Myelodysplastic syndromes and autoimmune phenomena – case report and literature overview

SAŽETAK Mijelodisplastični sindromi heterogena su skupina poremećaja hematopoeze karakterizirana displazijom jedne ili više linija hematopoeze u koštanoj srži, te inefektivnom hematopoezom i citopenijama u perifernoj krvi. Većinom se javljaju u pacijenata starije životne dobi i nose rizik za transformaciju u akutnu mijeloičnu leukemiju. Temelj tradicionalnog shvaćanja patogeneze ovog poremećaja jesu stečene genetske abnormalnosti krvotvorne matične stanice, uslijed starenja ili određenih noksi. Međutim, sve više kliničkih opservacija i imunoloških studija mijelodisplastične sindrome povezuje i s kroničnom upalom. Prikazujemo bolesnicu u dobi od 57 godina, kod koje je inicijalno postavljena dijagnoza autoimunosne bolesti, nedeterminirane kolagenoze, koja je liječena od strane nadležnog imunologa/reumatologa. Postupno se u laboratorijskim nalazima uočava pancitopenija te se hematološkom obradom postavlja dijagnoza mijelodisplastičnog sindroma s viškom blasta tipa 1, visokog rizika po IPSS-u (engl. kratica za International prognostic scoring system). Tijek liječenja ove bolesnice kompliciran je mnogobrojnim infektivnim komplikacijama, što govori u prilog poremećene funkcije imunosnog sustava, čak i prije same pojave autoimunosnih fenomena i mijelodisplazije. Epidemiološke studije pokazuju veću incidenciju autoimunosnih fenomena i bolesti u populaciji bolesnika s mijelodisplastičnim sindromom, u čak 10 – 20% bolesnika. Također pojava autoimunosnih manifestacija može prethoditi dijagnozi mijelodisplastičnog sindroma, i obratno. Pretkliničke studije nam daju uvid u podležeće imunološke patofiziološke mehanizme, pokazujući prisutnost imunosne deregulacije u ovim dvjema, naizgled vrlo različitim, skupinama bolesti. S obzirom na sve više podataka na kliničkoj i pretkliničkoj razini o povezanosti mijelodisplastičnih sindroma i autoimunosnih poremećaja, svakako je potrebno uzeti u obzir mogućnost istovremene pojave obiju bolesti, radi praćenja i liječenja bolesnika.SUMMARY Myelodysplastic syndromes are a heterogeneous group of hematopoiesis disorders, characterized by dysplastic changes in one or more hematopoietic lineages, ineffective hematopoiesis, and peripheral blood cytopenias. They occur mostly in elderly patients and carry a significant risk of acute myeloid leukemia transformation. Acquired genetic abnormalities are the foundation of traditional pathogenesis understanding, however, a growing body of evidence links myelodysplastic syndromes to chronic inflammation. Here we present a case of a female patient with autoimmune phenomena and myelodysplasia, as well as a literature overview corroborating the inflammatory pathogenesis of myelodysplastic syndromes. We present a case of a 57-year-old female patient, initially diagnosed with an autoimmune disease, undifferentiated connective tissue disease, continuously assessed and treated by a clinical immunologist-rheumatologist. Peripheral blood cytopenias occurred and worsened gradually, leading to the diagnosis of myelodysplastic syndrome with excess blasts, type 1, high-risk disease. The disease course of this patient was complicated by numerous infectious complications, occurring even before autoimmune phenomena and myelodysplasia. Epidemiological studies show a greater incidence of autoimmune phenomena and diseases in the population of patients with diagnosed myelodysplastic syndromes, even up to 10–20%. Also, the occurrence of autoimmune manifestations can precede myelodysplastic syndromes diagnosis, and vice-versa. Preclinical studies give insight into underlying immunological pathophysiological mechanisms, implying the presence of immune dysregulation in these two, seemingly very different, disease groups. Given the growing body of clinical and preclinical data on the association of myelodysplastic syndromes and autoimmune phenomena, it is necessary to consider the possibility of a joint occurrence in everyday clinical practice, which can have significant implications for patient management and new therapeutic options development

With food to health : proceedings of 11th International symposium

Proceedings contains 13 original scientific papers, 10 professional papers and 2 review papers which were presented at "10th International Scientific and Professional Conference WITH FOOD TO HEALTH", organised in following sections: Nutrition, Dietetics and diet therapy, Functional food and food supplemnents, Food safety, Food analysis, Production of safe food and food with added nutritional value

Health Effects of Ionizing Radiation on the Human Body

Radioactivity is a process in which the nuclei of unstable atoms spontaneously decay, producing other nuclei and releasing energy in the form of ionizing radiation in the form of alpha (α) and beta (β) particles as well as the emission of gamma (γ) electromagnetic waves. People may be exposed to radiation in various forms, as casualties of nuclear accidents, workers in power plants, or while working and using different radiation sources in medicine and health care. Acute radiation syndrome (ARS) occurs in subjects exposed to a very high dose of radiation in a very short period of time. Each form of radiation has a unique pathophysiological effect. Unfortunately, higher organisms—human beings—in the course of evolution have not acquired receptors for the direct “capture” of radiation energy, which is transferred at the level of DNA, cells, tissues, and organs. Radiation in biological systems depends on the amount of absorbed energy and its spatial distribution, particularly depending on the linear energy transfer (LET). Photon radiation with low LET leads to homogeneous energy deposition in the entire tissue volume. On the other hand, radiation with a high LET produces a fast Bragg peak, which generates a low input dose, whereby the penetration depth into the tissue increases with the radiation energy. The consequences are mutations, apoptosis, the development of cancer, and cell death. The most sensitive cells are those that divide intensively—bone marrow cells, digestive tract cells, reproductive cells, and skin cells. The health care system and the public should raise awareness of the consequences of ionizing radiation. Therefore, our aim is to identify the consequences of ARS taking into account radiation damage to the respiratory system, nervous system, hematopoietic system, gastrointestinal tract, and skin