Multiple myeloma is a disease of the plasma cells of the bone marrow, resulting in the proliferation and release of the monoclonal protein, which further causes end-organ damage. We report an unusual presentation of multiple myeloma, thereby insisting on the need for the treating physician to be aware of the various presentations that can be encountered in regular practice. It is often difficult to diagnose, and the diagnosis is usually made at a late stage of the disease. Even though uncurable, with recent advances, a proper regimen, newer chemotherapeutic agents, and stem cell transplantation, the disease can be brought into remission.

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Proteinuria is a broad term used to describe the pathological presence of proteins, including albumin, globulin, Bence-Jones protein, and mucoprotein in the urine. When persistent, proteinuria is a marker of kidney damage and represents a reliable predictor of the risk of progression of renal failure. Medical nutrition therapy is imperative for patients with proteinuria because it may slow the progression of renal disease. The aim of this review is to explore different nutritional approaches in the management of proteinuria and their influence on pathophysiological processes. As such, protein restriction is the main dietary intervention. Indeed, other management approaches are frequently used to reduce it regarding micro and macronutrients, but also the dietary style. Among these, the nutritional approach represents one of the most used and controversial interventions and the studies rarely take the form of randomized and controlled trials. With this work we aspire to analyze current clinical knowledge of how nutrition could influence proteinuria, potentially representing a useful tool in the management of proteinuric nephropathy.

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In this study, we describe a multimodal approach to diagnose a unique case of myeloma-related disease, extranodal secretory B-cell lymphoma with urinary bladder involvement, an IgG4 monoclonal gammopathy, and Bence-Jones proteinuria in a dog with a 6-year history of hyperglobulinemia that had not been further evaluated. A 12-year-old dog was presented for evaluation of a 1-week history of tenesmus. Urine sediment cytologic evaluation revealed low to moderate numbers of intermediate to large-sized lymphocytes. We describe a technique that yielded adequate numbers of viable neoplastic cells in shipped urine sediment for PARR and flow cytometry. Those studies demonstrated a clonal immunoglobulin gene rearrangement and an expansion of CD21-positive and MHC Class II-negative B cells, respectively. Protein electrophoresis with immunofixation and proteomic evaluation revealed a serum and urine IgG4 monoclonal gammopathy with Bence-Jones proteinuria. MUM1 immunocytochemistry performed on the urine sediment slides failed to label the neoplastic cells; thus, a plasma cell tumor was considered unlikely. Lack of response to a cyclophosphamide, vincristine, and prednisone chemotherapy regimen led to euthanasia without necropsy 21 days after diagnosis. Lymphoma is the most common hematopoietic malignancy and accounts for up to a quarter of all neoplasms in dogs, but lymphoid neoplasms arising primarily from extranodal sites are infrequently reported. Urinary tract neoplasia can be diagnosed by urine evaluation in about one-third of canine cases, but the diagnosis of lymphoid neoplasia via urine evaluation is rarely reported. This case highlights the utility of ancillary diagnostics on urine for detection of lymphoid malignancies.

UNASSIGNED: The International Myeloma Working Group and College of American Pathologists recommend a 24-h urine collection to determine the Bence Jones protein (BJP) excretion level for monitoring treatment response in patients with multiple myeloma (MM). There are several issues related to sample collection and the method is prone to inaccuracy.
UNASSIGNED: This study compared measured 24-h to random urine collections for the quantitation of BJP in a South African population.
UNASSIGNED: Sixty-six patients with MM submitted random urine samples with their routine 24-h urine collection from April 2016 - March 2018. Measured 24-h urine BJP was compared to two estimated 24-h BJP excretions calculated as follows: Estimation 1 (E1): Estimated 24-h BJP (mg/24 h) = Urine BJP/Creatinine ratio (mg/mmol) × 10. Estimation 2 (E2): Estimated 24-h BJP (mg/24 h) = Urine BJP/Creatinine ratio (mg/mmol) × 15 mg/kg for women or × 20 mg/kg for men.
UNASSIGNED: Correlation of estimation equations E1 and E2 to the measured 24-h urine BJP was 0.893. Patients showed no difference in classification of treatment response using either the E1 or E2 estimation equations when compared to the measured 24-h urine BJP results.
UNASSIGNED: This study demonstrates that the estimated 24-h BJP shows a high degree of correlation with the measured 24-h BJP and can likely be used to monitor treatment response in South African patients with MM.

The detection and quantification of immunoglobulin free light chains in serum and urine is recommended for the diagnosis and monitoring of monoclonal gammopathies according to the guidelines of the International Myeloma Working Group (IMWG). Several tests are currently available in the clinical laboratory to detect and quantify free light chains but although quality, efficiency, and effectiveness have been improved, the results are still variable and poorly harmonized and standardized. The present review article wants to analyze these aspects, with a keen eye on techniques, such as mass spectrometry, that could replace in the practical clinical laboratory the current methods including Bence-Jones protein assay and free light chain immunoassays.

Leptomeningeal myelomatosis (LMM) is a fatal complication that occurs in < 1% of patients with multiple myeloma. Many patients with LMM present with neurologic symptoms referable to cranial neuropathies, while the manifestation of communicating hydrocephalus has been underrecognized. A Japanese man with Bence Jones protein-κ multiple myeloma developed fever and headache at age 54 years. He then became somnolent and went into a coma. Neuroimaging analyses identified rapidly progressive communicating hydrocephalus due to meningitis. He died 83 days after the onset of headache without any response to treatment at age 55 years. No symptoms or signs associated with cranial nerves were found during the course of illness. Postmortem examination revealed hydrocephalus and diffuse infiltration of myeloma cells into the subarachnoid space of the cerebrum, cerebellum, and brainstem. In addition, the interstitial tissue of the choroid plexuses was filled with myeloma cells. These myeloma cells were positive for CD156 and light chain κ. The Ki-67 labeling index in myeloma cells of the central nervous system (CNS) was 30-40%. Histopathological examination further revealed many myeloma cells on the surface of the lateral, third and fourth ventricles and at the area postrema of the medulla oblongata. Patients with LMM can develop an aggressive form of communicating hydrocephalus. Given that cerebrospinal fluid, produced by epithelial cells in the choroid plexuses of the ventricles, passes into the subarachnoid space through the third and fourth ventricles, myeloma cells may invade the CNS through the choroid plexuses.

We herein report a case of a combined crystalline light chain tubulopathy, podocytopathy, histiocytosis, and cast nephropathy in a patient with monoclonal gammopathy of renal significance (MGRS). A 66-year-old female with impaired renal function was referred to our department. Despite intravenous fluid resuscitation, the kidney function worsened progressively; thus, a kidney biopsy was performed. The kidney biopsy revealed light chain proximal tubulopathy (LCPT) with crystals, light chain crystal podocytopathy (LCCP), crystal-storing histiocytosis (CSH), and light chain cast nephropathy (LCCN). Of note, LCCP and CSH were diagnosed via electron microscopy. Serum and urine immunoelectrophoresis (IEP) revealed the presence of monoclonal Bence-Jones protein and free κ light chains. Bone marrow aspiration showed < 10% plasma cell proliferation. Thus, we had encountered a rare case in which a variety of kidney lesions were combined with MGRS. Most of the LCPT, LCCP, and CSH cases show monoclonal IgG κ, while our case showed Bence-Jones protein κ.

Testing urine for Bence Jones Protein (BJP) had been a time old procedure used for screening and monitoring of monoclonal disorders since its description. However, has poor sensitivity and despite advances in diagnostic methods of monoclonal disorders it is being continued to be requested in individuals for evaluation of myeloma or plasma cell disorders. Effective utilization and minimizing untimely or unnecessary investigations is important in the evaluation and management of any medical condition. Though, we are hard-wired during our education with some \"trigger\" or \"peculiar\" words that make us jump to actions too quickly, without comprehending the actual problem. Supporting evidence is presented to avoid reflexive use of multiple tests and utilize tests that improve utilization, reduce waste, and uphold the Choosing Wisely principles in providing optimal care to the patients.