BOLEST TALOŽENJA LAKIH LANACA

Danijela Jovanović; Predrag Đurđević

doi:10.5937/smclk5-52533

Danijela Jovanović Katedra za Internu medicinu, Fakultet medicinskih nauka, Univerzitet u Kragujevcu
Predrag Đurđević Klinika za hematologiju, Univerzitetski klinički centar Kragujevac; Fakultet medicinskih nauka, Univerzitet u Kragujevcu, Kragujevac, Srbija

DOI: https://doi.org/10.5937/smclk5-52533

Ključne reči: bolest deponovanja lakih lanaca, dijagnostika, terapija

Sažetak

Bolest taloženja lakih lanaca predstavlja jedan od oblika bolesti taloženja monoklonskih imunoglobulina. Po učestalosti, reč je o retkom entitetu, koji se javlja kod osoba srednjeg životnog doba, češće muškog pola. Najčešće zahvata bubrege, sa kliničkom slikom nefritičkog sindroma, ali može biti lokalizovana i u drugim organima, kao što su jetra, pluća, srce, gastrointestinalni trakt, koža i drugi. U kliničkoj slici dominiraju simptomi i znaci zahvaćenog organa. Dijagnoza se najčešće postavlja biopsijom zahvaćenog organa, pri čemu se bojenjem na Kongo crveno razlikuje od amiloidoze lakih lanaca, a potom se vrši evaluacija koštane srži radi isključivanja drugih plazmocitnih diskrazija. Terapija je bazirana na terapijskim modalitetima za lečenje multiplog mijeloma, uključujući inhibitore proteazoma (bortezomib), uz autologu transplantaciju matičnih ćelija hematopoeze, ali i transplantaciju zahvaćenih organa ukoliko je došlo do potpunog gubitka funkcije. Podaci o terapiji monoklonskim antitelima (daratumumab) otvaraju nove terapijske mogućnosti u lečenju ove bolesti. Dijagnostika i lečenje ove bolesti zahtevaju multidisciplinarni pristup, prvenstveno nefrologa, hematologa i patologa.

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