التفاصيل البيبلوغرافية
| العنوان: |
Mijelodisplastični sindromi i autoimunosni fenomeni – prikaz bolesnika i pregled literature. (Croatian) |
| Alternate Title: |
Myelodysplastic syndromes and autoimmune phenomena – case report and literature overview. (English) |
| المؤلفون: |
Kerep, Ana Zelić, Kotris, Ana, Narančić, Marino, Smoljanović, Inga Mandac |
| المصدر: |
Lijecnicki Vjesnik; 2023, Vol. 145 Issue 1/2, p27-31, 5p |
| Abstract (English): |
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. [ABSTRACT FROM AUTHOR] |
| Abstract (Croatian): |
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. [ABSTRACT FROM AUTHOR] |
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| قاعدة البيانات: |
Complementary Index |