Hungarian Immunology

[Immunological aspects in gastroenterology]

BENE László és munkatársai

OCTOBER 10, 2005

Hungarian Immunology - 2005;4(03-04)

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Hungarian Immunology

[Does practice make a master?]

SZEGEDI Gyula

Hungarian Immunology

[Plasmacytoid dendritic cells - type I interferon producing cells]

MAGYARICS Zoltán, RAJNAVÖLGYI Éva

[Dendritic cells represent a multifunctional cell population classified to myeloid (mDC) and plasmacytoid (pDC) types. Both subsets circulate in the peripheral blood and are found in lymphoid and also in non-lymphoid tissues, where they act as sensors of environmental changes. Upon activation by a wide range of stimuli they undergo morphological and functional transition and give rise to professional antigen presenting cells, which migrate to lymphoid organs. A newly identified precursor subset of human dendritic cells has recently been identified as professional type I interferon producing cells (IPC) with multiple functional activities. With their capacity of priming, instructing and regulating various pathogen- and tumor-specific immune responses, IPC/pDC act as a link between innate and adaptive immunity. The role of pDC in the pathogenesis of various diseases is well established, and these cells also emerge as novel candidates of immunomodulation.]

Hungarian Immunology

[Transmission of antibodies from mother to offspring: evolutionary aspects]

BAINTNER Károly

[The earliest known form of transmission of antibody is the transport from the maternal circulation into the yolk during vitellogenesis (in birds and reptiles), followed by endodermal uptake and transport into the embryonal circulation. During the early mammalian evolution lacteal secretion and the development of the placenta opened new ways to feed the young. These changes also resulted in alterations in sites and mechanisms of transmission of immunoglobulins. In a few species (e.g. rabbit and rodents) the yolk-less yolk sac gained a new function, i.e. the absorption of uterine secretion. In most of the mammalian species the neonatal type Fc-receptor (FcRn) plays a key role in the transmission and confers IgG-selectivity on the process. In ungulates undigested colostral proteins, including antibodies, are absorbed non-selectively by the gut, mediated by sizable transport vacuoles. The limited postnatal transmission period (24 to 48 h) is compensated by the considerable length of the small intestine and the efficiency of absorption. In the human chorioallantoic placenta the two steps of transmission (maternal secretion and absorption by the offspring) were reduced to a single step. Absorption of IgG is often carried out in a proteolytic environment (yolk sac, gut lumen, intestinal vacuoles), and as a result, different mechanisms evolved for the protection of antibody.]

Hungarian Immunology

[Regulatory T cells in mixed connective tissue disease]

BARÁTH Sándor, ALEKSZA Magdolna, SZEGEDI Andrea, SIPKA Sándor, SZEGEDI Gyula, BODOLAY Edit

[INTRODUCTION - CD4+/CD25+high suppressor and IL-10 producing CD4+ regulatory T (IL-10 Treg) cells were investigated in the peripheral blood of 48 patients with mixed connective tissue disease (MCTD). Seventeen patients were in active and 31 patients in inactive state. PATIENTS AND METHODS - Measurement of the number of CD4+CD25+high suppressor and IL-10 Treg cells was carried out by flow cytometry. RESULTS - The absolute number and percent of CD4+CD25+high T cells decreased in MCTD patients compared to the healthy controls. The number of CD4+CD25+high Treg cells was lower in 17 active MCTD patients than in the inactive patients. The percent and absolute number of IL-10 Treg was elevated in the peripheral blood of patients with MCTD compared to the healthy controls. Corticosteroid and immunosuppressive drugs moved the number of regulatory T cells (CD4+CD25+high and IL-10 Treg cells) towards the normal value. CONCLUSIONS - Our results show that the decrease in the number of CD4+CD25+high T cells could play a key role in the immunoregulatory disturbance in MCTD. Elevation in the number of IL-10 Treg cells might be a compensatory mechanism to retain the balance of proinflammatory and anti-inflammatory cytokines.]

Hungarian Immunology

[Extensive flow cytometric characterization of plasmocytoid dendritic cell leukemia cells]

GOPCSA László, KORMOS Luca, BÁNYAI Anikó, TAMÁSKA Júlia, MATOLCSY András, GOGOLÁK Péter, RAJNAVÖLGYI Éva, PÁLÓCZI Katalin

[INTRODUCTION - Accumulating evidences suggest that non-T, non-B cell CD4+/CD56+ neoplasms with lymphoblastic morphology include clinically and immunophenotypically diverse entities. Although their cells of origin or classification are still controversial several entities clearly represent a distinct type of neoplasms that are clinically aggressive. CASE REPORT - In this work we present the immunophenotypic and genotypic features of bone marrow, peripheral blood, lymph node and skin lymphocytes from a patient diagnosed as plasmacytoid dendritic cell leukemia involving the skin, bone marrow, peripheral blood, lymph nodes, liver and spleen. For determination of immunophenotypic characteristics of malignant plasmacytoid dendritic cells 73 monoclonal antibodies detecting lineage markers, chemokine receptors, cytokine receptors, activation and co-stimulatory molecules were used. The malignant cells proved to express CD4+, CD56+ lineage negative leukemia phenotype characteristically positive for CD36, CD38, CD40, CD45, CD45RA, CD68, CD123, CD184, HLA-DR, BDCA2 and granzyme-B corresponding to the preplasmacitoid dendritic cell developmental stage. CONCLUSION - The presence of CD11a/CD18, CD84, CD91, CD95, αvβ5, CDw197 and the absence of CD52 and CD133 in this case can be regarded as additional features of malignant cells.]

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[In recent decades it has become increasingly important to involve patients in their diagnostic and treatment process to improve treatment outcomes and optimize compliance. By their involvement, patients can become active participants in therapeutic developments and their observations can be utilized in determining the unmet needs and priorities in clinical research. This is especially true in rare diseases such as Pompe disease. Pompe disease is a genetically determined lysosomal storage disease featuring severe limb-girdle and axial muscle weakness accompanied with respiratory insufficiency, in which enzyme replacement therapy (ERT) now has been available for 15 years. In our present study, patient reported outcome measures (PROMs) for individuals affected with Pompe disease were developed which included questionnaires assessing general quality of life (EuroQoL, EQ-5D, SF36), daily activities and motor performance (Fatigue Severity Score, R-PAct-Scale, Rotterdam and Bartel disability scale). Data were collected for three subsequent years. The PROM questionnaires were a good complement to the physician-recorded condition assessment, and on certain aspects only PROMs provided information (e.g. fatigue in excess of patients’ objective muscle weakness; deteriorating social activities despite stagnant physical abilities; significant individual differences in certain domains). The psychological effects of disease burden were also reflected in PROMs. In addition to medical examination and certain endpoints monitored by physicians, patient perspectives need to be taken into account when assessing the effectiveness of new, innovative treatments. With involvement of patients, information can be obtained that might remain uncovered during regular medical visits, although it is essential in determining the directions and priorities of clinical research. For all orphan medicines we emphasize to include patients in a compulsory manner to obtain general and disease-specific multidimensional outcome measures and use them as a quality indicator to monitor treatment effectiveness.]

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[Treatment and new evidences in neuromyelitis optica spectrum disorder Illés Zs, MD, PhD Ideggyogy Sz 2021;74(9–10):309–321. Neuromyelitis optica spectrum disorder (NMOSD) is associated with antibodies against AQP4 in about 80% of the cases. In about one-fourth of seronegative cases, antibodies against the MOG protein are present in the serum (MOG-antibody associated disease, MOGAD). This article discusses off-label azathioprine and mycophenolate mofetil in the treatment of NMOSD and reviews the evidence-based clinical aspects of B/plasma cell depletion, antagonization of IL-6 signaling and blocking the complement pathway. The review also summarizes basic aspects of NMOSD pregnancy focusing on treatment, and the different therapeutic approach in MOGAD. In the recent two years, phase 3 clinical trials provided class I evidence for the efficacy and safety of rituximab (anti-CD20), inebilizumab (anti-CD19), tocilizumab (anti-IL6R), satralizumab (anti-IL6R), and eculizumab (anti-C5) in combination with other immunosuppressants or in monotherapy. The treatment approach in MOGAD is complicated by the monophasic course in about half of the cases and by the potential disappearance of MOG antibody. The necessity of maintenance treatment in MOGAD should be decided after tapered oral steroid. Immunosuppression is recommended in NMOSD during pregnancy and lactation, and this should be considered for optimal selection of treatment in fertile female patients. The new monoclonal antibodies broadened treatment options NMOSD, and the treatment strategy of MOGAD has become more straightforward.]

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