Author/Editor		Mlakar, Uroš
Title		Akutna promielocitna levkemia: retrospektivna analiza zdravljenja v Kliničnem centru Ljubljana v obdobju 1990-2000
Translated title		Acute promyelocytic leukaemia: a retrospective analysis of patients treated at the Ljubljana medical centre between 1990-2000
Type		članek
Source		Med Razgl
Vol. and No.		Letnik 39, št. Suppl 5
Publication year		2000
Volume		str. 101-7
Language		slo
Abstract		Background. Acute promyelocytic leukaemia (APL) is a specific subtype of acute myeloid leukaemia (AML) characterized by unique morphologic features, by the t (15;17) translocation that fuses the retinoic receptor gene to PMP gene and by coagulopathy. APL deserves special consideration because it is treated differently from all other subtypes of AML. It is also unique in its sensitivity to the differentiating agent, all-trans retinoic acid (ATRA). Methods. In the paper, we analysed the treatment of 25 patients with APL who were admitted to the Department of Haematology, University Medical Centre, Ljubljana over a 10-year period. Diagnosis of APL was made according to the FAB classification. The median age of the patients was 48 years (range 18-83 yrs). The median initial value of the leukocyte count was 47.8 x 10(9)/L (range 0.6-229 x 10(9)/L). Results. The median of the overall survival of 25 patients with APL was 18 months; the estimated rate of survival at 10 years was 31%. Seven patients were treated with chemotherapy alone, eleven patients with ATRA followed by chemotherapy and five patients with ATRA plus chemotherapy. Seventeen (17/23) patients achieved complete remission. Five patients died within seven days. The median relapse-free survival duration was 19 months and the estimated rate of relapse-free survival at 10 years was 39%. Initial leukocyte count less than 10 x 10(9)/L (p 0.05) was a prognostic factor for longer relapse-free survival. Conclusions. Important advances have been made over the past ten years in the management of APL and it becomes one of the most curable leukaemia of adults. (trunc. at 2000 chars)
Summary		Izhodišča. Akutna promielocitna levkemija (APL) je posebna vrsta akutne mieloblastne levkemije (AML) z značilno morfologijo levkemičnih celic, v katerih je prisotna translokacija t (15;17), ki združi gen za receptor retinoične kisline z genom PML. Pogosto so prisotne tudi hude motnje v koagulaciji. APL se razlikuje od drugih vrst AML tudi po drugačnem načinu zdravljenja, ker je občutljiva na transretinoično kislino (TRK), ki povzroči dozorevanje levkemičnih promielocitov. Metode. V prispevku smo analizirali 25 bolnikov z APL, ki smo jih obravnavali v obdobju desetih let na Kliničnem oddelku za hematologijo, KC Ljubljana. Za diagnozo smo uporabili diagnostične kriterije klasifikacije FAB. Mediana starosti bolnikov je bila 48 let (razpon 18-83 let), mediana števila levkocitov ob ugotovitvi bolezni pa 47,8 x 10(9)/L (razpon O,G-229 x 10(9)/L). Rezultati. Mediana preživetja 25 bolnikov je bila 18 mesecev, verjetnost 10-letnega preživetja pa 31 %. Sedem bolnikov smo zdravili samo s kemoterapijo, enajst zaporedno s TRK in kemoterapijo in pet hkrati s TRK in kemoterapijo. Pri sedemnajstih (17/23) smo dosegli remisijo, pet bolnikov je umrlo sedem dni po ugotovitvi bolezni. Mediana trajanja remisije je bila 19 mesecev, verjetnost 10-letnega preživetja brez relapsa pa 39 %. Remisija pri bolnikih s številom levkocitov pod 10 x 10(9)/L je bila daljša kot pri ostalih bolnikih (p = 0,05). Zaključki. V preteklih desetih letih se je uspešnost zdravljenja APL zelo izboljšala, zato sodi APL med levkemije odraslih z visoko stopnjo ozdravljivosti. Sedanja priporočila zdravljenja APL so:1. indukcija s sočasno kombinacijo TRK in antraciklinskega citostatika, 2. zgodnje prepoznavanje in zdravljenje sindroma TRK, 3. vsaj dva konsolidacijska kroga kemoterapije, 4. vzdrževalno zdravljenje s TRK v presledkih z neprekinjeno KT v nizkih odmerkih ali brez nje, 5. "molekularni nadzor" in zdravljenje molekularnega relapsa.
Descriptors		LEUKEMIA, PROMYELOCYTIC, ACUTE SURVIVAL ANALYSIS