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Common variable immune deficiency (CVID) accounts for approximately 20% of all cases of primary immune deficiencies, and is characterized by low serum levels of IgG, IgA, and/or IgM. The diagnosis is usually made between 20 and 40 years of age, sometimes earlier. CVID patients are divided into two major groups based on complications observed 1 group consists of patients with predominant infections, and 2 group includes patients with inflammatory and/or hematological complications, such as lymphadenopathy, splenomegaly, autoimmune cytopenia, enteropathy, and/or granulomatous conditions. The most prevalent gastrointestinal symptom is transitory or persistent diarrhea. Central diabetes insipidus (CDI) is a rare disease associated with decreased synthesis or release of antidiuretic hormone that leads to an excessive production of diluted urine (polyuria). Different factors can lead to the development of CDI, including autoantibodies to arginine vasopressin-producing cells. Celiac disease is an autoimmune condition affecting small intestine in genetically predisposed individuals, which can be associated with endocrinopathies. Here, we describe a patient with CVID, CDI, gluten-sensitive diarrhea, and anemia of combined type (thalassemia minor and B12-deficiency anemia).Donor lymphocyte infusion (DLI) is typically used in 3 clinical situations therapeutically for proven relapse of malignancy, prophylactically in patients with high-risk of relapse, and in case of mixed chimerism. Mixed chimerism, which occur after transplantation can be a sign of possible rejection. In case of increased mixed chimerism, immunotherapy with donor lymphocyte infusions could reverse this process. After DLI, both acute and chronic graft-versus-host disease and marrow aplasia are well-known toxicities. In this paper, we present a case report of young patient with chronic granulomatous disease (CGD) after allogeneic hematopoietic stem cell transplantation (allo-HSCT), with successful immunotherapy following mixed chimerism, which was complicated by bone marrow aplasia that required a second stem cell infusion. DLI seems to be an effective and highly promising treatment method of transplant rejection in patients with CGD but can induce bone marrow aplasia and may require a second stem cell infusion.Recent studies have reported that commensal microorganisms are not just "passive occupants" but may play a crucial role in the immune system activation. DNQX purchase It is well-known that in critically ill patients, the microbiome is modified and may be associated with the development of immunosuppression in sepsis, contributing to the development of acute renal injury, cardiovascular diseases, or more importantly, respiratory system disturbances. The conviction of lung sterility has gone down in history. The presence of characteristic gut microbiome, such as Bacteroidetes and Enterobacteriaceae, was demonstrated in lungs of critically ill patients. This bacteria's translocation, especially in ischemia-reperfusion injury, results in increased concentration of inflammation response markers and may play a pivotal role in the pathogenesis of respiratory system disturbances, including acute respiratory distress syndrome. Recent studies have shown that ischemia-reperfusion injury is often observed in intensive care units (ICUs) and predispose to microbiome disturbances that are strictly connected with immune system activation and epithelial damage. Potential effects of dysbiosis treatment are under highly activated investigation. Therefore, it is possible that microbiota-targeted therapy may constitute the future therapeutic path in ICUs.At present, secondary immune deficiencies have become a clinical problem, recognized in different specialties. The aim of this paper was to increase awareness and support the need for screening at-risk populations. Secondary immune deficiencies result in variety of conditions, but not all of them require immunoglobulin replacement therapy, as specific antibody response might be preserved. Moreover, the management of secondary immune deficiencies vary between countries and different medical disciplines. This literature review presents the most common causes and clinical presentation of secondary immunodeficiencies with predominant impaired antibody production. We present diagnostic guidelines for patients at-risk, with an emphasis on the role of prophylactic vaccination as a treatment and diagnostic tool. This review considers the specificity and disparities of the Polish healthcare system and ultimately, suggests that management teams should include a clinical immunologist experienced in the treatment of humoral immunodeficiencies.Alopecia areata is a condition that affects hair follicles and leads to hair loss ranging from small well-defined patches to complete loss of all body hair. Despite its high incidence, the pathobiology is not fully understood, and no single concept could be universally accepted. Alopecia areata is mostly considered to be an autoimmune disease, in which the collapse of hair follicle immune privilege plays a key role. Higher incidence rate in the female population and increased overall risk of other autoimmune disorders militate in favor of autoimmune hypothesis. Antibodies against multiple components of hair follicles almost exclusively attack in anagen phase, where melanogenesis takes place. It suggests involvement of melanogenesis-associated autoantigens as a target epitope. Some investigators believed that alopecia areata is not a truly autoimmune disease but is only 'consistent with' autoimmune mechanisms. High frequency of a positive family history up to 42% may reflects the contribution of heredity factors. In addition, no specific target autoantigen has been identified so far, and autoantibodies to hair follicle-associated antigens are detectable in normal individuals.Natural killer (NK) frequency and NK cytotoxicity (NKc) are key determining factors of a clinical outcome. In our previous study, we showed the prognostic clinical significance of immune parameters when they are beyond the optimal range (accentuated). In this study, we attempted to explain the disparity of accentuated but physiologically and immunologically normal NK parameters that might serve as negative clinical prognostics indications of failed pregnancies. We have analyzed NK%, NKc levels, and their reciprocal correlation in 2,804 patients with reproductive failures. In the entire clinical population, NK% correlates with NKc. Interestingly, we found this relationship to be strongly dependent on NK level's status. NK%-NKc correlation was the strongest (r = 0.2021, p 17.5%). Patients with NK% between 15-17.5% manifested lower but still significant correlation NK%-NKc (r = 0.1213, p = 0.0155). Additionally, significant correlation (r = 0.2689, p less then less then 0.0001) between NK% and NKc was observed in a group of patients with NK levels less then 7% (1.7-7%). While patients' groups with NK% (7-15%) did not reveal NK%-NKc association. This led us to hypothesize that the qualitative-quantitative status of NK population is responsible for their cytotoxic activity. Consistent with our hypothesis, the "balanced zone" NK% is tightly controlled, and thus does not correlate directly with NKc. In contrast, the "accentuated zones" of NK% escape this control and directly affecting NKc. Demonstrated phenomena supports our idea about the clinical significance of immune accentuation and explains its novel physiological role.

Surgical intervention affects local and systemic immune responses, especially in obese individuals. Many studies have attempted to evaluate immunological response to surgical trauma. Surgery changes the quantity and phenotype of circulating blood dendritic cells (DCs), including a decrease of total DCs post-operatively. The study aimed to evaluate the percentage and changes of myeloid, lymphoid DCs, and myeloid to lymphoid DCs ratio in obese and normal weight patients undergoing laparoscopy.

The study enrolled asymptomatic patients with gallstones, who underwent laparoscopic cholecystectomy. Blood samples were obtained before the surgery as well as 24 and 48 hours after the surgery. Cells were collected using a FACSCalibur flow cytometry, and phenotypes were analyzed with CellQuest software.

No statistically significant differences were observed between obese and normal-weighted patients in all studied time periods, except for the myeloid to lymphoid DCs ratio assessed at 48-post-operative hour. The myeloid DCs percentage increased significantly in the post-operative period within both studied groups. The percentage of lymphoid DCs increased significantly in obese patients in all studied time periods.

Laparoscopy induces immunomodulation, such as changes of myeloid and lymphoid dendritic cells, especially in obese patients. link2 We describe new findings, in which minimally invasive surgical trauma promotes the increase of percentage of circulating DCs in the early post-operative period.

Laparoscopy induces immunomodulation, such as changes of myeloid and lymphoid dendritic cells, especially in obese patients. We describe new findings, in which minimally invasive surgical trauma promotes the increase of percentage of circulating DCs in the early post-operative period.Hereditary angioedema due to C1-inhibitor deficiency (C1-INH-HAE) type I and II is a rare and life-threatening disease caused by SERPING1 gene mutations. Previous genetic studies indicated a wide spectrum of disease-associated variants in the SERPING1 gene and often lack of correlation with patient's phenotypes. The aim of this study was to evaluate the presence, type, and localization of mutations in the SERPING1 gene in 41 Polish patients with C1-INH-HAE and their relation with case/family history, type of C1-INH-HAE, fC1-INH, age of onset, and disease severity. Sanger sequencing and MLPA method were used for detection of disease-associated variants. link3 In 34 (82.9%) patients, mutations located in various regions of SERPING1 gene were revealed. The detected alterations in patients with C1-INH-HAE type I differed and were positioned in various exons/introns of the SERPING1 gene. The most frequent disease-associated variants appeared in exon 3 (especially in type I) and in exon 8 (type I and II). Out of 20 different disease-causing variants, 9 were not previously described. We did not find any relation between the type and location of the mutations and no type of features included in phenotype evaluation of the patients, such as case and family history, type of C1-INH-HAE, age of onset, biochemical parameters, or severity of disease.

To assess the level of acidic mammalian chitinase (AMCase) expression and IL-8 in nasal inferior turbinate mucosa in patients with mild and moderate to severe allergic rhinitis (AR).

Participants in this case-control study were divided into three groups, including patients with moderate and severe persistent allergic rhinitis, cases with mild forms of persistent AR, and control or healthy group. We obtained biopsies of nasal inferior turbinate mucosa from all participants. Expression of AMCase and IL-8 mRNAs were evaluated by real-time polymerase chain reaction (PCR). The serum levels of AMCase and IL-8 were determined by ELISA. The number of eosinophils per field, blood eosinophils, total serum IgE levels, and specific serum IgE levels were measured. Patients' clinical manifestations were assessed by total nasal syndrome score (TNSS).

Expression of AMCase and IL-8 in patients with moderate and severe perineal allergic rhinitis were significantly elevated compared to the control group and patients with mild persistent allergic rhinitis.