Primary central nervous system lymphoma — a review of current therapeutic strategies

Pierwotny chłoniak ośrodkowego układu nerwowego (PCNSL) jest wysoce agresywnym klinicznie chłoniakiem pozawęzłowym umiejscowionym w mózgowiu, rdzeniu, oponach mózgowych, nerwach czaszkowych i/lub gałce ocznej. Stanowi około 3% rozpoznań wszystkich guzów mózgu oraz 2–3% chłoniaków nie-Hodgkina. Aż 95% przypadków PCNSL stanowi chłoniak rozlany z dużych komórek B (DLBCL), pozostałe to chłoniaki wysoce agresywne (chłoniak Burkitta, chłoniak limfoblastyczny) oraz chłoniak strefy brzeżnej i chłoniaki z komórek T. Od lat 70. XX wieku zachorowalność na PCNSL wzrasta. Pomimo wielu podobnych cech w obrazie patomorfologicznym PCNSL rokuje znacznie gorzej od DLBCL, NOS (not otherwise specified, inaczej nieokreślony). W aktualnie obowiązującej klasyfikacji nowotworów układu krwiotwórczego i limfoidalnego Światowej Organizacji Zdrowia (WHO 2008) wyróżniono DLBCL CNS jako odrębną jednostkę diagnostyczną.  Primary central nervous system lymphoma (PCNSL) is a highly aggressive extranodal lymphoma subtype arising in the brain parenchyma, spinal cord, meninges, cranial nerves, and/or intraocularly. PCNSL accounts for 3% of brain tumours and 2–3% cases of non-Hodgkin’s lymphoma. Diffuse large B-cell lymphoma (DLBCL) is a primary diagnosis in 95% of all PCNSL, with highly aggressive lymphomas (Burkitt’s lymphoma, lymphoblastic lymphoma) and marginal zone lymphoma (MZL) or T-cell lymphomas accounting for the other 5%. Over the last 40 years, PCNSL rates have been increasing. Although PCNSL shares many histopathological features with DLBCL (not otherwise specified; NOS), its prognosis is generally far worse. The current WHO 2008 classification for cancers of the haematopoietic system and lymphomas assigns DLBCL CNS into a distinct diagnostic entity of lymphoma

Plerixafor for patients who fail cytokine-or chemotherapy-based stem cell mobilization: Results of a prospective study by the Polish Lymphoma Research Group (PLRG)

Autologous hematopoietic stem cell transplantation (autoHSCT) requires collection of sufficient number of hematopoietic stem cells. The goal of this study was to evaluate efficacy of plerixafor used in patients with lymphoid malignancies failing conventional stem cell mobilization.This was a prospective, non-interventional study. All consecutive patients (n = 109) treated with plerixafor in 11 centers were reported. The drug was used either in case of previous mobilization failure (n = 67) or interventionally, in case of insufficient CD34 cell output during current mobilization (n = 42). Successful mobilization was defined as resulting in collection of ≥ 2 × 10 CD34 cells/kg for single autoHSCT or ≥ 4 × 10 CD34 cells/kg for double procedure.The overall rate of successful mobilization was 55% (55% for single and 56% for double autoHSCT). The median total number of collected CD34 cells/kg was 2.4 (range, 0-11.5) for patients intended for a single transplantation while 4.0 (0.6-16.9) for double procedure. The number of circulating CD34 cells increased after the use of plerixafor regardless of baseline values. The median fold increase was 3.3 (0.3-155). Data from this observational study confirm high efficacy of plerixafor used in routine clinical practice as salvage for patients with lymphoid malignancies failing conventional stem cell mobilization

Analysis of ibrutinib efficacy in a subgroup of chronic lymphocytic leukemia patients with 17p deletion: observational study of the Polish Adult Leukemia Group (PALG)

BackgroundThe 17p deletion is regarded as the strongest poor prognostic factor in chronic lymphocytic leukemia (CLL). Results of recently performed clinical trials have suggested that ibrutinib significantly improves the outcome in this patient group.AimThe study aimed at analyzing the efficacy and adverse events profile of ibrutinib monotherapy in CLL patients with 17p deletion treated in routine clinical practice outside clinical trials.Materials and MethodsClinical response and adverse events profile of ibrutinib monotherapy were assessed in thirty-five CLL patients with 17p deletion treated within the ibrutinib named patients program in Poland.ResultsOverall response rate was 80% (28/35 patients) with median observation time of 24.2 months (range 0,1 – 30,9). Complete remission was observed in 5 patients (14.3%), partial remission in 11 (31.4%), partial remission with lymphocytosis in 13 (37.1%), whereas stable disease and progression was noted in 4 (11.4%) and 1 (2.9%) respectively. Response was not assessed in 1 patient. Median progression-free survival was 29.5 months, whereas median overall survival was not reached. Eleven patients died (7 because of infection, 1 of CLL progression, 1 of sudden cardiac death, 1 of disseminated breast cancer and 1 of unknown causes). In 13 patients (37.1%) at least one 3 or 4 grade adverse event occurred. In 11 patients (31.4%) the treatment was temporary withheld or the dose reduced due to adverse events.ConclusionIbrutinib is characterized by high clinical efficacy and acceptable toxicity in CLL patients with 17p deletion in daily clinical practice