A Bardy1, A Tiple2, M Rabant3, J-L Kémény4, K El Karoui5, M Hermet1, G Guettrot-Imbert1, P Deteix2, J-O Bay6, O Aumaître1, M André7. 1. Service de médecine interne, CHU Gabriel-Montpied, 58, rue Montalembert, 63000 Clermont-Ferrand, France. 2. Service de néphrologie, CHU Gabriel-Montpied, 58, rue Montalembert, 63000 Clermont-Ferrand, France. 3. Service d'anatomopathologie, hôpital Necker, Assistance publique-Hôpitaux de Paris, 149, rue de Sèvres, 75015 Paris, France; Université Paris-Descartes, 75006 Paris, France. 4. Service d'anatomie pathologique, CHU Gabriel-Montpied, 58, rue Montalembert, 63000 Clermont-Ferrand, France. 5. Service de néphrologie adultes, hôpital Necker, Assistance publique-Hôpitaux de Paris, 149, rue de Sèvres, 75015 Paris, France; Inserm U845, hôpital Necker, Assistance publique-Hôpitaux de Paris, 149, rue de Sèvres, 75015 Paris, France. 6. Service d'hématologie clinique, CHU Estaing Clermont-Ferrand, 1, place Lucie-Aubrac, 63000 Clermont-Ferrand, France. 7. Service de médecine interne, CHU Gabriel-Montpied, 58, rue Montalembert, 63000 Clermont-Ferrand, France. Electronic address: mandre@chu-clermontferrand.fr.
Abstract
PURPOSE: Myeloproliferative neoplasms (MPN) are hematological disorders characterized by clonal expansion of one or more medullary lines. Renal complications are rare, chiefly as acute renal failure. Glomerular involvement is exceptional METHODS: We report on a retrospective multicenter case series of eight patients who presented with a glomerulopathy (GP) associated with MPN RESULTS: All GP were revealed by a major proteinuria frequently associated with nephrotic syndrome and oedema. Histology was mainly characterized by lesions of focal segmental glomerulosclerosis associated with increased mesangial cellularity. The pathophysiology is still unclear but platelet-derived growth factor (PDGF) and transforming growth factor-β (TGF-β), which play a central role in the MPN may be involved. A majority of patients developed chronic renal failure despite of a therapy intended to block the renin-angiotensin system CONCLUSION: Monitoring of proteinuria during the follow-up of MPN would allow earlier diagnosis of renal involvement. Further studies on a larger scale are needed to specify the pathophysiological mechanisms involved and the management of these complications.
PURPOSE: Myeloproliferative neoplasms (MPN) are hematological disorders characterized by clonal expansion of one or more medullary lines. Renal complications are rare, chiefly as acute renal failure. Glomerular involvement is exceptional METHODS: We report on a retrospective multicenter case series of eight patients who presented with a glomerulopathy (GP) associated with MPN RESULTS: All GP were revealed by a major proteinuria frequently associated with nephrotic syndrome and oedema. Histology was mainly characterized by lesions of focal segmental glomerulosclerosis associated with increased mesangial cellularity. The pathophysiology is still unclear but platelet-derived growth factor (PDGF) and transforming growth factor-β (TGF-β), which play a central role in the MPN may be involved. A majority of patients developed chronic renal failure despite of a therapy intended to block the renin-angiotensin system CONCLUSION: Monitoring of proteinuria during the follow-up of MPN would allow earlier diagnosis of renal involvement. Further studies on a larger scale are needed to specify the pathophysiological mechanisms involved and the management of these complications.