The Role of Interferon in the Treatment of Cutaneous T-Cell Lymphoma

Interferon alfa se že mnogo let uspešno uporablja za zdravljenje kožnih T-celičnih limfomov in je za to bolezen še vedno nepogrešljivo zdravilo. Pri kožnih limfomih izkoriščamo predvsem njegovo antiproliferativno in imunomodulatorno delovanje. V sodobnih smernicah za zdravljenje kožnih T-celičnih limfomov je zdravilo izbire v stadijih IIB in III, kjer se pogosto uporablja v kombinaciji s sistemsko PUVA ali s sistemskim retinoidom. V monoterapiji se priporoča v stadijih IA do IIA, kadar so izčrpane lokalne možnosti zdravljenja. Kot vzdrževalno zdravljenje v manjših odmerkih pomembno podaljša remisijo. Režim zdravljenja prilagajamo individualno, glede na stadij bolezni, razširjenost kožnih sprememb in bolnikovo odzivnost na zdravilo. Zdravljenje je povezano z nekaterimi akutnimi in kroničnimi neželenimi sopojavi, ki so pogostejši pri večjih odmerkih, vendar so predvidljivi in praviloma reverzibilni.IFN-alpha has been used successfully for the treatment of cutaneous T-cell lymphoma for many years and it remains an indispensable drug for this condition. Its antiproliferative and immunomodulatory properties are used in the treatment of this disease. According to the current guidelines for the treatment of cutaneous T-cell lymphoma, it is considered as the first-line drug for stages IIB and III, where it is often administered concomitantly with systemic PUVA and retinoids. As monotherapy, it is recommended in stages IA and IIA, if skin directed therapies are not effective. As a maintenance therapy with low dosages, it is associated with prolongation of the duration of remissions. Optimal treatment regimen is adjusted individually, according to the stage of disease, dissemination of skin lesions and the patient’s tolerance for IFN. Several acute and chronic side effects can occur during the treatment with IFN-alpha, which are dose-related, predictable and often reversible

Dystrophic Epidermolysis Bullosa Inversa – Case Report and Review of the Literature

Dystrophic epidermolysis bullosa inversa is a very rare subtype of inherited dystrophic epidermolysis bullosa with a unique clinical manifes- tation. Generalized blistering in the neonatal period and in early infancy im- proves with age, with lesions becoming restricted to intertriginous areas, axial parts of the trunk, and mucous membranes. In contrast to other variants of dystrophic epidermolysis bullosa, the inverse type has a more favorable prog- nosis. We present a case of a 45-year-old female patient with dystrophic epi- dermolysis bullosa inversa, diagnosed in adulthood based on typical clinical presentation, transmission electron microscopic findings, and genetic analy- sis. Additionally, genetic analysis revealed that the patient also suffered from Charcot-Marie-Tooth disease, a hereditary motor and sensory neuropathy. To our knowledge, the coexistence of these two genetic diseases has not been reported so far. We describe clinical and genetic findings in the patient and re- view previous reports on dystrophic epidermolysis bullosa inversa. A possible temperature-related pathophysiology for the peculiar clinical manifestation is discussed

Dystrophic Epidermolysis Bullosa Inversa – Case Report and Review of the Literature

Dystrophic epidermolysis bullosa inversa is a very rare subtype of inherited dystrophic epidermolysis bullosa with a unique clinical manifes- tation. Generalized blistering in the neonatal period and in early infancy im- proves with age, with lesions becoming restricted to intertriginous areas, axial parts of the trunk, and mucous membranes. In contrast to other variants of dystrophic epidermolysis bullosa, the inverse type has a more favorable prog- nosis. We present a case of a 45-year-old female patient with dystrophic epi- dermolysis bullosa inversa, diagnosed in adulthood based on typical clinical presentation, transmission electron microscopic findings, and genetic analy- sis. Additionally, genetic analysis revealed that the patient also suffered from Charcot-Marie-Tooth disease, a hereditary motor and sensory neuropathy. To our knowledge, the coexistence of these two genetic diseases has not been reported so far. We describe clinical and genetic findings in the patient and re- view previous reports on dystrophic epidermolysis bullosa inversa. A possible temperature-related pathophysiology for the peculiar clinical manifestation is discussed

Priporočila za obravnavo bolnikov z malignimi limfomi

no abstractni abstrakt

Vloga interferona pri zdravljenju kožnih limfomov

IFN-alpha has been used successfully for the treatment of cutaneous T-cell lymphoma for many years and it remains an indispensable drug for this condition. Its antiproliferative and immunomodulatory properties are used in the treatment of this disease. According to the current guidelines for the treatment of cutaneous T-cell lymphoma, it is considered as the first-line drug for stages IIB and III, where it is often administered concomitantly with systemic PUVA and retinoids. As monotherapy, it is recommended in stages IA and IIA, if skin directed therapies are not effective. As a maintenance therapy with low dosages, it is associated with prolongation of the duration of remissions. Optimal treatment regimen is adjusted individually, according to the stage of disease, dissemination of skin lesions and the patient's tolerance for IFN. Several acute and chronic side effects can occur during the treatment with IFN-alpha, which are dose-related, predictable and often reversible.Interferon alfa se že mnogo let uspešno uporablja za zdravljenje kožnih T-celičnih limfomov in je za to bolezen še vedno nepogrešljivo zdravilo. Pri kožnih limfomih izkoriščamo predvsem njegovo antiproliferativno in imunomodulatorno delovanje. V sodobnih smernicah za zdravljenje kožnih T-celičnih limfomov je zdravilo izbire v stadijih IIB in III, kjer se pogosto uporablja v kombinaciji s sistemsko PUVA ali s sistemskim retinoidom. V monoterapiji se priporoča v stadijih IA do IIA, kadar so izčrpane lokalne možnosti zdravljenja. Kot vzdrževalno zdravljenje v manjših odmerkih pomembno podaljša remisijo. Režim zdravljenja prilagajamo individualno, glede na stadij bolezni, razširjenost kožnih sprememb in bolnikovo odzivnost na zdravilo. Zdravljenje je povezano z nekaterimi akutnimi in kroničnimi neželenimi sopojavi, ki so pogostejši pri večjih odmerkih, vendar so predvidljivi in praviloma reverzibilni

Primer bolnika z Mycosis fungoides

No abstract.Ni abstrakta

Priporočila za obravnavo bolnikov z malignimi limfomi

no abstractni abstrakt