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The article presents a case study of a single-center experience with children undergoing hematopoietic stem cell transplantation (HSCT) and experiencing hemophagocytic lymphohistiocytosis (HLH) as either an indication or sequel to the transplant. Topics include the clinical manifestations and management of primary and secondary HLH, the challenges of diagnosing HLH after HSCT, and the need for improved treatment strategies.

Introduction: patients treated with hematopoietic stem cell transplantation (HSCT) lose immune memory accumulated through a lifetime. They are at increased risk of developing infections with microorganisms such as Haemophilus influenza, Streptococcus pneumoniae and others for which vaccines are available. Therefore, all patients after HSCT should be routinely revaccinated. Systemic reimmunization after HSCT is a relatively neglected area especially in countries which have not national recommendations and there is lack of systemic regulations in health care system., Objective: the rate of immunization before transplantation and the persistence of vaccine-specific antibodies after HSCT was assessed., Study Design: a group of38 children after stem cell transplantation (19 autologous, 19 allogeneic) was studied., Results: only a few patients completed standard vaccination protocol before HSCT. At the median time of 29 (range: 6-67) months after autologous and 13 (range: 8-33) months after allogeneic HSCT, when the revaccination was commenced, the majority of children had concentration of antibody lower than the minimum protective thresholds. That was 82% for tetanus, 71% for Hib and varicella, 46% for HBV and 38% for diphtheria., Conclusions: all HSCT recipients should be routinely revaccinated to stimulate the immunity to the vaccine-preventable diseases.

Currently, granulocyte colony stimulating factor (G-CSF) alone or in combination with myelosuppresive chemotherapy remain the standards of CD34+ cells mobilization allows the safe and successful collection of adequate peripheral blood stem cells (PBSC) for autologous transplantation. However, in up to 30% of patients mobilization of PBSC is ineffective. This report presents our experience in mobilization and collection of peripheral blood stem cells in 82 children with different proliferative disease. In mobilization G-CSF was administered alone in steady state (56 patients, pts) or in combination with myelosuppresive chemotherapy (26 pts). The CD34+ cell count at least 10 cells/ml was required to start apheresis procedure, which was repeated, if needed, during following 1-4 days until collection of at least 2 (optimally 3) x106 CD34+ cells/kg b.w. of recipient was obtained. Three pts in each group (3/ 56 and 3/26) failed the first course of mobilization. The median number of CD34+ cells mobilized was 4.8 (0.5-15) x106/kg b.w. The minimal and optimal number of CD34+ cells for transplantation was achieved in 85% and 61% of patients in the G-CSF + chemotherapy group and in 84% and 54% in the G-CSF group, respectively. The efficacy of presented mobilization arms in our group was similar. However, the incidence of infection and total hospitalization time during mobilization were higher in chemotherapy + G-CSF group.

Total parenteral nutrition (TPN) is still of great importance for haematopoietic stem cell transplantation (HSCT) patients because one of the major adverse effects of the high-dose therapy followed by HSCT is an inadequate oral nutrition intake. The aim of the study was analysis of TPN of young patients in the HSCT period. Twenty-two patients 1.8-20.8 year-old, median 5.4, treated with high-dose therapy and autologous HSCT because of malignancy were included into the study. Grafts contained 1.35-7.9 x 106, median 3.75 x 106 CD34+ cells/kg. Engraftment occurred as follows: granulocytes >0.5 x 109/l on +11 d (8-25); platelets >20 x 109/l on +23 d (12-67). Patients were given isoenergetic, isonitrogenous TPN until they consumed less than 50 % of their required diet orally. Proteins intake was 0.8-2.0 g/kg per d, fats intake 1.0-3.0 g/kg per d. Total non-proteins energies-nitrogen grams index was 140:1-200:1. Supplementation of electrolytes, microelements, trace elements and vitamins was dependent on individual patient requirement. TPN duration did not correlate with CD34+ cells number but correlated with platelets reconstitution. The assessment of nutritional condition demonstrated no differences in anthropometric parameters, but increase of serum albumin levels after TPN. Requirement for P3 - was above the normal ranges and correlated positively with platelets reconstitution. Requirement for P3 - and K+ was higher in patients with mucositis than in other patients. Any complications due to TPN were observed. Adequately composed isoenergetic and isonitrogenous TPN with replacement of electrolytes according to their requirement in the early post-transplantation period allows not only improvement in nutritional status of patients but also could contribute to reconstitution of haematopoiesis.

In departments of neurology, neurosurgery and hospice care there is a group of patients with compete motor function impairment having normal central nervous system function. Victims of spinal cord injury, cerebral palsy, cerebral stroke, loss of extremities, neuromuscular diseases, between others belong to them. Since two decades an intensive studies of use of brain waves to steer peripheral equipments has been performed. Brain Computer Interface and Brain-Machine Interface will allow in the near future for even partial restore of skills in permanently disabled patients. Recently new sets composed of games steered by brain waves have been introduced to the market. Exercises with such equipment will help to control an ability to concentrate and precise steer of the peripheral electronic equipments. The next phase will be use of the new skills to steer the wheelchairs and other computer programs with the brain signals to control own healthy organs or artificial machines.

Unlabelled: The aim of the study was the evaluation of safety and efficacy of vaccination in children after stem cell transplantation., Patients and Methods: 21 patients, 1.4-22 (average 7.8) years old, 13 boys and 8 girls after autologous (11-52%) and allogeneic (10-48%) transplantation were included in the vaccination protocol. Indications for transplantation were: neoplastic disease--16, immunodeficiencies--3 and aplastic anaemia 2 cases. Time between transplantation and beginning of vaccination protocol was 0.8-4 (average 1.5) years. Vaccination protocol was constructed on the basis of the European Group for Blood and Marrow Transplantation indications. We have evaluated: (1) quality of recipient immune reconstitution and protection against common pathogens (2) immunogenicity of revaccination schedule; (3) safety of the vaccination programme., Results: With the exception of one patient presenting with repeated fever, lymph node enlargement, muscle and joint pain, no important side effects were observed. Meningococcial meningitis developed in one patient who refused vaccination. The mean concentrations of antibodies in the plasma before and after vaccination were as follows: anti-diphteria (54; 2285), anti-tetanus (136; 3149) and anti-hepatitis B virus (anti-HBs: 24; 474) IU/ml., Conclusions: (1) Vaccination in patients after transplantation is efficient and well tolerated. (2) Significant increase of antibody level was detected. (3) Any delay in beginning the vaccination can result in life threatening complications.

Infections are one of the most important clinical problem and most frequent cause of interventions among chronically ill children under hospice care. Frequent and long-lasting hospitalizations before admission to the hospice cause patients' colonization with nosocomial pathogens. These pathogens usually cause returning infections, difficult to cure in home care. The aim of the study was evaluation of colonization by multidrug-resistant organisms and infections' frequency in chronically and incurably ill children under care of the Cracow Children's Hospice of Father J. Tischner. We analyzed infections in patients of the Hospice in 2008-2009. Frequency of infections, their localization, pathogens and necessity of hospitalization were evaluated. On the basis of microbiological examination we distinguished infections caused by multidrug resistant pathogens. Ninety microbiological examination were made in 24 children. Urine, stool, pharyngeal and nasal swap and others were examined. Nosocomial pathogens including Gram-negative rods with ESBL phenotype, Gram-positive Enterococci with HLAR phenotype and Staphylococci with MRCNS and MRSA phenotype were isolated in 36 (40%) examinations, in 17 (71%) patients. Frequency of infections was higher in patients colonized by nosocomial pathogens in comparison with patients without colonization, but difference was not statistically important. There are many factors that increase risk of infections and make them difficult to treat, like: immobilization, impaired swallowing and coughing reflexes, thorax deformation, neurogenic bladder, tracheostomy. Multi-drug resistant pathogens are additional risk factor that can lead to the necessity of hospitalization. In chronically and incurably ill patients time of hospitalization should be minimized to reduce the risk of colonization with multi-drug resistant pathogens.

Congenital and acquired neurodegradative diseases are always the reason for prolonged stay in hospital, at the beginning of the establishment of diagnosis and treatment and afterwards for stabilizing all functional adaptation to an existence with the severe disability. Also infections of the lower respiratory tract accompanying the later course of the disease are usually directed to hospital treatment. The aim of the study was to delineate the role of hospice care of patients staying at home, in economical approach to the medical care of severly and incurably ill children. The study group consisted of 29 children with neurodegradative diseases, aged 6 months to 18 years, admitted to the home care of Priest Józef Tischner Cracovian Children' Hospice. The costs of yearly treatment (based on 2008 data) of the infections of the lower airways in the studied group, performed at home under the hospice care and in hospital, were compared. The actual expenses of home treatment were counted. Considering the hospital therapy costs, the simulation was performed following median expenses of a 10-day-treatment of a 20 kg-in-weight child with uncomplicated lower respiratory tract infection in pediatric department with the use of the first line therapy antibiotic. Three parameters were taken to calculations: the medical care costs, the expenses of laboratory tests and X-ray pictures and the costs of antibiotics. In studied children 61 cases of lower respiratory tract infections were diagnosed in 2008 (the median incidence was 2,1 per year; ranged 0-7), of which 48 cases were treated at home. The median time of antibiotics administration in home treatment was 13 days. In 31% of infections more than one antibiotic was used. In 19% of cases in home therapy parenteral medicine was necessary. The median summarized cost of treat- ment at home was calculated as 2657 zl. The need for hospital care in our group concerned 13 incidences. The median estimated cost of treatment of the lower airways infection in hospital for one child equaled 4942 zl. The expenses of home treatment of the lower airways infections under the hospice care were twice lower than the costs of the therapy in hospital. Apart from the obvious psychological and social benefits, also economic aspect contributes to the promotion of the hospice care of staying-at-home patient in the improvement of medical care for children with severe neurodegradative diseases.

Angiogensis plays the crucial role in growth and dissemination of neoplastic diseases, both for solid tumors and hematopoietic malignancies. Development of the abundant neoplastic vasculature results from an imbalance between pro- and antiangiogenic regulatory mechanisms. The investigation was focused on expression of the main regulatory angiogeneic factors in different phases of chronic myeloid leukemia (CML) and on influence of leukemic cells on the human umbilical vein endothelial cells proliferation. The groups of 29 patients with CML and of 14 healthy controls were enrolled to the study. The expression of vascular endothelial growth factor (VEGF), basic fibroblast growth factor, interleukin-8, angiopoetin-1, platelet factor-4, extracellular matrix metalloproteinases (MMP) -2 and -9 as well as tissue inhibitors of metalloproteinases was determined in peripheral blood and bone marrow mononuclear cells with the use of quantitative real-time PCR method and additionally the concentration of VEGF with the flow cytofluorometry. We evaluated the phosphorylation of endothelial mitogen activated protein kinase in human umbilical vein endothelial cells after incubation in CML cells conditioned media, applying Western blot technique. We determined an influence of the leukemic cells on the endothelial cells proliferation with the colorimetric metabolic MTT test. We showed, that in peripheral blood and bone marrow mononuclear cells in CML patients most of the studied factors were increased at the time of CML diagnosis and became lower in remission. In newly diagnosed CML patients an expression of VEGF, MMP-2 and MMP-9 was particularly elevated. In remission, the levels of VEGF and metalloproteinases, specifically MMP-9, were decreased. If failed to achieve remission, the patients presented the elevated expression of most of the investigated angiogenic factors. In the acceleration or blast crisis phase angiopoetin, VEGF and MMP-2 levels were particularly high. We noticed the markedly enhanced human umbilical vein endothelium proliferation after an incubation in CML cells conditioned media, both in the test of mitogen activated protein kinase phosphorylation in endothelial cells and in the metabolic test for the proliferation intensity of endothelium. The differences of an angiogeneic potential found between clinical phases of CML, and the ability of leukemic cells to stimulate endothelial proliferation, point at the significance of neovascularisation in CML pathogenesis. Further studies are necessary to delineate the possibility of the use of angiogenic inhibitors in the treatment of this malignancy.

Hepatic venocclusive disease (VOD) lastly named sinusoidal obstruction syndrome (SOS) is a serious toxicity associated with high dose therapy used to prepare patients for stem cell transplantation. A sizable proportion of patients who develop VOD/SOS die. It is clear that injury to endothelial cells and hepatocytes in zone 3 of the liver acinus is the initial event in the pathogenesis of VOD/SOS. Although clinical presentation and diagnostic criteria are well known, the cause of VOD/SOS, its prevention and treatment remain still unclear. Currently treatment of VOD/SOS has largely consisted of supportive measures designed to maintain intravascular volume and decrease interstitial edema. Other treatments used with various measures of success have included substitution of antitrombine and glutamine or aggressive fibrinolitic and antithrombotic therapy. Despite these treatments, the outcome remains fairly dismal.