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C2 deficiency represents the most frequent type of a complement deficiency. Clinical manifestation includes infections caused by encapsulated bacteria (Steptococcus pneumoniae, Neisseria meningitidis) such as meningitis, gonitis, pneumonia or septicaemia. A causative treatment has not been available yet. A prophylactic vaccination and/or a long-term antibiotics prophylaxis are recommended. Here we report 2 patients from 2 unrelated families. The first patient suffered from recurrent otitis in his childhood. He underwent osteomyelitis, meningitis complicates with hear-loss, and one episode of pneumonia during adulthood. The second index patient underwent uncomplicated meningitis in his preschool age. He has been treated for recurrent upper-airways infections later. His sister has been completely asymptomatic. The deletion 28 bp (c.841-849+19del28) in C2-gene was detected in all of them in homozygous form. Our paper highlights the variability of a clinical manifestation in homozygous carriers, ranged from asymptomatic cases to patients with history of severe complications. The diagnosis is frequently made even in adulthood.

Immune system is the integral part of the complex body response, inflammation, which is raised either by the exposure to external signals, predominantly pathogens or by damage of own structures. Predominantly innate immunity is equiped by the receptors recognizing pathogenic PAMPs or signals of own damage DAMPs. The inflammatory response is reflecting the actual demand of our body. The potential of the inflammatory response is so powerful that its intensity and extent have to be carefully regulated on many levels.

Vitamin C is a water soluble micronutrient commonly found in our diet which orchestrates the function of both innate and adaptive immune system, influencing both cellular and humoral immune responses. Vitamin C inhibits excessive activation of the immune system to prevent tissue damage, but also supports antibacterial activity, stimulates NK cells and differentiation of Th0 subset into Th1 characterized by interferon γ production. In addition, vitamin C interferes with the synthesis of proinflammatory cytokines, or with the expression of adhesive molecules. Moreover, vitamin C as an antioxidat protects the immune cells against intracellular ROS (reactive oxygen species) formed in the inflammatory response. Vitamin C as an enzymatic cofactor is extremely important in maintaining tissue integrity, and plays a crucial role in formation of skin, epithelial and endothelial barriers.

Diseases of salivary glands may be associated with salivary flow rate disturbances. Production of the saliva is evaluated by sialometric tests. The stress is putted on salivary flow rate disturbances in Sjögren's syndrome, drug-induced and postirradiative sialopathy, and diabetes mellitus. The possibility of the stimulation and substitution of the saliva is discussed.

Protective immune response in urinary tract is frequently impaired in patients with diabetes. Immunity in this mucosal compartment displays unique characteristics; e.g. absence of physiological microflora and lack of mucus. Pathogens are identified by the PRR receptors expressed on both epithelial and immune cells. Inflammatory response characterised by the acumulation ofgranulocytes is followed. Both protective and harm characteristics of inflammatory response are inseparable linked and delineated by gene polymorphisms in PRR receptors.

Chronic lymphocytic leukemia (CLL) is characterized by extraordinary heterogeneity in terms of clinical course with overall survival ranging from several months to dozens of years. It is currently not possible to accurately predict the future clinical course in an individual patient. Angiogenesis has been recently reported as a potential prognostic factor in various hematological malignancies including CLL. The objective of the present study was to quantify plasma levels of key angiogenic activators vascular endothelial growth factor (VEGF) and basic fibroblast growth factor (bFGF) in patients with CLL and determine their potential change after intensive fludarabine-based treatment. Peripheral blood EDTA plasma concentrations of bFGF and VEGF were measured using comercially available enzyme-linked immunosorbent assay in 73 patients with untreated CLL (43 males, 30 females, median age, 65 years, range 31-88) and 80 healthy donors serving as control group. We found statistically significant increase in concentrations in patients with chronic lymphocytic leukemia compared to the control group (p < 0.0001 for both cytokines). No differences in angiogenic factors were noted between subgroups with low vs. intermediate vs. high-risk stage according to modified Rai staging or males vs. females. In twelve patients who achieved at least partial response after intensive fludarabine-based treatment, levels of bFGF as well as VEGF decreased significantly (bFGF, p = 0.0005; VEGF, p = 0.0068); in addition, they were no more significantly different from controls (bFGF, p = 0.524; VEGF, p = 0.728). Our data showed that key angiogenic activators bFGF and VEGF were elevated in plasma ofCLL patients. Furthemore, treatment with intensive fludarabine-containing regimens resulted in significant decrease of both cytokines. These data suggest that angiogenic cytokines may indeed play a significant role in CLL biology and that treatment with combination of fludarabine, cyclophosphamide +/- rituximab may exhibit antiangiogenic properties. Further studies with longer follow-up are necessary for evaluation of a possible association between angiogenic markers and progression-free survival or overall survival.

The pathogenesis of diabetes mellitus I is multifactorial. Both genetic predisposition and environmental factors are involved in the deregulation of immune system with subsequent breakage of self-tolerance. Autoantibodies against numerous autoantigens are raised during this long-term process but the principal pathogenic mechanism is cytotoxic killing of beta cells of pancreas mediated predominantly by T cells. There could be the chance to interrupt this pathogenetic process by manipulation with immune system to achieve induction of tolerance. Such clinical trials are now under progress.

The immunophenotyping of the lymphoproliferative diseases is considered to be an integral part of the diagnostic process pointing to the correct classification of the individual diseases and to the choice of the most suitable type of therapy. This article describes the immunophenotype of particular types of B-lymphoproliferative disorders included in the World Health Organization classification (WHO) and the prognostic importance of some markers. The panel of specific monoclonal antibodies directed to B-cell lineage used in our department is displayed.

The authors present normal values of selected constitutive and activating platelet surface antigens expression. The results were obtained by flow cytometry analysis after immunofluorescent staining of the peripheral whole blood specimens from 25 healthy adults. Data are presented as a mean value and standard deviation (SD) of percentage of positive labelled platelets: constitutively expressed molecules CD9: 94.97% (+/- 2.95), CD31: 92.78% (+/- 2.97), CD36: 90.98% (+/- 5.25), CD41: 95.62% (+/- 2.23), CD42a: 94.98% (+/- 2.62), CD42b: 94.13% (+/- 2.58), activation molecules CD62P: 0.45% (+/- 0.33), CD63: 0.32% (+/- 0.22). This study is the first report in Czech literature.

Multiple myeloma is one of the most common haematologic malignancies. Currently there are numerous studies looking for new prognostic markers in multiple myeloma. The most important of them are the markers related to proliferative activity of neoplastic cells or to size of tumor mass. The subject of this paper are the results obtained from investigation of some such laboratory markers in a group of patients with monoclonal gammopathies diagnosed at our department in the last 3 years. We analyzed blood and bone marrow samples from 51 patients with new diagnosed monoclonal gammopathies, 14 of them were patients with monoclonal gammopathy of undetermined significance and 37 patients had multiple myeloma. 17 patients with multiple myeloma were treated by high-dose chemotherapy regimen. We assessed significance of selected laboratory markers for differential diagnosis of monoclonal gammopathies and for monitoring of activity of multiple myeloma. Among the investigated parameters, we verified the significance of cell cycle analysis of bone marrow plasmatic population and of the determination of the number of circulating myeloma cells in differential diagnosis of monoclonal gammopathies. In our opinion, the determination of soluble CD138, beta 2-microglobulin and neopterin serum levels can be also recommended as helpful markers for a solution of this problem. Except of beta 2-microglobulin serum level we did not find statistically significant correlation with activity of multiple myeloma in any of the investigated parameters.

Apoptosis is a one example of genetically-regulated cell death. It is characterized by the phylogenetically highly conserved activation of specific genes and proteins followed by energy-consuming proteolytic autodestruction. Apoptosis seems to be physiological and highly selective mechanism to eliminate either old or injured cells in organisms. It is in contrary to mitosis. A highly complex combination of various stimuli both extracellular and intracellular origins is required for induction of apoptosis together with preserved oxidative phosphorylation.

Study of apoptosis seems to be relevant to clinical medicine nowadays. Dysregulation of apoptosis is under the certain circumstances the cause of various pathological processes in organism. The numerous methods to analyse apoptosis are now well established. It is very likely that our better understanding of apoptosis could lead to improvement in diagnosis and treatment of diseases such as immunopathology, cancer, and others.

Blood platelet activation is a complex process involved in the physiological hemostasis and also in a number of disorders. Platelets change their morphology, modify their surfaces glycoprotein receptors with adhesive functions. Procoagulant microparticles are shedding and the aggregates of platelets with leucocytes are appearing. The activated platelet can be detected by flow cytometry after imunofluorescent staining.

The functional fluorescent dye efflux assays are used in the study of the multidrug resistance of the malignant cells to the cytotoxic drugs which is caused by the overexpression of P-glycoprotein (P-gp) or another membrane transport system--a multidrug resistance related protein (MRP). P-glycoprotein and a multidrug resistance related protein are involved in the efflux of cytotoxic drugs out of the cell and are responsible for the resistance. Fluorescent dye efflux mediated by these proteins could be evaluated by the flow cytometry. This test seems to be an optimal approach to study the multidrug resistance. With help of the specific inhibitors such as verapamil and cyclosporine A, the functional capacity of these proteins and the possibility to overcome the multiresistant phenotype can be revealed. The cell line K562 with transfected P-glycoprotein gene serves as a model system for the studying of the transport function with the use of the fluorescent substrates and P-glycoprotein inhibitors.

The advent of new therapeutic approaches to multiple myeloma made necessary the introduction of novel methods for detection of minimal residual disease. Among others approaches residual disease can be detected by the immunofluorescence using flow cytometry. We have examined the co-expression of CD19, CD38, CD45, CD54, CD56, and CD138 molecules in cells of peripheral blood and bone marrow aspirates in patients with multiple myeloma by 3-color flow cytometry. For the detection and characterization of multiple myeloma cells, combinations of following antibodies were used: anti-CD19 FITC, anti-CD38 FITC, anti-CD38 PE, anti-CD54 FITC, anti-CD56 PE-Cy5, anti-CD45 PE, anti-CD45 PE-Cy5 (Immunotech) and anti CD138 PE (Serotec). The samples were analyzed using EPICS XL (Coulter) flow cytometer, and the analysis was based on at least 10,000 events. Samples from 17 patients were analyzed. The percentage of multiple myeloma cells ranged between 0.3% and 54.2% in bone marrow aspirates and between 0.0 and 11.8% in periferal blood. The expression of CD138, CD38, CD54 and CD56 molecules was found in 100%, 100%, 85% and 68% of examined cases, respectively. In our opinion, multiple myeloma cells are best characterized by following combinations of antibodies: CD38 FITC/CD138 PE/CD45 PE-Cy5, CD54 FITC/CD138 PE/CD56 PE-Cy5 or CD54 FITC/CD38 PE/CD 56 PE-Cy5. The identification of a malignant clone is the first and the most important step in the characterization of the disease, determination of its prognosis and the detection of residual disease after treatment. Three-color flow cytometry represents a method which can meet these goals.

Interactions between endothelial cells, adherence of endothelial cells to the molecules of extracellular matrix, adhesion of leukocytes and platelets to the activated endothelial cell lining are mediated by adhesion molecules belonging into four families: immunoglobulins, integrins, selectins, and cadherins. Adhesion interactions mediate intercellular signaling. Soluble forms of adhesion molecules modulate adhesion interactions. The determination of soluble adhesion molecules in blood is used for indirect evaluation of function or dysfunction of endothelial cells. It is possible to modulate abnormal function of endothelial cells using monoclonal antibodies against adhesion molecules.

Pathogenicity of Helicobacter pylori is based partially on the production of toxic substances. On the other hand the gastric mucosal injury may be caused by the Helicobacter pylori-induced host inflammatory response. Considerable antibody response during Helicobacter pylori infection provides a ground for indirect non-invasive serological detection. Appropriate clinical applications of serological tests are based on a good understanding of the antibody response characteristics during Helicobacter pylori infection.

The paper deals with the types of antigens for detection of anti-Helicobacter pylori antibodies. There are informations about the protein composition of Helicobacter pylori and about the specificity and immunogenicity of particular proteins. The authors review and evaluate different types of antigenic preparations used in serological tests and inform about the commercial kits for serological detection of Helicobacter pylori infection.

The authors made immunophenotyping analyses of blood cells of 50 patients with the diagnosis of B-CLL. Malignant cells contained in all instances HLA DR and CD24 molecules. The superficial molecule CD5 was expressed on cells in 85.7% molecule CD6 on 66.7% cases. By statistical analysis the relationship between selected clinical parameters and expression of surface molecules CD5, CD6 and CD10 was sought. A statistically significantly higher number of leucocytes was found in patients with expression of molecule CD6.

Contemporary therapeutic procedures used in the treatment of malignant diseases are based on the no-selective action of cytotoxic or cytostatic substances. The sensitivity of tumour cells to cytostatics can be, however, reduced by the action of several mechanisms among which expression of the membrane glycoprotein P-170 is the most important. The latter actively transfers the cytotoxic substance from the cell and is responsible for multiple drug resistance-MDR). Glycoprotein P-170 can be assessed on leukaemic cells immunochemically by flow cytometry. A very perspective procedure is assessment of the in vitro sensitivity of leukaemic cells to clinically used cytostatics.

Using immunocytochemical phenotyping, the authors assessed the origin of malignant cells in a patient with malignant centroblastic lymphoma. The lymphoma cells expressed the thymocytic surface molecule CD1, pan T-lymphocytic markers CD2, CD5, CD6, CD7 with concurrent absence of complex CD3 and receptors for antigen on T lymphocytes. The proliferating activity of malignant cells could not be controlled by intensive cytostatic therapy.

The authors present the results of immunophenotyping analysis of cells from 67 patients with the diagnosis of B-CLL from group of 393 adults examined by immunophenotyping because of a haematological malignity. Characteristic for B-CLL is the finding of surface molecules HLA DR, B lymphocytic superficial molecules and CD5. Aberrant membrane equipment of B-CLL cells is suggested by the rare absence of some pan B lymphocytic sign. Marked reduction of T lymphocyte ratios, their sub-populations and NK cells is associated with immunodeficiency manifestations which are common in patients with B-CLL.

The authors describe a case of myeloid leukemia of the myelomonocytic type (M4, FAB 1976) where blastic cells bear the lymphoblastic cALL sign. These cases of the so-called hybrid phenotype of acute leukemia are rare. The importance of the hybrid phenotype for the course and prognosis of the disease is not known.

Crohn's disease is a disease with unknown etiology of which immunological mechanisms participate. As standard treatment is sometimes ineffective, in 15 patients therapeutic plasmapheresis was used. Before plasmapheresis and immediately after it some immunological parameters were assessed. In the serum a significant drop of all immunoglobulins of the basic classes was recorded: (P less than 0.05) and of circulating immunocomplexes (P less than 0.02). Marked clinical improvement was observed in three patients, which as compared with data in the literature, is somewhat less. The cause is in the author's opinion in the selection of patients (plasmapheresis is usually most successful in patients with marked activity of the disease and non-intestinal symptoms) even in case of less active plasma exchange. Despite this plasmapheresis can be recommended in patients with a poor response to common conservative treatment and in some of them we may expect a marked therapeutic effect with a favourable influence on the prognosis of the disease.

The prerequisite of effective infection with the virus of human immunodeficiency (HIV) are the interaction between the CD4 complex of the susceptible cell and outer glycoproteins of the virus. The CD4 complex is found above all on the sub-population of helper inducer T lymphocytes and cells of the monocyto-macrophage line. These interactions are essential for the penetration of the virus into the cell and participate also in the fusion of infected and non-infected cells which is typical for HIV infection. The outer layer glycoproteins of HIV are immunogenic, however, the antibodies which are formed do not neutralize the virus. It is even probable that antibodies can under certain conditions facilitate the penetration of HIV into cells which have on their surface receptors for the Fc fragment of immunoglobulin, i.e. in the first place into cells of the monocyte-macrophage line. These cells play obviously a key role in HIV infection because they are resistant to the cytopathic action of the virus and during their numerous physiological functions serve above all as cells, presenting antigen to T lymphocytes and thus are repeatedly in close contact with other cellular elements of the immune system and may thus help to spread HIV in the organism.

The authors evaluate clinical experience based on a correlation between the clinical condition and the finding of Campylobacter pylori (CP) in a total of 1027 specimens of the gastric mucosa. They confirmed the high incidence of CP in patients with a fresh duodenal ulcer (g 7%) but also in the inactive stage of duodenal ulcer disease (97.8%) and in patients with a pyloric ulcer (94.4%). An analogous incidence of CP in patients with so-called isolated duodenitis (in the course of the disease no ulcer lesion was ever detected), i.e. in 95.5%, indicates the relationship of this condition with peptic ulcer disease. The authors indicate diagnostic possibilities as regards CP infection by cultivation, rapid urease tests and microscopic examination and they emphasize the practical value of urease tests. Based on their own experience the authors confirm the high eradication effect of bismuth preparations, in particular bismuth subsalicylate and colloid bismuth subcitrate.