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Mycosis fungoides and Sezary syndrome: therapeutic approach and outcome in 113 patients.
Int J Dermatol. 2005 Jul; 44(7):559-65.IJ

Abstract

BACKGROUND

Mycosis fungoides (MF) and Sezary syndrome (SS) are the most common forms of cutaneous T-cell lymphoma (CTCL). Various topical and systemic therapeutic alternatives are available, but there is no standard or definite curative treatment regimen. When making a decision about the appropriate treatment modality, the age and compliance of the patient, stage of the disease, treatment accessibility, and previous treatment history should be considered.

AIM

To determine the therapeutic response of patients with MF and SS to different treatment modalities. Patients were evaluated with respect to their clinical and demographic features.

METHODS

One hundred and thirteen patients diagnosed clinically and dermatopathologically with MF and SS between March 1984 and June 2001 were included in the study.

RESULTS

Of the 113 patients studied, 110 had a diagnosis of MF and three had a diagnosis of SS; 101 patients (89.4%) were diagnosed with early stage (IA, IB, IIA) and 12 (10.6%) with late stage (IIB, III, IVA, IVB) disease. The age at diagnosis varied between 12 and 81 years (mean, 45.6+/-15.8 years). Fifty-five (48.7%) patients were male and 58 (51.3%) were female. The duration of the skin lesions varied between 1.5 months and 32 years (mean, 6.1 years). Psoralen plus UVA (PUVA) was the most commonly used initial treatment modality in early stage disease (91%), with a complete remission (CR) rate of 80.4%. With PUVA+interferon-alpha (INF-alpha) treatment, CR was 57% in the early stages and 33.3% in the late stages. For late stage disease, systemic therapies, such as pentostatin, gemcitabine, and fludarabine, alone or in combination with INF-alpha, were preferred. Of the 113 patients, eight (7% of the total and 57.1% of the advanced stage cases) died of MF; 21.4% of the late stage patients showed partial remission and 14.2% showed CR. None of the patients diagnosed with early stage disease died of MF, but two (1.9%) progressed to late stage disease.

CONCLUSIONS

PUVA and PUVA+INF-alpha are effective treatment modalities, especially for early stage MF. Once the disease has progressed, both MF and SS are very resistant to treatment regimens, including chemotherapeutic agents. It is important to diagnose and treat these diseases, especially MF, in the early stages for lasting remission.

Authors+Show Affiliations

Department of Dermatology, Ankara University Faculty of Medicine, Ankara, Turkey. ranaanadolu@hotmail.comNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

15985024

Citation

Anadolu, Rana Yavuzer, et al. "Mycosis Fungoides and Sezary Syndrome: Therapeutic Approach and Outcome in 113 Patients." International Journal of Dermatology, vol. 44, no. 7, 2005, pp. 559-65.
Anadolu RY, Birol A, Sanli H, et al. Mycosis fungoides and Sezary syndrome: therapeutic approach and outcome in 113 patients. Int J Dermatol. 2005;44(7):559-65.
Anadolu, R. Y., Birol, A., Sanli, H., Erdem, C., & Türsen, U. (2005). Mycosis fungoides and Sezary syndrome: therapeutic approach and outcome in 113 patients. International Journal of Dermatology, 44(7), 559-65.
Anadolu RY, et al. Mycosis Fungoides and Sezary Syndrome: Therapeutic Approach and Outcome in 113 Patients. Int J Dermatol. 2005;44(7):559-65. PubMed PMID: 15985024.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Mycosis fungoides and Sezary syndrome: therapeutic approach and outcome in 113 patients. AU - Anadolu,Rana Yavuzer, AU - Birol,Ahu, AU - Sanli,Hatice, AU - Erdem,Cengizhan, AU - Türsen,Umit, PY - 2005/6/30/pubmed PY - 2005/11/3/medline PY - 2005/6/30/entrez SP - 559 EP - 65 JF - International journal of dermatology JO - Int J Dermatol VL - 44 IS - 7 N2 - BACKGROUND: Mycosis fungoides (MF) and Sezary syndrome (SS) are the most common forms of cutaneous T-cell lymphoma (CTCL). Various topical and systemic therapeutic alternatives are available, but there is no standard or definite curative treatment regimen. When making a decision about the appropriate treatment modality, the age and compliance of the patient, stage of the disease, treatment accessibility, and previous treatment history should be considered. AIM: To determine the therapeutic response of patients with MF and SS to different treatment modalities. Patients were evaluated with respect to their clinical and demographic features. METHODS: One hundred and thirteen patients diagnosed clinically and dermatopathologically with MF and SS between March 1984 and June 2001 were included in the study. RESULTS: Of the 113 patients studied, 110 had a diagnosis of MF and three had a diagnosis of SS; 101 patients (89.4%) were diagnosed with early stage (IA, IB, IIA) and 12 (10.6%) with late stage (IIB, III, IVA, IVB) disease. The age at diagnosis varied between 12 and 81 years (mean, 45.6+/-15.8 years). Fifty-five (48.7%) patients were male and 58 (51.3%) were female. The duration of the skin lesions varied between 1.5 months and 32 years (mean, 6.1 years). Psoralen plus UVA (PUVA) was the most commonly used initial treatment modality in early stage disease (91%), with a complete remission (CR) rate of 80.4%. With PUVA+interferon-alpha (INF-alpha) treatment, CR was 57% in the early stages and 33.3% in the late stages. For late stage disease, systemic therapies, such as pentostatin, gemcitabine, and fludarabine, alone or in combination with INF-alpha, were preferred. Of the 113 patients, eight (7% of the total and 57.1% of the advanced stage cases) died of MF; 21.4% of the late stage patients showed partial remission and 14.2% showed CR. None of the patients diagnosed with early stage disease died of MF, but two (1.9%) progressed to late stage disease. CONCLUSIONS: PUVA and PUVA+INF-alpha are effective treatment modalities, especially for early stage MF. Once the disease has progressed, both MF and SS are very resistant to treatment regimens, including chemotherapeutic agents. It is important to diagnose and treat these diseases, especially MF, in the early stages for lasting remission. SN - 0011-9059 UR - https://www.unboundmedicine.com/medline/citation/15985024/Mycosis_fungoides_and_Sezary_syndrome:_therapeutic_approach_and_outcome_in_113_patients_ L2 - https://doi.org/10.1111/j.1365-4632.2004.02033.x DB - PRIME DP - Unbound Medicine ER -