Correction to: Primary immunodeficiencies in Central and Eastern Europe-the power of networking Report on the activity of the Jeffrey Modell Foundation Centers Network in Central and Eastern Europe.

Due to authors' internal mistake they have misspelled the name of one of the co-authors in this article.

Primary immunodeficiencies in Central and Eastern Europe-the power of networking Report on the activity of the Jeffrey Modell Foundation Centers Network in Central and Eastern Europe.

Jeffrey Modell Foundation centers' network activities in Central and Eastern Europe (JMF CEE) have contributed to the development of care for patients with primary immunodeficiencies. On the data continuously collected from individual centers in participating countries since 2011, we demonstrate a steady improvement in a number of aspects concerning complex care for patients with primary immunodeficiencies. The presented data show an improvement of awareness about these rare diseases across the whole Central and Eastern European region, an increase in newly diagnosed patients as well as genetically confirmed cases, earlier establishment of diagnosis, and improved access to clinical treatment. We also present an active patient involvement that is reflected in the expansion of patient organization centers and their activities. The cooperation within the JMF CEE network has also contributed to greater international exposure of participating centers and further to the gradual development of research activities in the rapidly evolving field of primary immunodeficiencies. The improvement of all important aspects of the complex field of primary immunodeficiencies within the JMF CEE network documents the strength and advantages of the joint and coordinated networking.

[Evaluation of Photo- and Cytotoxicity of Chlorin e6 Ester Derivatives and Their Liposomal Forms].

Photophysical characteristics and photosensitizing activity of the chlorin e6 dimethyl and trimethyl ester derivatives in various solution and their liposomal forms were studied. It was shown that in lipid vesicles chlorin e6 ester derivatives are predominantly in the monomeric state and possess optimal photophysical properties and high photochemical activity. The rate of redistribution of the chlorin e6 dimethyl ester from lipid vesicle to cells was higher as compared with that one of the chlorin e6 trimethyl ester. The increase of the serum concentration in the incubation medium has a different effect on processes of accumulation of the liposomal forms of the chlorin e6 dimethyl and trimethyl ester derivatives by the cells. Cell culture studies showed that application of liposomal forms of the chlorin e6 dimethyl and trimethyl ester derivatives significantly decreases their cytotoxicity but keeps high cytotoxic effect of photodynamic activity of the chlorin e6 ester derivatives.

[Use of LAK-cells and systemic chemotherapy with hyperthermia in the management of chemo-resistant tumors].

Tentative results of LAK-cell and whole-body hyperthermia (WBH) were evaluated in 19 children with advanced chemorefractory tumors. LAK-cells were obtained by extracorporeal incubation of peripheral blood lymphocytes: a germ-cell rhabdomyosarcoma was detected in 4, Askin's tumor--2--2, renal cell carcinoma--2 and miscellaneous--7. Autologous LAK-cells were infused twice: on completion of WBH as body temperature fell to as low as (+) 40 deg. C and on day after WBH. The latter was well tolerated. Complete or partial response to thermochemobiotherapy was reported in 8 patients. Overall 5-year survival was 43% (median follow-up--12.6 months).

[Role of three-color flow cytofluorometry in the algorithm for detection of minimal residual disease in children with acute lymphoblastic and acute myeloblastic leukemia].

Three-color flow cytofluorometry (FCF) was used to identify minimal residual disease in 124 patients with acute lymphoblastic leukemia (ALL) treated in accordance with the ALL-myeloblastic leukemia (MBL)-2002/2008 protocol and 36 patients with MBL treated under the MBL-MM-2000 protocol. It was shown that approximately a fifth of children with ALL, 3-color FCF could not monitor minimal residual disease, which required the use of 4- or more-color FCF and parallel determination in these MBL samples by molecular genetic methods. An algorithm of study of MBL by FCF, by additionally using molecular genetic methods, is presented.

[Drug sensitivity of tumor cells in varieties of acute childhood leukemia].

Drug sensitivity of tumor cells was studied in 154 children diagnosed with acute leukemia. Cellular sensitivity in acute lymphoblastic leukemia (ALL) was higher than in acute myeloid one (AML), while T-line ALL cells were more sensitive to dexamethasone than those of B-line. It was suggested that lower drug sensitivity of ALL cells at the earlier stages of cell differentiation was due to their reduced susceptibility to apoptosis. Among AML cells, M3 variant was less sensitive to cytozar while M4 - to vepeside. Reduced sensitivity to vincristine was found in series M3 > M1 - M2 > M4 while that to L-asparaginase was in M1- M2 > M4 > M3.

[Effect of interleukin-2 on apoptosis and function of human lymphocytes in in-vitro cytostatics culture].

In vitro experiments were conducted to determine the impact of interleukin-2 (IL-2) on apoptosis and function of cytostatic-cultured lymphoid (mononuclear) cells (MNC) of peripheral blood from healthy subjects and children with cancer. Neither slight or any effect on vepesid-16 or carboplatin--cultured MNC apoptosis, nor any phytohemagglutinin--induced proliferation was found. By contrast, in carboplatin- cultured MNC from healthy subjects, IL-2 significantly potentiated their toxicity for tumor cells by producing interferon-gamma. It was concluded that IL-2 predominantly supported MNC functional activity rather than inhibited lymphoid MNC apoptosis in in vitro culture with cytostatic drugs.

Plasma level of tumor necrosis factor-alpha (TNF-alpha) correlates with leukocytosis and biological features of leukemic cells, but not treatment response of children with acute lymphoblastic leukemia.

We have analyzed the clinical significance of the plasma level of tumor necrosis-alpha (TNF-alpha) detection in childhood acute lymphoblastic leukemia (ALL) at diagnosis. 49 patients (pts) with B-lineage ALL (3-17 years of age) and 30 healthy children (age-related control) were enrolled in study. The mean value of the level of TNF-alpha was 43.4 +/- 8.1 pg/ml in ALL pts and 30.7 +/- 3 pg/ml in control. In ALL pts the level of TNF-alpha positively correlated with blast cell count in peripheral blood (R = +0.432; P = 0.008); negatively correlated with percentages of S-phase leukemic cells (R = -0.446; P = 0.042) and culture-induced apoptotic cells (R = -0.411; P = 0.057). Pts with TNF-alpha level above the median value were characterized by higher WBC count (P = 0.025), lower percentage of S-phase leukemic cells (P = 0.02) and tendency to lower percentage of apoptotic cells (P = 0.07) vs pts with TNF-alpha level below the median value. No significant association of the level of TNF-alpha with treatment response at days 8 and 15 or 3-year overall survival was defined. We conclude that the elevated plasma level of TNF-alpha is a useful marker to assess disease activity/progression, but not prognosis of childhood B-lineage ALL.