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1.
J Eur Acad Dermatol Venereol ; 34(4): 762-768, 2020 Apr.
Article in English | MEDLINE | ID: mdl-31591786

ABSTRACT

BACKGROUND: Reliable prognostic factors for patients with primary cutaneous anaplastic large cell lymphoma (PCALCL) are lacking. OBJECTIVE: To identify prognostic factors for specific survival in patients with PCALCL. METHODS: Using the convenience sampling method, patients with PCALCL diagnosed from May 1986 to August 2017 in 16 University Departments were retrospectively reviewed. RESULTS: One hundred eight patients were included (57 males). Median age at diagnosis was 58 years. All of them showed T1-3N0M0 stages. Seventy per cent of the cases presented with a solitary lesion, mostly at the limbs. Complete response rate after first-line treatment was 87%, and no advantage was observed for any of them (surgery, radiotherapy, chemotherapy or other approaches). Nodal and visceral progression rate was 11% and 2%, respectively. 5-year specific survival (SSV) reached 93%; 97% for T1 patients and 84% for T2/T3 patients (P = 0.031). Five-year SSV for patients developing early cutaneous relapse was 64%; for those with late or no relapse, 96% (P = 0.001). Estimated median SSV for patients showing nodal progression was 103 months (95% CI: 51-155 months); for patients without nodal progression, estimated SSV did not reach the median (P < 0.001). Nodal progression was an independent predictive parameter for shorter survival (P = 0.011). CONCLUSION: Multiple cutaneous lesions at presentation, early skin relapse and nodal progression portrait worse prognosis in patients with PCALCL.


Subject(s)
Lymphoma, Primary Cutaneous Anaplastic Large Cell/mortality , Lymphoma, Primary Cutaneous Anaplastic Large Cell/pathology , Disease Progression , Female , Humans , Lymphatic Metastasis , Male , Middle Aged , Neoplasm Staging , Prognosis , Retrospective Studies , Spain , Survival Rate
5.
Clin Exp Dermatol ; 43(2): 137-143, 2018 Mar.
Article in English | MEDLINE | ID: mdl-28994134

ABSTRACT

BACKGROUND: Data regarding response to treatment in lymphomatoid papulosis (LyP) are scarce. AIM: To assess the daily clinical practice approach to LyP and the response to first-line treatments. METHODS: This was a retrospective study enrolling 252 patients with LyP. RESULTS: Topical steroids, methotrexate and phototherapy were the most common first-line treatments, prescribed for 35%, 20% and 14% of the patients, respectively. Complete response (CR) was achieved in 48% of treated patients. Eczematous lesions significantly increased relative risk (RR) of not achieving CR (RR = 1.76; 95% CI 1.16-2.11). Overall median time to CR was 10 months (95% CI 6-13 months), and 78% of complete responders showed cutaneous relapse; both results were similar for all treatment groups (P > 0.05). Overall estimated median disease-free survival (DFS) was 11 months (95% CI 9-13 months) but DFS for patients treated with phototherapy was 23 months (95% CI 10-36 months; P < 0.03). Having the Type A LyP variant (RR = 2.04; 95% CI 0.96-4.30) and receiving a first-line treatment other than phototherapy (RR = 5.33; 95% CI 0.84-33.89) were significantly associated with cutaneous early relapse. Of the 252 patients, 31 (13%) had associated mycosis fungoides unrelated to therapeutic approach, type of LyP or T-cell receptor clonality. CONCLUSIONS: Current epidemiological, clinical and pathological data support previous results. Topical steroids, phototherapy and methotrexate are the most frequently prescribed first-line treatments. Although CR and cutaneous relapse rates do not differ between them, phototherapy achieves a longer DFS. Presence of Type A LyP and use of topical steroid or methotrexate were associated with an increased risk of early relapse.


Subject(s)
Antimetabolites, Antineoplastic/therapeutic use , Lymphomatoid Papulosis/drug therapy , Methotrexate/therapeutic use , Phototherapy , Skin Neoplasms/drug therapy , Steroids/therapeutic use , Administration, Topical , Adolescent , Adult , Aged , Aged, 80 and over , Child , Child, Preschool , Disease-Free Survival , Female , Humans , Infant , Lymphomatoid Papulosis/mortality , Lymphomatoid Papulosis/therapy , Male , Middle Aged , Mycosis Fungoides/mortality , Neoplasms, Multiple Primary , Receptors, Antigen, T-Cell , Retrospective Studies , Skin Neoplasms/mortality , Skin Neoplasms/therapy , Young Adult
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