphomatoid papulosis, 12.6% (including patients with associated mycosis fungoides/Sézary syndrome); CD30(+) anaplastic large-cell lymphoma, 0.9%; peripheral T-cell lymphomas, 2.9%; B-cell lymphoma, 4.5%. CONCLUSION: The most striking finding is the much lower relative frequency of primary cutaneous B-cell lymphomas at US institutions (4.5%) versus the approximately 20% reported by European groups. The reason for this difference requires further study.

Cutaneous T cell lymphoma: update of treatment.

The state-of-the art therapy of cutaneous T cell lymphoma (CTCL) is reviewed. Commonly used treatments for early-stage (patch/plaque) mycosis fungoides (MF) include topical corticosteroids, mechlorethamine, carmustine, ultraviolet light B and PUVA. Total skin electron beam (TSEB) therapy is indicated for widespread infiltrated plaque and tumor stage disease. Low-dose methotrexate is often useful for resistant patch/plaque MF and erythrodermic CTCL. Interferon alpha (IFN-alpha) is indicated for methotrexate failures and recurrent tumors following TSEB therapy. Photopheresis may be helpful for early-stage erythrodermic CTCL but is very costly. Retinoids may be of value for early and moderately advanced CTCL particularly in combination with other agents such as IFN-alpha and PUVA. Systemic disease usually requires combination chemotherapy such as that used for non-Hodgkin's lymphoma; however, responses are usually short lived.

Mycosis fungoides masquerading as an ischemic foot.

This case is of a man with bilateral lower-extremity ischemia and a solitary nonhealing ulcerated lesion of the right great toe. After revascularization with an aortobifemoral bypass, his right ABI increased from 0.5 to approximately 0.75, but the ulcerated toe lesion did not show signs of healing and instead progressed to a deeper ulceration exposing bone. Because of presumptive osteomyelitis, we performed a great toe amputation, and immunohistochemical analysis of the lesion revealed late plaque stage mycosis fungoides (MF). We present this case to alert the vascular surgeon to this diagnostic possibility when confronted with an apparent ischemic lesion and to describe what made this particular lesion suspicious for MF. To the best of our knowledge, this is the first case of MF presenting solely as an ischemic lesion.

Prognosis in cutaneous T-cell lymphoma by skin stage: long-term survival in 489 patients.

BACKGROUND: Although a number of studies have documented the long-term survival of patients with cutaneous T-cell lymphoma (CTCL), none have provided data as to the relative survival of all 4 skin stages. OBJECTIVE: We document survival of CTCL patients by T stage relative to that of an age-, sex-, and race-matched population. METHODS: The survival of 489 patients with CTCL registered since 1957 was compared with that of a California control population. RESULTS: For stage T1 (< 10% skin involved) there was no significant difference between the observed and expected survivals. For the other 3 stages the observed survival was significantly inferior to that of the expected survival (P = .002). At 10 years the relative survivals were: T2 (10% or more skin involved) 67.4%, T3 (tumor stage) 39.2%, T4 (generalized erythroderma) 41.0%. T2 plaque stage patients had an inferior relative survival (P = .001), whereas T2 patch stage patients did not. Lymphadenopathy had an unfavorable impact on prognosis. There was a strong trend toward diagnosing CTCL at an earlier stage in more recent years. We estimate that from 15% to 20% of our patients died of CTCL or related complications. CONCLUSION: The relative survival of CTCL patients worsens with increasing skin stage, although stages T3 and T4 had closely similar survivals. The great majority of patients with CTCL do not die of their disease.

Topical corticosteroids for mycosis fungoides. Experience in 79 patients.

OBJECTIVE: To determine the effectiveness of topical corticosteroids in the management of mycosis fungoides. DESIGN: Prospective study. SETTING: Academic referral center, Veterans Affairs Medical Center, and private practice. PATIENTS: Seventy-nine patients with patch or plaque stage of mycosis fungoides. Fifty-one were stage T1 (less than 10% of skin involved) and 28 were stage T2 (10% or more of skin involved). Seventy-five had patch-stage and 4 had plaque-stage disease as determined by histological examination. INTERVENTION: Patients were treated with topical class I to III corticosteroids. Of the stage T1 patients, all used class I corticosteroids, and 4 (8%) also used class II or III corticosteroids. Of the stage T2 patients, 19 (68%) used class I and 12 (43%) used class II or III compounds. Some patients used more than 1 class of corticosteroid. Applications were almost always twice daily. Three stage T1 and 2 stage T2 patients used plastic film occlusion. Baseline and monthly morning serum cortisol levels were obtained during treatment. MAIN OUTCOME MEASURES: Response to treatment and side effects. RESULTS: The median follow-up period was 9 months. Thirty-two (63%) of stage T1 patients achieved complete remission and 16 (31%) achieved partial remission, for a total response rate of 48 (94%). The comparable figures for stage T2 patients were 7 (25%), 16 (57%), and 23 (82%), respectively. Responses were determined by clinical examination. Thirty-nine patients achieved clinical clearing. In 7 of these, posttreatment biopsy specimens were obtained, and all showed histological clearing. Reversible depression of serum cortisol levels occurred in 10 (13%). Minor skin irritation occurred in 2 patients and localized, reversible skin atrophy in 1. CONCLUSION: Topical corticosteroids, especially class I compounds, are an effective treatment for patch-stage mycosis fungoides.

Mycosis fungoides with onset before 20 years of age.

BACKGROUND: Recent identification of mycosis fungoides (MF) in a man in whom the diagnosis was established at age 22 months prompted us to evaluate our experience with early onset MF. OBJECTIVE: Our purpose was to summarize the clinical characteristics and course of 24 patients in whom MF began by history before age 20 years and was confirmed by biopsy in 13 by that time. METHODS: A retrospective study was conducted. RESULTS: All 24 patients had patch/plaque disease and represented 4.3% of the 557 patients with cutaneous T-cell lymphoma seen by us since 1971. None progressed to a more advanced stage in up to 24 years (median, 12 years) after histologic diagnosis. Five patients (21%) presented with hypopigmentation. CONCLUSION: Early onset MF is not more aggressive than that appearing in adult life. MF should be considered in the differential diagnosis of chronic dermatoses in young persons, particularly in those presenting with hypopigmentation.

Low-dose methotrexate to treat erythrodermic cutaneous T-cell lymphoma: results in twenty-nine patients.

BACKGROUND: Most patients with erythrodermic cutaneous T-cell lymphoma (CTCL) have intense, generalized, refractory pruritus. In some large series the median survival was approximately 3 years. OBJECTIVE: Our purpose was to review our experience with methotrexate in the treatment of 29 patients with erythrodermic CTCL observed for up to 129 months. METHODS: This is a retrospective study. Data are presented in terms of response rates, freedom from treatment failure, and overall survival. RESULTS: Twelve patients (41%) achieved complete remission, and five (17%) achieved partial remission for a total response rate of 58%. The median freedom from treatment failure was 31 months, and the median survival was 8.4 years. Side effects caused treatment failure in only two patients. CONCLUSION: Low-dose methotrexate is a valuable first-line treatment for the majority of patients with early to intermediate-stage erythordermic CTCL.

Assessing clinical outcomes in cutaneous T-cell lymphoma.

It is apparent that multiple end points are required to adequately describe the response to therapy for CTCL. Investigators may differ as to which end points are best suited for their data. In general, multiple end points provide a better overall view of response to treatment as compared with a single end point. Regardless, measures of response and end points should be clearly defined and the methods of calculating end points clearly stated.

Topical treatment of early cutaneous T-cell lymphoma.

The two major topical treatment modalities for cutaneous T-cell lymphoma (CTCL) are mechlorethamine (nitrogen mustard) and topical carmustine (BCNU). Topical mechorethamine hydrochloride has been used since the late 1950s as therapy for CTCL. Topical BCNU solution is a highly effective treatment for early stage mycosis fungoides. This article describes these two modalities.

Topical carmustine (BCNU) for patch/plaque mycosis fungoides.

This article summarizes experience with topical carmustine (BCNU) solution in 172 patients with patch/plaque stage mycosis fungoides. At 36 months 92% of stage-T1 (less than 10% skin involvement) and 64% of stage-T2 (10% or more skin involvement) patients were either in complete or partial remission. The most frequent side effect was an erythematous reaction which was followed by a benign-type telangiectasia in some patients. No secondary skin cancers were seen. Bone marrow depression was uncommon. Topical BCNU is an effective treatment for patch/plaque stage mycosis fungoides.