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Early diagnosis of mycosis fungoides: Correlation and utilization of clinical and pathologic findings
P2107
P2109
CLINICAL AND HISTOLOGIC RESPONSE OF EARLY-STAGE CUTANEOUS T-CELL LYMPHOMA TO LOW-DOSE COMBINATION THERAPY WITH BEXAROTENE AND PSORALEN PLUS ULTRAVIOLET A Joan Guitart, MD, Northwestern University Medical School, Chicago, IL, United States; Victor Stevens, PhD, Ligand Pharmaceuticals, San Diego, CA, United States; Philip Dehazya, PhD, Millennix Inc, Purchase, NY, United States A multicenter study evaluated the safety and efficacy of combining reduced doses of both bexarotene capsules and PUVA therapy in patients with histopathologically confirmed stage IB-IIA cutaneous T-cell lymphoma (CTCL). Patients were randomized to receive 150 or 300 mg bexarotene daily and PUVA therapy (3 times/wk) for 24 weeks of combination therapy. Efficacy was based on a physician’s global assessment and percentage of body surface area involvement with CTCL evaluated monthly. Responses were confirmed by a skin biopsy. Thirty-eight patients (male, 22; female, 16; mean age, 55 years) were randomized to receive 300 mg (n = 19) or 150 mg (n = 19) of bexarotene daily with concomitant PUVA therapy for 6 to 24 weeks. Four patients were not evaluable for response. The overall response rate (complete [CCR] + partial [PR]) among the remaining 34 patients was 82% (28/34). Sixteen patients (16/34, 47%) had CCR and 12 (12/34, 35%) had PR. The remaining 6 patients had stable disease (6/34, 18 %). On average, it took 8 weeks and a total of 63 J/cm2 of UV energy to first achieve a CCR/PR (range 426 weeks). Best responses were observed after an average of 12 weeks (range 8-26 weeks) and a total light dose of 104 J/cm2. Patients with stable disease had received an average of 53 J/cm2. Posttreatment biopsies were performed on 14 of the 16 CCR patients and results corroborated with the clinical response in 13 of 14 cases (93%). In the patients with a partial response, repeat biopsies were performed on 10 patients with 7 biopsies (7/10, 70%) negative for mycosis fungoides, despite some lingering erythema and scaling.
EARLY DIAGNOSIS OF MYCOSIS FUNGOIDES: CORRELATION AND UTILIZATION OF CLINICAL AND PATHOLOGIC FINDINGS Peter Heald, MD, John Soderberg, MD, MPH, Earl Glusac, MD, Yale University, New Haven, CT, United States
Adverse events were reported in 36 patients (36/38, 95%). More than half of the patients experienced elevated triglyceride levels (20/36, 56%), mostly NCI grade 1 (25%) or grade 2 (15%). Grade 3 elevations of triglycerides (>5-10 3 ULN) were seen in 6 patients (6/36, 17%) and were controlled by adjusting the doses of the lipidlowering drug and/or bexarotene. Eight patients experienced hypothyroidism (8/36, 22%). Four serious adverse events were reported. Three were unrelated and a single case of pulmonary infiltrate was reported as being related to therapy. These data suggest that safe and rapid clearing of early-stage CTCL can be achieved with concomitant bexarotene and PUVA therapy.
Mycosis fungoides(MF) is a malignancy for which prognosis at diagnosis is assessed by a stage I-IV system. To diagnose MF at stage IA, there must be objective and reproducible criteria that can be used to assess the strength of diagnosis. To develop these, we retrospectively reviewed the charts of all patients newly diagnosed with MF-type cutaneous T-cell lymphoma (CTCL) that had been diagnosed at our institution for the past 10 years. From the total number of patients reviewed, 155 had a diagnosis of MF-type CTCL, but only 42 patients with stage IA were studied. At the time of examination, each patient had his/her examination performed by the same physician. Lesion morphology and distribution were assessed as classic, consistent, or atypical (modified from Cooper). In addition, all evaluations provided information on demographics and duration of symptoms and signs. The skin biopsies of all 42 patients were reviewed for the following: intraepidermal lymphocytes larger than those within the dermis, Pautrier’s microabscesses, disproportionate exocytosis (exocytosis of lymphoctes into the epidermis in the relative absence of spongiosis), basilar lymphocytes, haloed lymphocytes, convoluted lymphocytes, papillary dermal fibrosis, and the presence of a band-like lymphocytic infiltrate. After all of the biopsy slides had been graded, they were then assessed for the overall degree of likelihood of MF-type CTCL. The categories and their score for statistical studies were as follows: diagnostic, consistent, compatible with, or suggestive of MF. We then examined whether a statistical comparison could be made in the correlation of clinical and histological traits. By using Wilcoxon scores (ranked sums), the clinical variables of morphology and distribution were compared individually to median histology scores to examine whether any real difference existed. Tests of independence for morphology or distribution versus histological scores were also done using this test; however, there was not a statistically significant difference in histology scores between the different patterns of clinical presentation. Additionally, there was no correlation with age, sex, or duration of lesion. The results emphasize the importance of incorporating clinical findings into the diagnosis of stage IA MF-type CTCL. We propose that the diagnosis of MF can be made when any one of the morphology, distribution, or pathology findings are assessed as classic and the others are regarded as consistent. Nothing to disclose.
75% sponsored by Ligand Pharmaceuticals
P2110 EPSTEIN-BARR VIRUS–ASSOCIATED PERIPHERAL T-CELL LYMPHOMA SHOWING FOLLICULOTROPISM Hye-Jin Choi, MD, Seung-Seog Han, MD, Kyoung-Jin Kim, MD, PhD, Sung-Eun Chang, MD, PhD, Department of Dermatology, Asan Medical Center, University of Ulsan, Seoul, Korea
Mycosis fungoides is a chronic progressive cutaneous lymphoma that initially stimulates eczema or other inflammatory dermatoses, such as patchy or plaque-like skin disease. Clobetasol propionate lotion 0.05% is a class I (superpotent) corticosteroid formulation indicated for the relief of the inflammatory and pruritic manifestations of corticosteroid-responsive dermatoses in patients 18 years or older. Clobetasol propionate lotion 0.05% may provide the patient with improved clinical outcomes because of its longer duration of response and higher drug recovery in the epidermis compared with the cream and emollient cream formulations. This poster will present efficacy and tolerability results of clobetasol propionate lotion 0.05% in the treatment of mycosis fungoides.
Epstein-Barr virus (EBV)–associated peripheral T-cell lymphoma has heterogeneous clinicohistologic features, but it is believed to have the primary features of prolonged fever, weight loss, hepatosplenomegaly, lymphadenopathy, multiorgan involvement, anemia, and high serum alkaline phosphatase and lactate dehydrogenase. The clinical behavior seems to be aggressive with a median survival of only a few months despite intensive chemotherapy. We present a case of EBV-associated aggressive peripheral T-cell lymphoma with remarkable follicular involvement in a 35-year-old man. He had multiple alopecia on the scalp, several erythematous patches with some follicular hyperkeratotic spikes on the trunk, and crusted papules on the penis of 5 months’ duration. He had been hospitalized for the evaluation of chronic diarrhea, fatigue, weight loss (17 kg in 6months), and intermittent fever of 7 months’ duration. Skin biopsies from the various types of the lesion showed similar features: a moderate periadnexal infiltrate composed of atypical lymphocytes sparing epidermis in the absence of follicular mucinosis. Immunohistochemically, most of the lymphoid cells stained for CD3 and CD4. In addition, simultaneous localization of CD8 and TIA-1 was found with positivity for EBV-encoded RNA (EBER). Molecular genetic analysis confirmed the monoclonal nature of the T-cell infiltrate. Further evaluation revealed that the patient had concurrent gastrointestinal involvement of lymphoma, which was also positive for EBER. The skin lesion progressed despite systemic chemotherapy, and the patient died of septic shock. We thought the cytotoxic phenotype with EBV association in this patient may in part explain the aggressive clinical behavior of this patient. In conclusion, we should emphasize a prognostic factor of lymphoma classification, which would help us to choose optimal treatment modality.
100% supported by Galderma
Nothing to disclose.
P2108 CLOBETASOL PROPIONATE 0.05% IN PATIENTS WITH MYCOSIS FUNGOIDES Joseph Bikowski, MD, Bikowski Skin Care Center, Sewickley, PA, United States
MARCH 2005
J AM ACAD DERMATOL
P139
P2109
CLINICAL AND HISTOLOGIC RESPONSE OF EARLY-STAGE CUTANEOUS T-CELL LYMPHOMA TO LOW-DOSE COMBINATION THERAPY WITH BEXAROTENE AND PSORALEN PLUS ULTRAVIOLET A Joan Guitart, MD, Northwestern University Medical School, Chicago, IL, United States; Victor Stevens, PhD, Ligand Pharmaceuticals, San Diego, CA, United States; Philip Dehazya, PhD, Millennix Inc, Purchase, NY, United States A multicenter study evaluated the safety and efficacy of combining reduced doses of both bexarotene capsules and PUVA therapy in patients with histopathologically confirmed stage IB-IIA cutaneous T-cell lymphoma (CTCL). Patients were randomized to receive 150 or 300 mg bexarotene daily and PUVA therapy (3 times/wk) for 24 weeks of combination therapy. Efficacy was based on a physician’s global assessment and percentage of body surface area involvement with CTCL evaluated monthly. Responses were confirmed by a skin biopsy. Thirty-eight patients (male, 22; female, 16; mean age, 55 years) were randomized to receive 300 mg (n = 19) or 150 mg (n = 19) of bexarotene daily with concomitant PUVA therapy for 6 to 24 weeks. Four patients were not evaluable for response. The overall response rate (complete [CCR] + partial [PR]) among the remaining 34 patients was 82% (28/34). Sixteen patients (16/34, 47%) had CCR and 12 (12/34, 35%) had PR. The remaining 6 patients had stable disease (6/34, 18 %). On average, it took 8 weeks and a total of 63 J/cm2 of UV energy to first achieve a CCR/PR (range 426 weeks). Best responses were observed after an average of 12 weeks (range 8-26 weeks) and a total light dose of 104 J/cm2. Patients with stable disease had received an average of 53 J/cm2. Posttreatment biopsies were performed on 14 of the 16 CCR patients and results corroborated with the clinical response in 13 of 14 cases (93%). In the patients with a partial response, repeat biopsies were performed on 10 patients with 7 biopsies (7/10, 70%) negative for mycosis fungoides, despite some lingering erythema and scaling.
EARLY DIAGNOSIS OF MYCOSIS FUNGOIDES: CORRELATION AND UTILIZATION OF CLINICAL AND PATHOLOGIC FINDINGS Peter Heald, MD, John Soderberg, MD, MPH, Earl Glusac, MD, Yale University, New Haven, CT, United States
Adverse events were reported in 36 patients (36/38, 95%). More than half of the patients experienced elevated triglyceride levels (20/36, 56%), mostly NCI grade 1 (25%) or grade 2 (15%). Grade 3 elevations of triglycerides (>5-10 3 ULN) were seen in 6 patients (6/36, 17%) and were controlled by adjusting the doses of the lipidlowering drug and/or bexarotene. Eight patients experienced hypothyroidism (8/36, 22%). Four serious adverse events were reported. Three were unrelated and a single case of pulmonary infiltrate was reported as being related to therapy. These data suggest that safe and rapid clearing of early-stage CTCL can be achieved with concomitant bexarotene and PUVA therapy.
Mycosis fungoides(MF) is a malignancy for which prognosis at diagnosis is assessed by a stage I-IV system. To diagnose MF at stage IA, there must be objective and reproducible criteria that can be used to assess the strength of diagnosis. To develop these, we retrospectively reviewed the charts of all patients newly diagnosed with MF-type cutaneous T-cell lymphoma (CTCL) that had been diagnosed at our institution for the past 10 years. From the total number of patients reviewed, 155 had a diagnosis of MF-type CTCL, but only 42 patients with stage IA were studied. At the time of examination, each patient had his/her examination performed by the same physician. Lesion morphology and distribution were assessed as classic, consistent, or atypical (modified from Cooper). In addition, all evaluations provided information on demographics and duration of symptoms and signs. The skin biopsies of all 42 patients were reviewed for the following: intraepidermal lymphocytes larger than those within the dermis, Pautrier’s microabscesses, disproportionate exocytosis (exocytosis of lymphoctes into the epidermis in the relative absence of spongiosis), basilar lymphocytes, haloed lymphocytes, convoluted lymphocytes, papillary dermal fibrosis, and the presence of a band-like lymphocytic infiltrate. After all of the biopsy slides had been graded, they were then assessed for the overall degree of likelihood of MF-type CTCL. The categories and their score for statistical studies were as follows: diagnostic, consistent, compatible with, or suggestive of MF. We then examined whether a statistical comparison could be made in the correlation of clinical and histological traits. By using Wilcoxon scores (ranked sums), the clinical variables of morphology and distribution were compared individually to median histology scores to examine whether any real difference existed. Tests of independence for morphology or distribution versus histological scores were also done using this test; however, there was not a statistically significant difference in histology scores between the different patterns of clinical presentation. Additionally, there was no correlation with age, sex, or duration of lesion. The results emphasize the importance of incorporating clinical findings into the diagnosis of stage IA MF-type CTCL. We propose that the diagnosis of MF can be made when any one of the morphology, distribution, or pathology findings are assessed as classic and the others are regarded as consistent. Nothing to disclose.
75% sponsored by Ligand Pharmaceuticals
P2110 EPSTEIN-BARR VIRUS–ASSOCIATED PERIPHERAL T-CELL LYMPHOMA SHOWING FOLLICULOTROPISM Hye-Jin Choi, MD, Seung-Seog Han, MD, Kyoung-Jin Kim, MD, PhD, Sung-Eun Chang, MD, PhD, Department of Dermatology, Asan Medical Center, University of Ulsan, Seoul, Korea
Mycosis fungoides is a chronic progressive cutaneous lymphoma that initially stimulates eczema or other inflammatory dermatoses, such as patchy or plaque-like skin disease. Clobetasol propionate lotion 0.05% is a class I (superpotent) corticosteroid formulation indicated for the relief of the inflammatory and pruritic manifestations of corticosteroid-responsive dermatoses in patients 18 years or older. Clobetasol propionate lotion 0.05% may provide the patient with improved clinical outcomes because of its longer duration of response and higher drug recovery in the epidermis compared with the cream and emollient cream formulations. This poster will present efficacy and tolerability results of clobetasol propionate lotion 0.05% in the treatment of mycosis fungoides.
Epstein-Barr virus (EBV)–associated peripheral T-cell lymphoma has heterogeneous clinicohistologic features, but it is believed to have the primary features of prolonged fever, weight loss, hepatosplenomegaly, lymphadenopathy, multiorgan involvement, anemia, and high serum alkaline phosphatase and lactate dehydrogenase. The clinical behavior seems to be aggressive with a median survival of only a few months despite intensive chemotherapy. We present a case of EBV-associated aggressive peripheral T-cell lymphoma with remarkable follicular involvement in a 35-year-old man. He had multiple alopecia on the scalp, several erythematous patches with some follicular hyperkeratotic spikes on the trunk, and crusted papules on the penis of 5 months’ duration. He had been hospitalized for the evaluation of chronic diarrhea, fatigue, weight loss (17 kg in 6months), and intermittent fever of 7 months’ duration. Skin biopsies from the various types of the lesion showed similar features: a moderate periadnexal infiltrate composed of atypical lymphocytes sparing epidermis in the absence of follicular mucinosis. Immunohistochemically, most of the lymphoid cells stained for CD3 and CD4. In addition, simultaneous localization of CD8 and TIA-1 was found with positivity for EBV-encoded RNA (EBER). Molecular genetic analysis confirmed the monoclonal nature of the T-cell infiltrate. Further evaluation revealed that the patient had concurrent gastrointestinal involvement of lymphoma, which was also positive for EBER. The skin lesion progressed despite systemic chemotherapy, and the patient died of septic shock. We thought the cytotoxic phenotype with EBV association in this patient may in part explain the aggressive clinical behavior of this patient. In conclusion, we should emphasize a prognostic factor of lymphoma classification, which would help us to choose optimal treatment modality.
100% supported by Galderma
Nothing to disclose.
P2108 CLOBETASOL PROPIONATE 0.05% IN PATIENTS WITH MYCOSIS FUNGOIDES Joseph Bikowski, MD, Bikowski Skin Care Center, Sewickley, PA, United States
MARCH 2005
J AM ACAD DERMATOL
P139