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What causes cast nephropathy?

What causes cast nephropathy?

Cast nephropathy is caused by the interaction and aggregation of filtered free light chains and Tamm–Horsfall protein causing intratubular obstruction and damage. The key to treating cast nephropathy is rapid lowering of free light chains, because this correlates with renal recovery.

How is cast nephropathy treated?

The key to treating cast nephropathy is rapid lowering of FLCs as this correlates with renal recovery. Newer chemotherapy agents lower FLCs and have been referred to as “renoprotective”. However there remains great interest in using various extracorporeal therapies to remove serum FLCs.

Can multiple myeloma cause renal failure?

Kidney failure is a common complication of multiple myeloma. When first diagnosed, as many as 20-40% of patients with multiple myeloma will have some amount of kidney failure. Multiple myeloma can affect the kidney in several ways.

What causes light chain nephropathy?

Light chain cast nephropathy (LCCN) is the most common renal disease caused by multiple myeloma (MM). In addition to ordinary light chain protein casts, there are a few rare casts with unique shapes, including light chain amyloid casts (LCAC) and light chain crystal casts (LCCC).

Is cast nephropathy?

Myeloma cast nephropathy, also referred to as light-chain cast nephropathy, is the formation of plugs (urinary casts) in the kidney tubules from free immunoglobulin light chains leading to kidney failure in the context of multiple myeloma. It is the most common cause of kidney injury in myeloma.

Why does myeloma cause renal failure?

Renal failure is a frequent complication in patients with multiple myeloma (MM) that causes significant morbidity. In the majority of cases, renal impairment is caused by the accumulation and precipitation of light chains, which form casts in the distal tubules, resulting in renal obstruction.

How long can you live with light chain disease?

Median survival for patients with light-chain deposition disease (LCDD) is about 4 years. The largest series published so far has reported after a median follow-up of 27 months; 57% of patients developed uremia and 59% of patients died.

What is the treatment for light chain disease?

The goal of treating LCDD is to slow the production of light chains and their damage to organs. Treatment may include chemotherapy with a drug called Bortezomib; autologous stem cell transplantation; immunomodulatory drugs; and/or kidney transplant. If untreated, end-stage renal disease occurs in 70% of cases.

What is cast nephropathy?

Light chain cast nephropathy (“myeloma kidney”) is a disease resulting from acute tubular injury (Fig. 1.146) due to the intratubular formation of large, eosinophilic, PAS-negative, and often cracked or fractured casts composed of a restricted light chain (Fig. 1.147).

What are the treatment options for cast nephropathy?

Treatment of Cast Nephropathy. The main objectives in treating cast nephropathy are to minimize the propensity of filtered light chains to form intratubular casts, to decrease production of the light chains by the clonal plasma cells, and to enhance removal of the light chains from the circulation.

Can a tubular protein cast cause interstitial nephritis?

Acute pyelonephritis, acute interstitial nephritis, and acute tubular necrosis can all have interstitial inflammation and tubule cell injury, and many diseases can result in the presence of tubular protein casts.

How are plasma cells involved in cast nephropathy?

Plasma cells are often present in close contact with these crystals. 173,174 Usually, the glomeruli do not show any changes unless they are involved by amyloid or by light chain disease. The concomitant presence of light chain disease or amyloidosis with cast nephropathy is rare.

What are the features of light chain cast nephropathy?

It is important to know the classic histologic features of light chain cast nephropathy, as in some cases the presence of LCCN may be the initial presentation of an underlying subclinical myelomatous process. 1. Light chain casts have an eosinophilic appearance on H&E stain, with a pale/light pink appearance on PAS.

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