Haemodiafiltration with ultrafiltrate regeneration in the removal of free light chains in multiple myeloma and acute kidney injury / Hemodiafiltración con regeneración de ultrafiltrado en la eliminación de cadenas ligeras libres en mieloma múltiple y lesión renal aguda

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[Renal involvement in Erdheim-Chester disease]. / Il coinvolgimento renale nella Malattia di Erdheim-Chester.

The Erdheim-Chester disease is a rare form of Langherans cells. Since 1987 it is distinguished from other istiocytosis previously identified. The diagnosis of the disease relies on defined radiological (bone imaging) and pathological (histiocytic infiltration) criteria. Bone disease is crucial but systemic manifestations are reported more frequently at onset. Renal involvement is always asymptomatic at onset of disease or in the follow-up. In this review we analyze the reports of the literature; we highlight 3 pathological mechanisms of renal involvement: renal and retroperitoneal infiltration, urinary tract obstruction, renal arteries stenosis. No treatment to date has demonstrated an improvement in survival of patients with EC. Renal involvement is therefore symptomatic (ureteral stenting, percutaneous nephrostomy) or is adopted a wait-and-see attitude.