| Abstract | Bolesnici s kroničnim mijeloproliferativnim neoplazmama (MPN) u četvrtini slučajeva prilikom dijagnoze imaju kroničnu bubrežnu bolest (KBB). Patohistološke studije ukazuju kako bi mogla postojati glomerulopatija povezana s MPN. Ciljevi ovog rada su proučiti povezanost opterećenja mutiranim JAK2 V617F alelom s prisustvom KBB i s dinamikom bubrežne funkcije tijekom vremena.
Analizirano je ukupno 230 bolesnika, 98 s PV, 94 s ET, 20 s PMF i 18 s drugim MPN. Medijan opterećenja mutiranim alelom bio je 26.3%, raspon 0.1-96. Medijan serumskog kreatinina bio je 84 mcmol/L, raspon 36-174. Ukupno 46/230 (24.9%) bolesnika imalo je KBB. Bolesnici s KBB pri dijagnozi imali su veće opterećenje mutiranim alelom (medijan 37.6 vs 23.4%, P=0.040). Dinamika vrijednosti kreatinina tijekom vremena značajno se razlikovala ovisno o razini opterećenja mutiranim alelom pri dijagnozi (2% pogoršanje vs. 7% poboljšanje u pacijenata s većim opterećenjem mutiranim alelom vs. pacijenti s manjim opterećenjem mutiranim alelom, (P=0.032)). Veće opterećenje JAK2 V617F muiranim alelom pri dijagnozi povezano je s većom učestalosti KBB kao i nepovoljnom dinamikom (pogoršanjem) bubrežne funkcije tijekom vremena u bolesnika s kroničnim mijeloproliferativnim neoplazmama. Navedeno govori u prilog povezanosti povećane aktivnosti maligne bolesti i lošije bubrežne funkcije. |
| Abstract (english) | Patients with chronic myeloproliferative neoplasms (MPN) often have a chronic kidney disease (CKD) at the time of diagnosis. Two diseases may be causally related as pathohistological studies suggest existence of MPN-related glomerulopathy. Aims of this study are to examine the correlation between renal function and JAK2-mutation allele burden in MPN patients and analyse dynamics of mutant allele burden (MAB) and CKD.
230 MPN patients were enrolled: 98 polycythaemia vera, 94 essential thrombocythemia, 20 primary myelofibrosis, 18 MPN not classified. At the time of the diagnosis 24.9% of patients had CKD. MPN patients with higher mutant allele burden stratified at median value (>26.3%) had higher occurrence of CKD at baseline (32.6% vs 16.7%, P=0.012). MAB differed significantly between different MPN subtypes at the baseline (P<0,001), median value in PV patients was 47,5%, 16,5% in ET, 39,5% in PMF and 21,6% in MPN unclassified. Patients with CKD at the time of diagnosis had higher MAB (mean 37,6 vs. 23,4%, P=0,040). Dynamics of kidney function significantly differed regarding baseline allele burden with mean 2% worsening and 7% improvement in serum creatinine levels in patients with higher vs lower baseline mutant allele burden, respectively, P=0.032. JAK2 V617F mutated MPN patients with higher mutant allele burden may have higher occurrence of conomitant CKD and unfavorable dynamics of kidney function over time. Although causative relationship cannot be inferred due to the limitations of the study design, our observations support the view that biology of MPN and CKD might be intertwined. |
