Avtor/Urednik		Modic, Mojca; Zver, Samo
Naslov		Zdravljenje kronične idiopatične trombotične trombocitopenične purpure s splenektomijo
Prevedeni naslov		Management of chronic idiopathic thrombotic thrombocytopenic purpura with splenectomy
Tip		članek
Vir		Zdrav Vestn
Vol. in št.		Letnik 71, št. 7-8
Leto izdaje		2002
Obseg		str. 453-5
Jezik		slo
Abstrakt		Background. Treatment of thrombotic thrombocytopenic purpura (TTP) with plasma exchange (PE) has reduced mortality rates from 90% in untreated patients to less than 20%. Despite primary effective PE, relapses occur in up to 40%. Despite primary effective PE, relapses occur in up to 40% of patients. Beside PE, second line treatment options are corticosteroids, vincristine, defibrotide, prostacycline. We describe two patients with relapsing TTP treated successfully with splenectomy. Patients and methods. Case 1. A 20-year female was diagnosed in October 2000 with idiopathic chronic relapsing TTP. First episode of PE plus methylprednisolone treatment led to complet remission. Subsequent relapses occured 3, 6 and 8 months after first TTP episode. Every relaps was effectivelly treated with PE and methylprednisolone. Because of more and more frequent TTP relapses laparoscopic splenectomy was performed. Currently, 7 months after splenectomy she is in complet TTP remission. Case Ž. A 18-year female, was diagnosed in January 1997 with idiopathic TTP. After PE and methylprednisolone complete remission was established. Relaps occured after nearly three years. Later on, TTP got chronic course with neurological clinical symptoms and plateled drops as soon as PE treatment was withdrawn. Besides PE and methylprednisolone she received also defibrotide. Because of chronic, PE dependant TTP, laparoscopic splenectomy was performed. Today, 15 months after splenectomy she is in complete TTP remission. Conclusions. In idiopathic relapsing and chronic TTP, PE treatment only is usually not sufficient for durable remission. Second line TTP treatment drugs have limited efficacy. Based on our experience in patients with relapsing and/or chronic TTP, laparoscopic splenectomy should be considered as a first treatment option, especialy in junger patients.
Izvleček		Izhodišča. Zdravljenje trombotične trombocitopenične purpure (TTP) splazmaferezo (PF) je zmanjšalo smrtnost bolnikov od 90% pri nezdravljenih na manj kot 20%. Kljub učinkovitosti PF se bolezen ponovi pri več kot 40% bolnikov. Poleg PF zdravimo te bolnike tudi z glukokortikoidi, imunoglobulini, vinkristinom, defibrotidom, prostaciklinom. Opisujemo 2 bolnici s kronično ponavljajočo TTP, ki smo ju uspešno zdravili s splenektomijo. Bolniki in metode. Primer 1. Pri 20-letni bolnici smo oktobra 2000 potrdili idiopatično kronično ponavljajočo TTP. V začetku smo jo zdravili s PF in metilprednisolonom in dosegli popolno remisijo. Vendar se je bolezen ponovila po 3, 6 in 8 mesecih po prvi epizodi TTP. Vsako ponovitev TTP smo zdravili ponovno s PF in metilprednisolonom. Ker so bile ponovitve bolezni vedno bolj pogoste, smo se odločili za laparoskopsko splenektomijo. Sedem mesecev po splenektomiji je bolnica še vedno v popolni remisiji. Primer 2. 18-letni bolnici smo januarja 1997 ugotovili idiopatično TTP. Zdravili smo jo s PF in metilprednisolonom ter dosegli popolno remisijo. Po treh letih se je bolezen ponovila. Dobila je kronični potek z nevrološkimi znaki in trombocitopenijo, čim smo prekinili zdravljenje s PF. Bolnica je poleg PF prejemala tudi metilprednisolon in defibrotid. Odločili smo se za laparoskopsko splenektomijo. 15 mesecev po splenektomiji ugotavljamo še vedno popolno remisijo TTP. Zaključki. Pri idiopatični kronični in ponavljajoči se TTP zdravljenje s PF ni dovolj za trajno remisijo bolezni. Tudi druga imunosupresivna zdravila, ki jih uporabljamo, niso dovolj za vzdrževanje remisije. Glede na naše izkušnje menimo, da je pri idiopatični kronični in/ali ponavljajoči se TTP zdravljenje s splenektomijo učinkovito, posebno pri mlajših bolnikih.
Deskriptorji		PURPURA, THROMBOCYTOPENIC, IDIOPATHIC SPLENECTOMY METHYLPREDNISOLONE ADULT PLASMA EXCHANGE REMISSION INDUCTION