RESUMEN
BACKGROUND AND OBJECTIVE: Primary cutaneous lymphomas are uncommon. This article describes the Primary Cutaneous Lymphoma Registry of the Spanish Academy of Dermatology and Venereology (AEDV) and reports on the results from the first year. PATIENTS AND METHODS: Disease registry for patients with primary cutaneous lymphoma. The participating hospitals prospectively recorded data on diagnosis, treatment, tests, and disease stage for all patients with primary cutaneous lymphoma. A descriptive analysis was performed. RESULTS: In December 2017, the registry contained data on 639 patients (60% male) from 16 university hospitals. The most common diagnoses, in order of frequency, were mycosis fungoides/Sézary syndrome (MF/SS) (348 cases, 55%), primary cutaneous B-cell lymphoma (CBCL) (184 cases, 29%), primary cutaneous CD30+ T-cell lymphoproliferative disorder (CD30+ CLPD) (70 cases, 11%), and other types of T-cell lymphoma (37 cases, 5%). In total, 105 (16.5%) of the cases recorded were incident cases. The most common diagnosis in the MF/SS group was classic MF (77.3%). Half of the patients with MF had stage IA disease when diagnosed, and the majority were either in partial remission (32.5%) or had stable disease (33.1%). The most widely used treatments were topical corticosteroids (90.8%) and phototherapy. The most common form of primary CBCL was marginal zone lymphoma (50%). Almost all of the patients had cutaneous involvement only and nearly half had stage T1a disease. Most (76.1%) were in complete remission. The main treatments were surgery (55.4%) and radiotherapy (41.9%). The most common diagnosis in patients with CD30+ CLPD was lymphomatoid papulosis (68.8%). Most of the patients (31.4%) had stage T3b disease and half were in complete remission. The most common treatments were topical corticosteroids (68.8%) and systemic chemotherapy (32.9%). CONCLUSION: The characteristics of patients with primary cutaneous lymphoma in Spain do not differ from those described in other series in the literature. The registry will facilitate clinical research by the AEDV's lymphoma group.
Asunto(s)
Linfoma de Células B/epidemiología , Linfoma Cutáneo de Células T/epidemiología , Sistema de Registros , Neoplasias Cutáneas/epidemiología , Bases de Datos Factuales , Humanos , Linfoma de Células B/diagnóstico , Linfoma de Células B/terapia , Linfoma Anaplásico de Células Grandes/epidemiología , Linfoma Cutáneo de Células T/diagnóstico , Linfoma Cutáneo de Células T/terapia , Papulosis Linfomatoide/diagnóstico , Papulosis Linfomatoide/epidemiología , Estudios Prospectivos , España/epidemiologíaRESUMEN
Two patients with diffuse cutaneous leishmaniasis caused by Leishmania mexicana were treated with two leishmanicidal drugs (pentamidine and allopurinol) combined with recombinant interferon-gamma restoring Th-1 favouring conditions in the patients. Parasites decreased dramatically in the lesions and macrophages diminished concomitantly, while IL-12-producing Langerhans cells and interferon-gamma- producing NK and CD8 + lymphocytes increased in a reciprocal manner. The CD4+/CD8 + ratio in the peripheral blood normalized. During exogenous administration of interferon-gamma the parasites' capacity to inhibit the oxidative burst of the patients' monocytes was abolished. Even though Th-1-favouring conditions were restored, both patients relapsed two months after therapy was discontinued. We conclude that the tendency to develop a disease-promoting Th-2 response in DCL patients is unaffected by, and independent of, parasite numbers. Even though intensive treatment in DCL patients induced Th-1 disease restricting conditions, the disease-promoting immunomodulation of few persistent Leishmania sufficed to revert the immune response.
Asunto(s)
Antiprotozoarios/uso terapéutico , Interferón gamma/uso terapéutico , Células de Langerhans/efectos de los fármacos , Leishmania mexicana , Leishmaniasis Cutánea Difusa/tratamiento farmacológico , Leishmaniasis Cutánea Difusa/inmunología , Pentamidina/uso terapéutico , Alopurinol/uso terapéutico , Animales , Antimetabolitos/uso terapéutico , Relación CD4-CD8/efectos de los fármacos , Quimioterapia Combinada , Humanos , Células Asesinas Naturales/efectos de los fármacos , Células Asesinas Naturales/inmunología , Células de Langerhans/inmunología , Células de Langerhans/parasitología , Leishmaniasis Cutánea Difusa/patología , Proteínas Recombinantes , Estallido Respiratorio/efectos de los fármacos , Estallido Respiratorio/inmunología , Resultado del TratamientoRESUMEN
Chemotherapy-induced acral erythema is a peculiar localized cutaneous response to several chemotherapeutic agents, mostly antimetabolites. Taxol is a recently developed antineoplastic drug that acts on the mitotic spindle and does not interfere with nucleic acid synthesis. We describe the first case of taxol-induced acral erythema and report on additional data concerning the pathogenesis of this kind of toxic eruptions.