Renal Manifestations of Plasma Cell Neoplasms
Dysproteinemia is an important disease entity characterized by excessive amounts of abnormal monoclonal proteins in the circulation. The patient may be asymptomatic or may have symptoms secondary to deposition in the organs. The symptoms depend on the org
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Renal Manifestations of Plasma Cell Neoplasms L. Nicholas Cossey and Shree G. Sharma
Introduction Dysproteinemia is an important disease entity characterized by excessive amounts of abnormal monoclonal proteins in the circulation. The patient may be asymptomatic or may have symptoms secondary to deposition in the organs. The symptoms depend on the organ involved and the severity of involvement. If the patient stays asymptomatic for a long period, disease may become widespread and the deposition of the abnormal protein may be extensive. It is not only important to diagnose the type of dysproteinemia, but is also important to find out the cause of dysproteinemia for appropriate treatment. Common clinical causes of dysproteinemia are plasma cell myeloma, monoclonal gammopathy of unknown significance (MGUS), primary amyloidosis, and lymphoproliferative disorders. The most common dysproteinemia-related kidney diseases include light chain cast nephropathy, light chain proximal tubulopathy (LCPT), amyloidosis, monoclonal immunoglobulin deposit diseases (MIDD) and a recently described entity known as monoclonal gammopathy of renal significance (MGRS), in which the patient’s dysproteinemia may present only with renal dysfunction. Plasma cell myeloma is one of the most common causes of dysproteinemia and frequently affects the kidney. Greater than 50% of the patients with plasma cell myeloma present with renal insufficiency at the time of diagnosis [1]. Renal biopsy is an important tool in these cases to confirm involvement of kidney. Renal biopsy interpretation can be challenging in subtle cases and at initial stages of the disease. The present chapter will discuss important findings seen on renal biopsy and their interpretation.
L. Nicholas Cossey S.G. Sharma (&) Nephropath, 10810 Executive Center Dr. Ste. 100, Little Rock, AR 72211, USA e-mail: [email protected] © Springer International Publishing Switzerland 2016 R.B. Lorsbach and M. Yared (eds.), Plasma Cell Neoplasms, DOI 10.1007/978-3-319-42370-8_6
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L. Nicholas Cossey and S.G. Sharma
Light Chain Cast Nephropathy Light chain cast nephropathy (a.k.a. myeloma cast nephropathy) commonly presents as acute kidney injury and is one of the most common manifestations of plasma cell myeloma. The patient often has non-nephrotic range proteinuria that is not detected by urine dipstick. This is due to the fact that dipstick detects albumin but the proteinuria in these patients is primarily due to light chains (a.k.a. Bence-Jones protein) [2]. On histopathology, the disease is characterized by formation of casts in the distal tubules of the kidney. The casts are formed by the combination of Tamm–Horsfall protein and monoclonal light chains and are brittle, which imparts a fractured cast appearance [3]. The casts can lead to both obstruction as well as cause direct toxicity to the tubules. In some cases, the patient has an identifiable precipitating factor such as dehydration, diuretics, hypercalcemia, infections, and nephrotoxins [2]. Renal biopsy is necessary to
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