Author/Editor		Zver, Samo
Title		Sistemska mastocitoza
Translated title		Systemic mastocytosis
Type		članek
Source		Zdrav Vestn
Vol. and No.		Letnik 70, št. 1
Publication year		2001
Volume		str. 23-5
Language		slo
Abstract		Background. Systemic mastocytosis is rare disease and it is incidence is not known. Skin is frequently involved in systemic mastocytosis and the most common skin manifestation is urticaria pigmentosa. Patients and methods. In 6-year period three patients with systemic mastocytosis were treated in Ljubljana region. Each of them had different subtype of disease. Systemic mastocytosis is often accompanied by hematological disease. Clinical picture is manifested by skin rashes, episodical skin redness with attacks of tachycardia, hypotension and syncopas, headache, nausea, diarrhoea, reduced body weight and disfunction of organic system infiltrated with mastocytes. Conclusions. Treatment is ussually symptomatical with corticosteroids, histamine receptors antagonists, proton pumpinhibitors, threecyclic antidepressives and natrium chromoglycate. Also newer treatment modalities such as interferon alpha injections and allogeneic stem cell transplantation are possible in subgroup of patients. Systemic mastocytosis usually progresses slowly, but concommitant hematological disease worsens prognosis of the disease.
Summary		Izhodišča. Sistemska mastocitoza je redka bolezen, tako da njena incidenca ni znana. Pogosto je v sklopu bolezni prizadeta tudi koža in najpogosteje gre za urticario pigmentoso. Slednjo še najpogosteje vidijo v dermatoloških ambulantah. Bolniki in metode. V 6-letnem obdobju smo v ljubljanski regiji zdravili tri bolnike s sistemsko mastocitozo, vsak pa je imel drugačno podvrsto bolezni. V grobem ločimo že navedeno kožno obliko mastocitoze, kjer je prizadeta samo koža, in sistemsko obliko mastocitoze s hkratno prizadetostjo kože ali brez te prizadetosti. Sistemsko obliko mastocitoze pogosto spremlja pridružena hematološka bolezen. V klinični sliki prevladujejo kožni izpuščaji, napadi rdečice telesa in hitrega bitja srca shipotenzijo, sinkope, glavoboli, hujšanje, slabosti, diareje in moteno delovanje z mastociti infiltriranega organa. Zaključki. Sistemsko mastocitozo simptomatsko zdravimo z glukokortikosteroidi, antagonisti histaminskih receptorjev, zaviralci protonske črpalke, tricikličnimi antidepresivi in natrijevim kromoglikatom. V zadnjih letih so opisani tudi primeri zdravljenja z interferonom alfa in alogenično presaditvijo krvotvornih matičnih celic. Bolezen praviloma napreduje počasi, sočasna hematološka bolezen pa pomeni krajše preživetje bolnikov.
Descriptors		MASTOCYTOSIS ADULT URTICARIA PIGMENTOSA INTERFERON-ALPHA