| Abstract | Kronična limfocitna leukemija (KLL) je B-stanična limfoproliferativna bolest. Klasična definicija KLL uključuje broj limfocita u krvi >5x109/L i infiltraciju koštane srži s >30% limfocita i obično izražava ekspresiju CD5, CD23, CD20 i CD19 antigena. To je bolest starije životne dobi i incidencija joj raste s dobi i dva puta je češća u muškaraca. U svijetu se koristi nekoliko klasifikacija uznapredovalosti KLL: Raijeva, Binetova i klasifikacija TTM. KLL može biti asimptomatska, ali se može očitovati i širokim spektrom simptoma kao što su limfadenopatija, splenomegalija, hepatomegalija i nespecifičnim simptomima kao što su umor, bljedoća i gubitak tjelesne mase. Dijagnoza se postavlja na temelju anamneze i fizikalnog pregleda, krvnih testova, morfološke analize koštane srži, imunofenotipskih pretraga i citogenetskih pretraga. Nepovoljni prognostički čimbenici su starija životna dob (>65 g.), muški spol, visoki klinički stadij (stadij 3 i 4 prema Raievoj klasifikaciji, stadij C prema Binetovoj klasifikaciji), mutacije p53, povišena koncentracija β2 mikroglobulina u krvi i LDH, izražaj CD38 jednak ili veći od 30% i izražaj ZAP-70 jednak ili veći od 20%. KLL je neizlječiva bolest, ali terapijom se može produžiti život i ublažiti simptome. Samo jedna trećinu novodijagnosticiranih bolesnika trebat će odmah liječiti. Razlikujemo prvu i drugu liniju terapije KLL. FCR kemoterapija je zlatni standard 1. linije terapije. Može se još koristiti i ibrutinib, klorambucil, idelalisib, a sve veća je upotreba monoklonskih antitijela. Drugu liniju liječenja koristimo kada je došlo do relapsa bolesti te se uglavnom daju inhibitori kinaze ibrutinib, idelalisb i venetoklast. Moguće je i provesti transplantaciju matičnih stanica. KLL je indolentna hematološka neoplazma te se očekuje petogodišnje preživljenje u 69% bolesnika. Očekivano petogodišnje preživljenje skraćuju nepovoljni prognostički čimbenici, posebno mutacija TP53. |
| Abstract (english) | Chronic lymphocytic leukemia (CLL) is a B-cell lymphoproliferative disorder. The diagnosis of CLL requires the presence of 5x109/L lymphocytes in the peripheral blood and infiltration of bone marrow more than 30% of lymphocytes and also expression of CD5, CD20, CD19 and CD23 antigenes. This disorder usually affects people of older age and the incidence of CLL increases with age and it is twice as common in men. There are three staging system: Rai, Binet, and Total tumor mass score. CLL can be asymptomatic or may have a wide range of symptoms. It may include lymphadenopathy, splenomegaly, hepatomegaly and nonspecific symptoms like extreme fatigue, paleness and unintentional weight loss. The diagnostic procedure includes a medical history and physical examination, blood tests, morphologic analysis of bone marrow, immunophenotyping search and cytogenetic search. Unfavorable prognostic factors are: elderly age, male sex, high clinical staging (Rai 3 and 4, Binet C), gene mutations in TP53, high level of beta-2 microglobulin, LDH, expression of CD38 equal or higher than 30%, and expression of ZAP-70 equal or higher than 20%. CLL is an incurable disorder but therapy can prolong life and decrease symptoms Only one third of newly diagnosed patients should be treated immediately. First-and second-line treatments are available. The “gold standard“ is FCR chemotherapy. It may also involve Ibrutinib, Idelalisb, and Chlorambucil. Monoclonal antibodies are increasingly used for therapeutic purposes as well. Second-line treatment with kinase inhibitors such as Ibrutinib, Idelalisb, and Venetoklast is the choice for patients with a relapse. Also, one of the options is allogeneic stem cell transplantation. CLL is an indolent hematological neoplasm and five-year survival rate is expected in more than 69% patients. The five-year survival rate is shorter when a patient has unfavorable prognostic factors, especially genetic mutations in TP53. |
