Renata Tomaszewska

CENTRAL VENOUS ACCESS PORTS IN CHILDREN TREATED FOR HEMATOPOIETIC MALIGNANCIES

During past 10 years 234 central venous access ports (CVAP) were implanted in 225 patients at the Department of Pediatric Hematology and Oncology in Zabrze. Mean exposure time was 745 days and total implantation time reached 173,768 days. Complications were encountered in 17 patients (7.6%). This mainly concerned central venous line infection, which led to removal of 10 CVAP (4.4%). The remaining complications necessitating removal of the CVAP consisted mainly of mechanical problems (catheter fracture, occlusion, and erroneous implantation to artery). In the opinion of the authors, subcutaneously implanted CVAP are a safe and effective option for high-dose chemotherapy deliverance in childhood cancer patients.

Outcome of refractory and relapsed acute myeloid leukemia in children treated during 2005–2011 – experience of the Polish Pediatric Leukemia/Lymphoma Study Group (PPLLSG)

Aim of the study Recent studies showed relatively better outcome for children with refractory (refAML) and relapsed acute myeloid leukemia (relAML). Treatment of these patients has not been unified within Polish Pediatric Leukemia/Lymphoma Study Group (PPLLSG) so far. The goal of this study is to analyze the results of this therapy performed between 2005–2011. Material and methods The outcome data of 16 patients with refAML and 62 with relAML were analyzed retrospectively. Reinduction was usually based on idarubicine, fludarabine and cytarabine with allogenic hematopoietic stem cell transplant (alloHSCT) in 5 refAML and 30 relAML children. Results Seventy seven percent relAML patients entered second complete remission (CR2). Five-year OS and disease-free survival (DFS) were estimated at 16% and 30%. The outcome for patients after alloHSCT in CR2 (63%) was better than that of those not transplanted (36%) with 5-year OS of 34% vs. 2-year of 7% and 5-year DFS of 40% vs. 12.5%. Second complete remission achievement and alloHSCT were the most significant predictors of better prognosis (p = 0.000 and p = 0.024). The outcome of refAML children was significantly worse than relAML with first remission (CR1) rate of 33%, OS and DFS of 25% at 3 years and 53% at 2 years, respectively. All survivors of refAML were treated with alloHSCT after CR1. Conclusions The uniform reinduction regimen of the documented efficacy and subsequent alloHSCT in remission is needed to improve the outcome for ref/relAML children treated within PPLLSG. The focus should be on the future risk-directed both front and second line AML therapy.

SIALIC ACID CONCENTRATION IN DIFFERENT STAGES OF MALIGNANT LYMPHOMA AND LEUKEMIA IN CHILDREN

Sialic acid concentration in the blood serum of 104 children suffering from leukemia and malignant lymphomas was determined. A significant higher concentration of sialic acid was found at the onset of the disease, compared to the controls. In the group of children suffering from acute lymphoblastic leukemia a dependence was noted between the stage of the disease and the concentration of sialic acid in the serum. The high concentration of sialic acid at the onset of the disease, although decreasing during treatment, rose again during relapses of the disease. It seems that determination of sialic acid concentration may be useful in monitoring treatment.

Outcome of acute lymphoblastic leukemia in children with down syndrome—Polish pediatric leukemia and lymphoma study group report

ABSTRACT Children with Down syndrome (DS) have a 20-fold increased risk of developing leukemia compared with the general population. The aim of the study was to analyze the outcome of patients diagnosed with Down syndrome and acute lymphoblastic leukemia (ALL) in Poland between the years 2003 and 2010. A total of 1848 children were diagnosed with ALL (810 females and 1038 males). Of those, 41 (2.2%) had DS. The children were classified into three risk groups: a standard-risk group—14 patients, an intermediate-risk group—24, a high-risk group—3. All patients were treated according to ALLIC 2002 protocol. The median observation time of all patients was 6.1 years, and in patients with DS 5.3 years. Five-year overall survival (OS) was the same in all patients (86% vs 86%, long-rank test, p = .9). The relapse-free survival (RFS) was calculated as 73% in patients with DS and 81% in patients without DS during a median observation time (long-rank test, p = .3). No statistically significant differences were found in the incidence of nonrelapse mortality between those two groups of patients (p = .72). The study was based on children with ALL and Down syndrome who were treated with an identical therapy schedule as ALL patients without DS, according to risk group. This fact can increase the value of the presented results.

Individualized tumor response testing profile has a prognostic value in childhood acute leukemias: multicenter non-interventional long-term follow-up study

Abstract A total number of 817 children with acute lymphoblastic leukemia (ALL) and 181 with acute myeloblastic leukemia (AML) were assessed for individualized tumor response testing (ITRT) profile as a prognostic factor in long-term follow-up. For each patient, ITRT, initial response to therapy and long-term outcome were assessed. In initial ALL, an impact on long-term response was shown in ITRT for 13 drugs, while in initial AML only for cytarabine. For patients with ALL, a combined five-drug ITRT profile for prednisolone, l-asparaginase, vincristine, cytarabine and daunorubicin or doxorubicin had predictive value for probability of disease-free survival (pDFS) in univariate analysis, whereas in multivariate analysis, bone marrow response by day 33 was the only prognostic factor. For patients with AML, no factor had prognostic value for pDFS in univariate analysis, while ITRT to cytarabine almost reached significance. In conclusion, ITRT can possibly be regarded as a risk factor in childhood acute leukemias.

Niedokrwistość autoimmunohemolityczna o ciężkim przebiegu u 2,5-miesięcznego niemowlęcia ☆ ☆☆

Przedstawiono 2,5-miesieczne niemowle, u ktorego rozwinela sie ciezka niedokrwistośc autoimmunohemolityczna, wymagająca bardzo czestych przetoczen koncentratow krwinek czerwonych. W leczeniu stosowano steroidy, immunoglobuliny w duzych dawkach i.v., uzyskując efekt kliniczny po 3,5 miesiącach. Pomocny w leczeniu zakazenia HSV byl acyclovir.