Langvinnt eitilfrumuhvítblæði á Íslandi árin 2003-2013 Nýgengi, aðdragandi greiningar og undanfari
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Issue Date
2016-04
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Chronic lymphocytic leukemia in Iceland from 2003 to 2013: Incidence, presentation and diagnosisCitation
Læknablaðið 2016, 102(04):171-177Abstract
B-eitilfrumna. Einstofna B-eitilfrumudreyri (monoclonal B-cell lymphocytosis, MBL) er talið forstig sjúkdómsins. Sjúkdómurinn er ólæknandi en hæggengur og greinist oft fyrir tilviljun við skoðun blóðhags. Langvinnt eitilfrumuhvítblæði hefur ekki verið rannsakað á Íslandi hvað varðar nýgengi, greiningu, sjúkdómseinkenni eða hækkanir á eitilfrumutalningu í blóði fyrir greiningu. Efniviður og aðferðir: Um er að ræða afturskyggna, lýsandi rannsókn sem nær til sjúklinga sem greindust á árunum 2003-2013. Fengin voru gögn yfir sjúklinga frá Krabbameinsskrá, blóðmeinafræðideild Landspítala og Læknasetrinu í Mjódd og var skráning tilfella rakin. Sjúkraskrár voru skoðaðar með tilliti til einkenna, greininga og meðferðar. Upplýsingar um lifun fengust úr Þjóðskrá og um dánarorsakir frá landlækni. Niðurstöður: Fjöldi sjúklinga sem greindist með langvinnt eitilfrumuhvítblæði á rannsóknartímabilinu var 161 (109 karlar, 52 konur). Nýgengi mældist 4,55/100.000 en aldursstaðlað nýgengi 3,00/100.000. Meðalaldur við greiningu var 70,9 ár (35-96 ár). Krabbameinsskrá skorti upplýsingar um 28 sjúklinga (17,4%), en upphafleg greining var í 47,2% tilfella eingöngu gerð með flæðisjá. Sjúkdómseinkenni voru til staðar við greiningu hjá 67 af 151 sjúklingi (44,4%). Rúmur þriðjungur hópsins fékk lyfjameð- ferð og var meðaltími að meðferð 1,3 ár. Fimm ára lifun var um 70% en miðgildi lifunar 9,4 ár. Hækkun eitilfrumutalningar (4,0x109 /L) í blóði (0,1- 13,4 árum) fyrir greiningu fannst hjá 85 af 99 sjúklingum (85,9%). Ályktun: Nýgengi langvinns eitilfrumuhvítblæðis á Íslandi er svipað því sem þekkist á Vesturlöndum. Bæta þarf lögbundna skráningu tilfella í Krabbameinsskrá, sérstaklega þegar greining byggir á frumuflæðisjárrannsókn eingöngu. Eitilfrumuhækkun var til staðar hjá stórum hluta sjúklinga fyrir greiningu.Introduction: Chronic lymphocytic leukemia (CLL) is characterized by the proliferation of monoclonal B-lymphocytes. MBL (monoclonal B-cell lymphocytosis) is considered a precursor state of the disease. Although CLL is incurable it is an indolent disorder and often detected incidentally on routine blood counts. Until now little information has been available on CLL in Iceland, including the incidence, diagnosis, symptoms or MBL precursor state. Material and methods: This is a retrospective, descriptive study including CLL patients diagnosed in Iceland over the years 2003-2013. Registries of patients with a CLL diagnosis were obtained from the Icelandic Cancer Registry, Landspitali National University Hospital and the Medical Center in Mjódd. Medical records were reviewed for information on symptoms, diagnosis and treatment. Survival data and causes of death were obtained from national registries. Results: The number of patients diagnosed with CLL over the study period was 161 (109 males, 52 females). The calculated incidence was 4.55/100,000, and the age-standardized incidence was 3.00/100,000. Mean age at diagnosis was 70.9 years (range 35-96 years). The Icelandic Cancer Registry lacked information on 28 patients (17.4%). The initial diagnosis of CLL was obtained exclusively with flow cytometry in 47.2% of cases. Symptoms were present at diagnosis in 67 of 151 patients (44.4%). One third of the group received chemotherapy and the average time to treatment was 1.3 years. Five-year survival was 70% and median survival was 9.4 years. Elevated lymphocyte counts (≥4,0x109 /L) in peripheral blood prior (0.1 to 13.4 years) to diagnosis of CLL was identified in 85 of 99 CLL patients (85.9%). Conclusion: The incidence of CLL in Iceland is similar to other Western countries. The registration of CLL cases in the Icelandic Cancer Registry must be improved, especially in cases where diagnosis is based solely on flow cytometry. Elevated lymphocyte counts were present in a large proportion of cases prior to the diagnosis of CLL.
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