Ultra‑ high‑risk chronic lymphocytic leukemia –  characteristics and treatment options


Authors: D. Lysák;  P. Jindra
Authors‘ workplace: Hematologicko‑onkologické oddělení FN Plzeň, přednosta prim. MU Dr. Pavel Jindra, Ph. D.
Published in: Vnitř Lék 2013; 59(10): 887-894
Category: Review

Overview

Chronic lymphocytic leukemia (CLL) is the most common form adult leukemia in western world. The disease is typically cha­racterized by heterogeneous clinical behavior ranging from indolent course to rapidly progressive disease. Using clinical and bio­logical factors we can stratify patients with CLL and prospectively identify those who can be expected unfavorable course. There is a special group known as ultra high‑risk chronic lymphocytic leukemia with an extremely poor prognosis. These are about 10– 15% of all patients with CLL. They do not respond to standard treatment and their survival is short with a median of 2– 3 years. For high‑risk patients are considered: patients with a proven TP53 defect, refractory to purine analogues or with early relapse after chemoimmunotherapy based on fludarabine (≤ 24 months). While the standard 1st line treatment protocol in younger patients is chemoimmunotherapy FCR, in case of ultra‑ high‑risk CLL other methods like allogeneic hematopoietic stem cell transplantation or clinical trials testing the new drugs should be considered. In particular, allogeneic hematopoietic stem cell transplantation is a very promising treatment modality that offers long‑term disease control and cure regardless of the unfavorable CLL subtype. Transplantation treatment should be therefore considered in all younger patients with ultra‑ high‑risk CLL, who should be without delay referred to a center for intensive hematological treatment.

Key words:
chronic lymphocytic leukemia –  high‑risk cytogenetics –  allogeneic transplantation


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