Poor prognosis in non-Caucasian patients with early-onset mycosis fungoides.

BACKGROUND: Mycosis fungoides (MF), the most common cutaneous T-cell lymphoma, is most often seen in middle-aged to elderly men. OBJECTIVE: We conducted a retrospective study of the demographics and prognosis of patients with onset of MF before age 40 years. METHODS: Demographic data (age, sex, and race) and histology from 1074 patents with cutaneous T-cell lymphoma were stratified by age of onset and race and analyzed using Chi-square test. RESULTS: Women presented before age 40 years more often than men (P = .038). Early onset of MF was diagnosed in 30 of 92 (32.6%) African American (AA), 31 of 87 (35.6%) Hispanic, and 103 of 809 (12.7%) Caucasian patients. MF was significantly more common in AA (P = .0008) and Hispanic (P = .0002) patients. Early-onset MF was more common among 21 of 60 AA women (35%, P = .0174) and 19 of 40 Hispanic women (47.5%, P = .0002) than among 50 of 350 Caucasian women (14.5%). Progression from initial TNM stage occurred in only 5 (10%) Caucasian, one (5%) Hispanic, and 8 (38%) AA women who presented before age 40 years. Six of 8 AA women who progressed died of their disease whereas two were long-term survivors after allogeneic transplantation. LIMITATIONS: This was a retrospective study at one cancer center. CONCLUSION: Although MF is considered to be a disease of middle-aged men, early-onset MF is more common among AA and Hispanic women. AA women with early onset may have a poor prognosis and should be considered for more aggressive therapy, including allogeneic transplantation.

Skin directed therapy for mycosis fungoides: a review.

Mycosis fungoides (MF) is a rare neoplasm of epidermotropic CD4+ lymphocytes and represents a majority of all cutaneous T cell lymphomas. Early stage MF is limited to cutaneous patches and plaques that can be treated with topical modalities with high response rates. More aggressive systemic treatment of early disease does not alter survival or cure the disease and could accelerate progression by causing immunosuppression. Topical corticosteroids, mechlorethamine, and carmustine have been the mainstays of early treatment of MF for more than 30 years. More recently, topical formulations of retinoids, novel rexinoids, methotrexate, immunomodulators, and photodynamic compounds have been investigated for their potential roles in treating early ME The future of topical treatments for MF is promising both as primary and adjunctive therapy.

CD4+ CD56+ hematodermic/plasmacytoid dendritic cell tumor with response to pralatrexate.

The CD4(+) CD56(+) hematodermic/plasmacytoid dendritic cell tumor is a rare, highly aggressive, systemic neoplasm for which effective therapies have not yet been established. These tumors express CD4, CD56, CD123, and T-cell leukemia/lymphoma (TCL)-1 and are clinically characterized by cutaneous involvement with spread to bone marrow and blood, and poor prognosis with current chemotherapy regimens. We describe a Caucasian woman who presented with plasmacytoid dendritic cell tumor, but an absence of systemic symptoms. Clinically, multiple cutaneous lesions were brown to violaceous firm nodules on the face, arms, and trunk. The patient underwent two courses of cyclophosphamide, Adriamycin, vincristine, and prednisone chemotherapy but relapsed quickly. The investigational agent, pralatrexate (30 mg/m(2)) was given weekly with vitamin B12 and folic acid and resulted in remarkable clinical response with regression of skin tumors. Our observation highlights pralatrexate as a promising therapeutic option for hematodermic/plasmacytoid dendritic cell lymphoma/leukemias.

Treatment of a patient with epidermodysplasia verruciformis carrying a novel EVER2 mutation with imiquimod.

Epidermodysplasia verruciformis (EV) is a rare disorder characterized by widespread human papillomavirus infection and malignant transformation. EV may be caused by mutations of the genes EVER1 or EVER2, which are located on the EV1 locus, 17q25. We describe a patient with EV and a novel homozygous gene mutation of EVER2 gene who was treated successfully with topical imiquimod.

Sustained efficacy and safety of infliximab in psoriasis: a retrospective study of 73 patients.

BACKGROUND: Infliximab inhibits T-cell activation by binding tumor necrosis factor-alpha (TNF-alpha). This medication is widely used in the US for treatment of psoriasis as an off-label indication. The durability of its effect is largely unknown. OBJECTIVE: To assess the proportion of patients still on infliximab 12 months after initiation of therapy for psoriasis. METHODS: Retrospective chart review analysis of 73 patients with psoriasis treated with infliximab for at least 12 months or those who experienced treatment failure in less than 12 months. The point where infusions were deemed to no longer be efficacious was determined by physician's global assessment (PGA). RESULTS: Of 73 patients who started infliximab at least 12 months prior to this chart review, 22 (30.1%) had discontinued treatment secondary to loss of efficacy. Thirty seven patients (50.7%) had no loss of efficacy and continued to receive infusions. Two patients (2.7%) discontinued due to loss of efficacy after greater than 12 months. Of the 22 patients who discontinued treatment due to loss of efficacy, 2 were on concomitant methotrexate (5-7.5 mg/wk) therapy. Of the 37 patients still receiving treatment with no loss of efficacy at 12 months, 3 patients were on concomitant methotrexate therapy. Five patients (6.8%) discontinued secondary to minor adverse events: sinus infection (1), acne (1), fever (1), arthralgia (1), and transient rash (1). Three patients (4.1%) discontinued due to major adverse events: reactivation of tuberculosis (1), breast cancer (1), and gastrointestinal bleeding (1). One patient discontinued infliximab secondary to concerns of possible lymphoma risk (though there were no signs of symptoms of disease on examination), and 3 patients discontinued due to insurance concerns. CONCLUSION: Infliximab treatment resulted in significant improvement in psoriasis, with 37 out of 73 patients (50.7%) experiencing no loss of efficacy. This longitudinal retrospective chart review demonstrates continued benefit of infliximab infusions in about half of patients after 1 year, though a notable percentage (30.1%) experienced loss of efficacy as determined by physician's global assessment (PGA) and a number of others discontinued due to adverse events or insurance difficulty.

Atypical CD8+ cutaneous T-cell lymphoma after immunomodulatory therapy.

Systemic immunomodulatory agents have recently been approved for the treatment of rheumatoid arthritis and psoriasis. Although lymphomas are known to emerge in the setting of immunosuppressive therapy, it has not been well described or established for the newer biologic immune response modifiers. Herein, we describe 2 patients who developed unusual CD8+ cutaneous lymphoproliferative disorders after treatment with efalizumab and infliximab. The mechanisms and occurrence of lymphoma after immune response modifiers are discussed.

Distal lower extremity paresthesia and foot drop developing during adalimumab therapy.

The development of tumor necrosis factor inhibitor biologic therapy is arguably one of the most significant achievements in the treatment of rheumatic diseases to date. Neurologic events suggestive of demyelination have been reported for patients receiving treatment with the antitumor necrosis factor agents etanercept and infliximab. We describe the onset of lower extremity paresthesia and foot drop in a patient who was receiving adalimumab for the treatment of psoriasis and examine the relationship of tumor necrosis factor antagonism and demyelinating disease.