“Primary immune deficiencies – today and tomorrow”, the symposium held on December 8-9, 2006 in Warsaw (Poland) has been a wonderful opportunity to celebrate the twenty fifth anniversary of the Department of Immunology in the Children’s Memorial Health Institute (CMHI) in Warsaw, to summarize the achievements of the team working in the field of pediatric clinical immunology, and to share own experience with guests coming from various centers in Poland and from abroad. It was the opportunity to recall the past times, when the team started its activities and the people, who shared their efforts in order to raise the team with its current achievements – most of all, professor Maria Goncerzewicz, the retired first director of the Institute, being also the founder of the Department of Immunology. The doctor’s team had also the opportunity to presents various moments of ups and downs of the everyday work in a humorous poem written by an anonymous author inspired by the team, describing their boss, professor Ewa Bernatowska in private and official situations.
Among results of various activities and achievements of the Department of Immunology CMHI during past ten years there are almost two hundred publications, numerous scientific grants completed or currently running, great number of centers in Poland and abroad cooperating in the field of diagnostics and treatment of primary immunodeficiencies. All these would not be possible without previous achievements of Polish nestors in clinical immunology, professor Zbigniew Rudkowski and the late professor Henryka Krystyna Siwińska-Gołębiowska. The commemorative medals of the Polish Society of Experimental and Clinical Immunology for outstanding input into the development of clinical immunology in Poland have been awarded to the Department of Immunology and personally to professor Ewa Bernatowska. Both medals have been given on behalf of the Society by professor Maciej Kurpisz (figure 1).
Clinical immunology today resembles a puzzle. Various clinical symptoms, numerous diagnostic tools, and many therapeutic options, compose a very complicated system. Among many people, whose knowledge of immunology is so important in finding the right clinical solution, is professor Jacques J.M. van Dongen from the Department of Immunology, University Medical Center Rotterdam. He is cooperating with the Department of Immunology and the Department of Clinical Genetics of the CMHI since 2000. Due to this cooperation confirmation of the diagnosis on the molecular level was possible in 71 out of 117 children with primary immune deficiency. This allowed to initiate substitution treatment with gammaglobulin preparations in patients with agammaglobulinemia, to perform bone marrow or hematopoietic stem cell transplantation in children with severe combined immunodeficiencies, and – what maybe is the most important – genetic counseling for parents of the children with many primary immune deficiencies. In recognition of his merits he has been awarded the medal “The friend of the Child’s Health Center” (figure 2). Professor van Dongen gave an impressive lecture summarizing mechanisms of many primary immune deficiencies and methods of their diagnosing by molecular and flow cytometric methods.
The celebration would not be complete without scientific program and lectures given by authors from many centers in Poland taking care of patients with primary immune deficiencies. The presentations have been divided into five sessions.
During the session on primary antibody deficiencies
dr. Pac presented problems with early diagnostics and treatment in patients with agammaglobulinemia. Professor Adam Jankowski gave a broad overview of clinical symptoms and diagnostics of IgA deficiencies. Describing the common variable immune deficiency (CVID) dr. A. Lewandowicz-
-Uszyńska focused on clinical symptoms and the diagnostics of CVID and dr. J. Michałkiewicz – on several phenomena found during cell culture in such patients.
The session on combined immunodeficiencies included presentations on spectrum of clinical symptoms in severe combined immunodeficiencies (dr. B. Wolska-Kuśnierz), cytometric diagnostics in individual immune deficiencies (dr. B. Piątosa), as well as presentations on clinical symptoms, diagnostics, and complex care in patients with such combined immunodeficiencies as Nijmegen syndrome or ataxia teleangiectasia, with lectures given by dr. K. Chrzanowska and dr. B. Pietrucha, respectively. Presentation of a case of combined immune deficiency with unknown etiology by
dr. A. Szaflarska completed the session.
Many participants of the symposium work in centers dedicated to general pediatrics. The session on fungal, bacterial, viral, and parasitic infections addressing various clinical symptoms of primary immunodeficiencies, with lectures on recurrent infections (prof. K. Zeman), pulmonary (dr. J. Paśnik), gastrointestinal (dr. A. Szaflarska), or skin infections (dr. A. Cywińska-Bernas) in children with primary immunodeficiencies, and presentations of therapeutic effects in aspergillosis in patients with chronic granulomatous disease (dr. M. Kurenko-Deptuch) or cryptosporidial infections in patients with hyper-IgM syndrome and CD4-lymphopenia (dr. E. Heropolitańska-Pliszka) completing this part of the meeting, gave scientific background and an extensive overview of clinical and diagnostic problems in such patients.
This first day composed of memories, congratulations, awards, and lots of interesting lectures has been completed by a totally unofficial meeting in Stary Młyn, with dinner, songs dedicated to Ewa Bernatowska, and dance continued until late night hours.
The second day of the symposium started with the session on vaccinations in immune disorders. Several topics have been addressed, among them vaccination with combined vaccines and vaccination in risk groups (prof. E. Bernatowska), prophylaxis of pneumococcal infections in children with asplenia or splenic dysfunction (dr. B. Mikołuć), invasive infections with encapsulated bacteria and new vaccines, that can be used for patients with immune disorders (dr. P. Grzesiowski). Vaccinations against varicella zoster and influenza have been thoroughly discussed by dr. T. Jackowska
and dr. E. Duszczyk.
Periodic fever syndromes may results from various inherited or non-inherited defects. They have been thoroughly discussed in the varia session by dr. B. Wolska--Kuśnierz. The case of Griscelli type II syndrome has been described by dr. Z. Kycler, and the diagnostic algorithm in children with suspected hemophagocytic syndrome by
dr. M. Klaudel-Dreszler.
There are several diseases with significant immune component. It is obvious, that many of them have not been discussed during this symposium, neither from the general or pediatric perspective. Despite many attempts undertaken worldwide awareness of primary immune deficiencies is not sufficient and the diagnostic guidelines for many immune disorders are missing. Therefore, the meeting of the Polish Working Group for Primary Immunodeficiencies aimed at discussing the plans for preparing diagnostic guidelines helpful for doctors not specialized in clinical immunology, but taking care of the patients, was very important. The next meeting aimed at discussing guidelines prepared in the meantime by participants of the Polish Working Group was planned for May to be held in Poznań.
