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  • 1 Pécsi Tudományegyetem, Általános Orvostudományi Kar, Klinikai Központ, I. számú Belgyógyászati Klinika, Hematológiai Tanszék, 7624 Pécs, Ifjúság út 13.
  • | 2 Debreceni Egyetem, Általános Orvostudományi Kar, Klinikai Központ, II. számú Belgyógyászati Klinika, Hematológiai Tanszék, Debrecen
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Összefoglaló. Az akut promyelocytás leukémia (APL) ritka, de jól gyógyítható leukémiatípus. Az APL-es betegek gondozását centralizáltan, az APL kezelésében jártas kórházakban kell végezni, ahol elérhető hematológiai intenzív ellátás. Az APL terápiája az elmúlt két évtizedben rohamosan fejlődött az igen aktív hatású gyógyszerek, a tretinoin és az arzén-trioxid bevezetésével. Immáron a hagyományos citotoxikus kemoterápia alkalmazása nélkül kezelhető a betegség. A legújabb klinikai irányelvek többféle kezelési stratégiát tartalmaznak, ezek egy része továbbra is alkalmazza a citotoxikus kemoterápiát. Ily módon számos kezelési lehetőség áll a gyakorló hematológus rendelkezésére, amikor egy új APL-es beteg ellátását tervezi. Az elmúlt három évtized multicentrikus vizsgálatai igazolták az ATRA + kemoterápia, majd később a kemoterápiával vagy anélkül adott ATRA + ATO alkalmazásának hatásosságát. Az APL optimális kezeléséhez azonban a diagnózis korai felállítása, az agresszív szupportív beavatkozások bevezetése, a kezeléssel összefüggő szövődmények megfelelő rendezése és a mérhető reziduális betegség (az MRD) monitorozása is hozzátartozik. A szerzők az akut promyelocytás leukémia legújabb kezelési irányelveit foglalják össze.

Summary. Acute promyelocytic leukemia (APL) is an uncommon but highly curable leukemia. Treatment of APL patients should be centralised in hospitals with proven experience in APL treatment and haematological intensive care. The treatment of acute promyelocytic leukemia (APL) has evolved rapidly in the past two decades after the introduction of highly active drugs, including tretinoin (all-trans-retinoic acid) and arsenic trioxide. It is now possible to treat this disease without the use of traditional cytotoxic chemotherapy. Today’s clinical guidelines include multiple regimens, some of which continue to use cytotoxic chemotherapy. This leaves the practicing oncologist with multiple treatment options when faced with a new case of APL. Multicenter studies over the past three decades have demonstrated the efficacy of ATRA plus chemotherapy and, subsequently, of ATRA plus ATO, with or without chemotherapy. However, the optimal management of APL also requires early diagnosis, institution of aggressive supportive measures, appropriate management of treatment-related complications and monitoring of measurable residual disease (MRD). The authors summarizes the updated guidelines in the management of acute promyelocytic leukemia.

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