Hiperplazija timusa kao retka etiologija čiste aplazije eritroidne loze

  • Isidora Arsenović University Clinical Center of Serbia, Clinic of Hematology, Belgrade, Serbia
  • Danijela Leković University Clinical Center of Serbia, Clinic of Hematology, Belgrade, Serbia; University of Belgrade, Faculty of Medicine, Belgrade, Serbia
  • Dijana Šefer University Clinical Center of Serbia, Clinic of Hematology, Belgrade, Serbia
  • Jelena Ivanović University Clinical Center of Serbia, Clinic of Hematology, Belgrade, Serbia
  • Mihajlo Smiljanić University Clinical Center of Serbia, Clinic of Hematology, Belgrade, Serbia
  • Andrija Bogdanović University Clinical Center of Serbia, Clinic of Hematology, Belgrade, Serbia; University of Belgrade, Faculty of Medicine, Belgrade, Serbia
DOI: https://doi.org/10.2298/VSP230803045A
Ključne reči: anemija, dijagnoza, histološke tehnike, aplazija crvene loze, čista, hirurgija, torakalna, video-asistirana, timom, timus, hiperplazija

Sažetak


 

Uvod. Hiperplazija timusa je retko stanje koje nastaje kao posledica povećanog broja ćelija timusa, koje se u nekim slučajevima može dovesti u vezu sa autoimunskim bolestima kao što je čista aplazija eritroidne loze (pure red cell aplasia – PRCA). Timektomija se smatra najefikasnijim terapijskim modalitetom za lečenje PRCA povezane sa timomom, sa stopom potpune remisije od 31,5%. Ostali načini lečenja mogu dovesti do parcijalne ali ne i kompletne remisije. Prikazana je bolesnica kod koje je PRCA bila povezana sa hiperplazijom timusa, sa naglaskom na efikasnosti timektomije kao incijalnog terapijskog pristupa. Prikaz bolesnika. Prikazana je, prethodno zdrava, žena stara 18 godina, sa teškom anemijom kod koje je nakon hematoloških ispitivanja, uključujući i potvrdu biopsijom koštane srži, postavljena dijagnoza PRCA. Vrednosti imunoloških i virusoloških analiza bile su u granicama referentnih vrednosti. Zbog pojave timoma u porodičnoj anamnezi  i poznate veze između timoma i PRCA, urađena je magnetna rezonanca grudnog koša kojom je dokazano postojanje hiperplazije timusa. Kod bolesnice je sprovedena najmanje invazivna hirurška procedura – totalna timektomija primenom video-asistirane torakoskopske hirurgije. Patohistološki pregled operativnog materijala potvrdio je prisustvo hiperplazije tkiva timusa, sa prisutnom „jednostavnom“ cistom unutar timusa. Nakon timektomije, hematološki parametri bolesnice su se značajno poboljšali. Zaključak. Tok i ishod lečenja bolesnice podržavaju primenu timektomije u lečenju PRCA koja je povezana sa hiperplazijom timusa. Ipak, potrebna su dalja istraživanja i praćenja kako bi se optimizovao terapijski pristup za to retko stanje.

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Objavljeno
2024/01/30
Broj časopisa
Vol. 81 Br. 01 (2024): Vojnosanitetski pregled
Rubrika
Prikaz bolesnika