#4295 SPECTRUM OF GLOMERULAR AND VASCULAR KIDNEY PATHOLOGY ASSOCIATED WITH MYELOPROLIFERATIVE NEOPLASMS

Abstract Background and Aims Myeloproliferative neoplasms (MPN) are chronic blood disorders defined by an overproduction of clonal, differentiated hematopoietic cells. A high prevalence kidney disease (CKD) is described in patients with MPN. The glomerular histological pattern MPN-related disease, glomerulopathy (MPN-RG), may not account for the entirety CKD risk this population, as it appears to be rare has mostly been reported myelofibrosis. inflammatory prothrombotic state clonal cells linked a systemic vasculopathy MPN patients, characterized thromboses microvascular disease. Thus, we put forward hypothesis that intrarenal vessel injury occur during MPN, vascular nephrosclerosis common presenting Method We conducted exhaustive, retrospective, multicenter study biopsies four pathology units. All adult history who underwent biopsy were included. performed systematic review compartments. MPN-RG was mesangial expansion negative immunofluorescence studies, at least one following: hypercellularity, or features thrombotic microangiopathy. Hematopoietic detected using anti-glycophorin C, anti-myeloperoxidase, anti-factor VIII immunohistochemistry (IHC). Chronic lesions evaluated Banff aah cv lesion definitions compared matched native biopsies. used multivariable logistic regression models assess determinants lesions, number antihypertensive drugs proxy hypertension severity. Results included 47 biopsy, including 16 myeloid leukemia, 14 polycythemia vera, 10 essential thrombocythemia 7 primary cases (29.8%) met our definition MPN-RG. strongly associated myelofibrosis (primary secondary, P = 0.021) poorer ESRD-free survival (P 0.007). Positive IHC circulating capillaries but positively correlated leukocyte count 0.004). Thirty-three (75.0%) had moderate-to-severe arteriosclerosis; 39 (84.8%) arteriolar hyalinosis. Multivariable 188 control revealed association between damage, which independent established factors such age, diabetes mellitus (Table 1 Table 2). Conclusion demonstrate strong confirm its poor renal prognosis. argue finding intracapillary should considered diagnosis Most notably, results show represent novel, factor nephrosclerosis, establish new link CKD. These findings raise hypotheses regarding pathophysiology general population.