Molecular Genetics Diversity of Primary Hemophagocytic Lymphohistiocytosis among Polish Pediatric Patients.

Hemophagocytic lymphohistiocytosis (HLH) is a clinical syndrome of life-threatening inflammation caused by an excessive, prolonged and ineffective immune response. An increasing number of HLH cases is recognized in Poland, but the genetic causes of familial HLH (FHL) have not been reported. We investigated the molecular genetics and associated outcomes of pediatric patients who met HLH criteria.

Results of two consecutive treatment protocols in Polish children with acute lymphoblastic leukemia.

The aim of the study was to retrospectively compare the effectiveness of the ALL IC-BFM 2002 and ALL IC-BFM 2009 protocols and the distribution of risk groups by the two protocols after minimal residual disease (MRD) measurement as well as its impact on survival. We reviewed the medical records of 3248 patients aged 1-18 years with newly diagnosed ALL who were treated in 14 hemato-oncological centers between 2002 and 2018 in Poland. The overall survival (OS) of 1872 children with ALL treated with the ALL IC 2002 protocol was 84% after 3 years, whereas the OS of 1376 children with ALL treated with the ALL IC 2009 protocol was 87% (P < 0.

Clinical characteristics and outcome of pediatric patients diagnosed with Langerhans cell histiocytosis in pediatric hematology and oncology centers in Poland.

Background: Langerhans cell histiocytosis (LCH) affects 1-2 in 1,000,000 people. The disease is not associated with increased risk of treatment failure (especially among older children), but appropriate procedures implemented in advance can eliminate complications which might appear and significantly worsen the patients' quality of life. Thus, we sought to evaluate the clinical features, management, and outcome of children with LCH treated in Polish pediatric hematology-oncology centers.

The Treatment of Opsoclonus-Myoclonus Syndrome Secondary to Neuroblastic Tumours-Single-Centre Experience and Literature Review.

: The opsoclonus-myoclonus syndrome (OMS) is characterised by opsoclons, myoclons and impaired balance, often concomitant with sleep disorder and behavioural difficulties. The symptoms develop as a result of autoimmune response triggered by a neuroblastic tumour (NT). OMS can also develop secondarily to a viral infection or as an immune response triggered by an unknown agent.

Prevalence, Epidemiology, Etiology, and Sensitivity of Invasive Bacterial Infections in Pediatric Patients Undergoing Oncological Treatment: A Multicenter Nationwide Study.

Infectious complications (IC) caused by bacterial strains often impede anticancer therapy. The study aimed to retrospectively analyze bacterial IC that could help predict the risk and optimize the empirical treatment for bacterial infections in pediatric cancer patients. Over a 72-month period, all-in 5,599 children with cancer: 2,441 patients with hematological malignancy (HM including acute leukemias, Hodgkin and non-Hodgkin lymphomas [NHLs], and Langerhans cell histiocytosis) and 3,158 with solid tumors (STs including central nervous system tumors, neuroblastoma, Wilms' tumor, soft tissue sarcoma, germ cell tumors, Ewing sarcoma, osteosarcoma, hepatoblastoma, and others) were enrolled into the study.

Infections in children with acute myeloid leukemia: increased mortality in relapsed/refractory patients.

The aim of this nationwide study was to describe the epidemiology and profile of bacterial infections (BI), invasive fungal disease (IFD) and viral infections (VI) in patients with and relapsed/refractory (rel/ref) acute myeloid leukemia (AML). Within the studied group of 250 children with primary AML, at least one infectious complication (IC) was diagnosed in 76.0% ( = 190) children including 85.

Quality of life in survivors of childhood brain tumour and the association of children's diseases on quality of their parents life.

Objective: Evaluation of children's quality of life (QoL) after finished brain tumour treatment and the association of children's diseases on quality of their parents' life.

Methods: The study group was consisted of 46 children after brain tumour treatment (aged 4, 5, to 29 years old). The control group was composed of 104 students of primary, secondary, and high schools.

NK Cells as Possible Prognostic Factor in Childhood Acute Lymphoblastic Leukemia.

Deficiency or impaired function natural killer (NK) cells might result in the development of serious infections and promote the development of malignancies. The aim of our study was to assess the prognostic role of NK cell percentage in bone marrow on the day of acute lymphoblastic leukemia (ALL) diagnosis. 84 children (49 males = 58%; median age 5 yrs) with ALL were enrolled.

Surface expression of Cytokine Receptor-Like Factor 2 increases risk of relapse in pediatric acute lymphoblastic leukemia patients harboring deletions.

We prospectively examined whether surface expression of Cytokine Receptor-Like Factor 2 (CRLF2) on leukemic blasts is associated with survival and induction treatment response in pediatric B-cell precursor acute lymphoblastic leukemia (BCP-ALL) patients. Flow cytometric analysis of bone marrow-derived leukemia cells revealed that 7.51% (29/286) of 386 pediatric BCP-ALL patients were CRLF2-positive (CRLF2pos) at diagnosis.

[Difficulties of the therapy in a boy with coexisting type 1 diabetes mellitus and idiopathic thrombocytopenic purpura].

Idiopathic thrombocytopenic purpura (ITP) is an acquired autoimmune disease, caused by antibodies against platelet glycoproteins, which provoke platelet destruction and inhibit platelet production in bone marrow. Type 1 Diabetes Mellitus (T1DM) is an acquired autoimmune disease in witch beta cells are destroyed by autoantibodies. Patient with T1DM since the age of 6, was treated by intensive functional insulin therapy by insulin pump.

Assessment of Angiogenesis in Children with Acute Lymphoblastic Leukemia Based on Serum Vascular Endothelial Growth Factor Assay.

Introduction: Vascular endothelial growth factor A (VEGFA) is a key proangiogenic cytokine. The role of angiogenesis in acute lymphoblastic leukemia (ALL) is still unclear. The purpose of the study was to assess angiogenesis in children with ALL based on serum VEGFA level determined at diagnosis and at remission with further participant subdivision into different risk groups.

PRES in the course of hemato-oncological treatment in children.

Introduction: Posterior reversible leukoencephalopathy syndrome (PRES) is a clinical syndrome of varying aetiologies, characterised by acute neurological symptoms of brain dysfunction with MRI abnormalities in posterior cerebral white and grey matter. In most cases, symptoms resolve without neurological consequences.

Aim: The aim of this paper is the analysis of predisposing factors, clinical outcomes and radiological features of PRES in eight children with hemato-oncological disease.

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

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).

Serum levels of vascular endothelial growth factor and basic fibroblast growth factor in children with brain tumors.

Background: Angiogenesis is the process of new vessel formation originating from the existing vascular network. It plays an important role in the growth and spread of malignancies, including brain tumors. The process of angiogenesis is characterized by increased expression of vascular endothelial growth factor (VEGF) and basic fibroblast growth factor (bFGF), and by the release of their soluble forms into circulation.

Blastic Plasmocytoid Dendritic Cell Neoplasm With Skin and Myeloid Location.

Blastic plasmocytoid dendritic cell neoplasm is one of the aggressive hematopoietic malignancy with predilection for the skin. It is a tumor derived from the plasmocytoid dendritic precursor cells. Skin is its most common location; however, it may also affect bone marrow, lymph nodes, or spleen.

Inflammatory Myofibroblastic Tumor of the Heart in the Infant: Review of the Literature.

Primary heart tumors are extremely rare, constituting approximately 0.02% of all malignancies. Inflammatory myofibroblastic tumor (IMT) constitutes <5% of primary heart tumors.

Renal function in children treated for central nervous system malignancies.

Aim: The aim of the study was to evaluate renal function and to assess the usefulness of the following nephrotoxicity markers: cystatin C (CYS C), beta-2 microglobulin (B2MG) and neutrophil gelatinase-associated lipocalin (NGAL) in 38 (18 girls, 20 boys) children previously treated for central nervous system malignancy.

Material: Median age at evaluation was 13.7 years (range 2.

Expression of myeloid antigens on lymphoblast surface in childhood acute lymphoblastic leukemia at diagnosis and its effect on early response to treatment: a preliminary report.

Immunodiagnosis of acute lymphoblastic leukemia (ALL) is based on the assessment of surface antigens. There are also cases in which both lymphoid and myeloid antigens can be found on the surface of lymphoblasts. The purpose of our research was to assess the expression of myeloid and lymphoblastic antigens in children with ALL, and to determine the impact of surface antigens on early response to treatment.