Access restricted to higher education institution's students and staff
master's thesis
Dijagnoza i liječenje Waldenstroemove makroglobulinemije

Jelena Šimić (2015)
Metadata
TitleDijagnoza i liječenje Waldenstroemove makroglobulinemije
AuthorJelena Šimić
Mentor(s)Toni Valković (thesis advisor)
Abstract
SZO definira WM kao limfoplazmacitični limfom u kojem je koštana srž infiltrirana klonalnim limfoplazmacitičnim stanicama koje proizvode IgM. Klinička slika može uključivati opće simptome, simptome hiperviskoznosti, perifernu neuropatiju, citopenije, te uvećanje jetre, slezene i/ili limfnih čvorova, a rijeđe se javljaju simptomi uzrokovani krioglobulinemijom, te simptomi disfunkcije organa uzrokovane amiloidozom lakih lanaca. Uvjet za dijagnozu jest dokaz malignih limfoplazmacitičnih stanica u aspiratu i bioptatu koštane srži potvrđen imunofenotipizacijom, uz nalaz imunoelektroforeze koji potvrđuje prisustvo monoklonalnog IgM. Bitno je učiniti hematološke i biokemijske analize krvi, te analizu viskoznosti seruma. CT se koristi za utvrđivanje ekstramedularne proširenosti bolesti, a fundoskopijom se otkrivaju promjene na mrežnici koje su posljedica hiperviskoznosti seruma. Međunarodnim prognostičkim „scoring“ sustavom se, pomoću parametara koji uključuju životnu dob, broj trombocita, te koncentraciju hemoglobina, monoklonalnog IgM i β2-mikroglobulina u krvi, određuje se medijan preživljenja. Kod asimptomatskih pacijenata koristi se „watch-and-wait“ pristup, dok se u onih sa manifestacijama bolesti u inicijalnoj terapiji primjenjuje neka od sljedećih kombinacija: DRC, bendamustin s rituksimabom, BDR ili kombinacija bortezomiba i rituksimaba bez deksametazona. Kod minimalno izražene kliničke slike koristi se samo rituksimab, a kod teškog općeg stanja primjenjuje se fludarabin. Simptomi uzrokovani paraproteinemijom u akutnoj se fazi reguliraju plazmaferezom, dok se dugoročno moraju držati pod kontrolom pomoću rituksimaba i kemoterapije. Relaps nakon ≥12 mjeseci liječi se inicijalno primjenjenim režimom, a ako se pojavi ranije daje se neka od drugih opcija prve linije ili se koriste agensi poput ibrutiniba, karfilzomiba ili talidomida. Kod agresivnijih oblika bolesti korisno je liječenje autolognim hematopoteskim matičnim stanicama.
KeywordsWaldenstrom’s macroglobulinemia diagnosis prognosis treatment
GranterSveučilište u Rijeci
Medicinski fakultet
Lower level organizational unitsKatedra za internu medicinu
PlaceRijeka
StateCroatia
Scientific field, discipline, subdisciplineBIOMEDICINE AND HEALTHCARE
Clinical Medical Sciences
Internal Medicine
Study programme typeuniversity
Study levelintegrated undergraduate and graduate
Study programmeMedicine
Academic title abbreviationdr. med.
Genremaster's thesis
Language Croatian
Defense date2015
Parallel abstract (English)
WHO defines WM as lymphoplasmacytic lymphoma in which the bone marrow is infiltrated by IgM-producing clonal lymphoplasmacytic cells. Clinical features include constitutional symptoms, symptoms of hyperviscosity, peripheral neuropathy, cytopenias, hepatomegaly, splenomegaly and/or lymphadenopathy, while symptoms caused by cryoglobulinemia or symptoms of organ dysfunction due to light-chain amyloidosis occur less frequently. To establish the diagnosis, it is required to prove the presence of malignant lymphoplasmacytic cells in the bone marrow aspirate and bioptate, together with occurrence of IgM monoclonal protein in patient's blood. Analyses of hematological and biochemical parameters of blood and serum viscosity are necessary. CT scans are used to detect extramedullary disease. Fundoscopy reveals retinal changes due to hyperviscosity. Parameters such as patient's age, platelet count, hemoglobin, β2-microglobulin and monoclonal IgM concentration are used in the International prognostic scoring system to estimate median survival time. „Watch-and-wait“ strategy is used in asymptomatic patients, while symptomatic patients are treated with first-line therapy regimens, such as DRC, bendamustine plus rituximab, BDR or bortezomib plus rituximab without dexamethasone. Rituximab alone is given to minimally symptomatic patients, and fludrabine is administered to those with poor performance status. Acute symptoms of hyperviscosity are treated with plasmapheresis, but in the long term they need to be controlled with rituximab and chemotherapy. Disease that relapsed ≥12 months following initial therapy is treated with the original regimen, whereas patients with remission <12 months are treated with alternative first-line agents or agents such as ibrutinib, carfilzomib and thalidomide. Clinically aggressive disease can be treated with autologous hematopoietic cell transplantation.
Parallel keywords (Croatian)Waldenströmova makroglobulinemija dijagnoza prognoza liječenje
Resource typetext
Access conditionAccess restricted to higher education institution's students and staff
Terms of usehttp://rightsstatements.org/vocab/InC/1.0/
URN:NBNhttps://urn.nsk.hr/urn:nbn:hr:184:102166
CommitterBosa Licul