Fibrillary glomerulonephritis associated with monoclonal gammopathy of undetermined significance showing lambda-type Bence Jones protein

A 79-year-old woman was admitted to our hospital because of leg edema due to a nephrotic syndrome. Urinary and serum immunoelectrophoresis showed positive for the lambda type of Bence Jones protein. A bone marrow aspiration test revealed mild plasmacytosis (6.4% of the total cells). These findings confirmed her diagnosis of monoclonal gammopathy of undetermined significance (MGUS). Her renal biopsy specimen revealed mild mesangial cell proliferation and an increase in the mesangial matrix. Immunofluorescence studies showed positive staining for IgG, IgA, C3, and kappa and lambda light chains in the capillary wall and mesangium area. Electron microscopy showed that the electron deposits in the thickened basement membrane were formed by randomly arranged 16- to 18-nm nonbranching fibrils. A Congo red stain for amyloid was negative. These findings corresponded with the diagnosis of fibrillary glomerulonephritis. Therefore, this case showed a rare combination of fibrillary glomerulonephritis and MGUS.     

Monoclonal gammopathy of undetermined significance: an update for nephrologists

Screening for a monoclonal protein is a common part of the assessment of patients presenting with a renal injury. While in the settings of acute kidney injury, chronic kidney disease and proteinuria monoclonal proteins can be associated with significant pathologies such as cast nephropathy, amyloidosis, and light chain deposition disease, they can also be an unrelated finding. The purpose of this review is to provide the nephrologist with an update to the diagnostic assessment and risk stratification of monoclonal proteins to avoid unnecessary investigation and monitoring of those patients with low-risk monoclonal gammopathies.     

Monoclonal gammopathy of undetermined significance,multiple myeloma,and osteoporosis

The finding of monoclonal gammopathy of undetermined significance (MGUS) is not infrequent during an evaluation for osteoporosis or a fracture. In most cases, the diagnosis is MGUS, whose prevalence increases with age. Although the impact of MGUS on bone mineral density, bone remodeling, and the fracture risk remains unclear, this asymptomatic hematological disorder may constitute a risk factor for osteoporosis. Furthermore, each year, 1% of patients with MGUS progress to multiple myeloma, a disease whose pathophysiology and association with bone loss and pathological fractures are increasingly well understood. Osteoporotic fractures, although probably common in myeloma patients, are less likely to be recognized. Here, we discuss the pathophysiology of myeloma and MGUS and their impact in terms of bone mineral density, osteoporotic fractures, and bone turnover markers.     

Neoplastic and non‐neoplastic complications of solid organ transplantation in patients with preexisting monoclonal gammopathy of undetermined significance

Monoclonal gammopathy of undetermined significance (MGUS) occurs in 3–7% of the elderly population, with higher prevalence in renal failure patients, and is associated with a 25‐fold increased lifetime risk for plasma cell myeloma (PCM), also known as multiple myeloma. Using the California State Inpatient, Emergency Department, and Ambulatory Surgery Databases components of the Healthcare Cost and Utilization Project (HCUP), we sought to determine whether patients with MGUS who undergo solid organ allograft (n = 22 062) are at increased adjusted relative risk (aRR) for hematologic malignancy and other complications. Among solid organ transplant patients, patients with preexisting MGUS had higher aRR of PCM (aRR 19.46; 95% CI 7.05, 53.73; p < 0.001), venous thromboembolic events (aRR 1.66; 95% CI 1.15, 2.41; p = 0.007), and infection (aRR 1.24; 95% CI 1.06, 1.45; p = 0.007). However, when comparing MGUS patients with and without solid organ transplant, there was decreased aRR for PCM with transplant (aRR 0.34; 95% CI 0.13, 0.88; p = 0.027), and increased venous thromboembolic events (aRR 2.33; 95% CI 1.58, 3.44; p < 0.001) and infectious risks (aRR 1.44; 95% CI 1.23, 1.70; p < 0.001). While MGUS increased the risk of PCM overall following solid organ transplantation, there was lower risk of PCM development compared to MGUS patients who did not receive a transplant. MGUS should not preclude solid organ transplant.     

Successful management of polyneuropathy associated with IgM gammopathy of undetermined significance with antibody-based immunoadsorption

Peripheral polyneuropathies associated with monoclonal IgM gammopathy of undetermined significance often have a progressive course and optimal treatment has not been established. We report on a patient diagnosed with polyneuropathy associated with benign IgM gammopathy, who was successfully treated with antibody-based immunoadsorption only. The neurological symptoms of the patient improved continuously over six months of treatment. Controlled trials should be performed to define this indication for antibody-based immunoadsorption therapy.     

Dense deposit disease in association with monoclonal gammopathy of unknown significance

Several renal pathologic entities have been reported in patientswith lymphoplasmacytic disorders with their typical excess immunoglobulinproduction. We report dense deposit disease in a patient whowas discovered to have an IgG kappa monoclonal protein withoutclinical evidence of underlying lymphoplasma cytic malignancyduring investigation for chronic renal failure with associatednephrotic range proteinuria. This case is unusual since densedeposit disease occurs only rarely in older patients and hasnot been reported in association with monoclonal gammopathyof unknown significance. Because of the diversity of renal lesionsassociated with lymphoplasmacytic disorders, renal biopsy isnecessary to assess the type of renal lesion in this patientpopulation.     

Why renal biopsy is crucial in monoclonal gammopathy of renal significance (MGRS)

International Urology and Nephrology -     

IgG monoclonal gammopathy associated with lymphoproliferative disorders.

The association of IgG monoclonal gammopathy with lymphocytic lymphoma and chronic lymphocytic leukaemia is reported. Although rare, such immunoglobulin synthesis may have prognostic importance and is of value in monitoring the effect of therapy. The morphological features which should draw attention to this pattern of protein abnormality are briefly reviewed. This finding emphasises the histogenesis of these tumours from the immune system and questions their classification on a purely morphological basis.     

The presence of papillary features in thyroid nodules diagnosed as atypia of undetermined significance or follicular lesion of undetermined significance increases cancer risk and should influence treatment

Background

The objective of this study was to evaluate the influence of papillary features on risk of malignancy (ROM) within the Atypia of Undetermined Significance or Follicular Lesion of Undetermined Significance (AUS-FLUS) Bethesda System for Reporting Thyroid Cytopathology (BSRTC) diagnostic category.

Myeloma and monoclonal gammopathy of uncertain significance associated with acquired von Willebrand's syndrome. Seven new cases with a literature review

OBJECTIVES: Acquired von Willebrand's syndrome (AvWS) is an uncommon complication of monoclonal gammopathy of uncertain significance (MGUS) or myeloma. We investigated clinical and laboratory test abnormalities, pathophysiological hypotheses, and treatment options in this poorly known condition. PATIENTS: Five patients with MGUS and two with myeloma who met classic criteria for acquired von Willebrand's syndrome were included in this retrospective study. RESULTS: Acquired von Willebrand's syndrome was diagnosed before the gammopathy in five of the seven patients. The severity of the bleeding events was chiefly dependent on the degree of von Willebrand's factor deficiency and on the presence or absence of gastrointestinal tract angiodysplasia. Bleeding event severity was similar in patients with nonmalignant and malignant disease. An antibody that inhibited von Willebrand's factor was detected in all seven patients. Clotting returned to normal after treatment of the malignancy in one of the two patients with myeloma. In patients with MGUS, treatment is warranted only when bleeding occurs or before surgery. Von Willebrand's factor concentrates were of limited efficacy because of their short half-life. Intravenous immunoglobulins had a longer-lasting effect (about 3 weeks); this treatment was used on a regular basis in two patients with recurrent bleeding. CONCLUSIONS: A diagnosis of von Willebrand's syndrome in adulthood should prompt a search for a monoclonal gammopathy. In patients with gammopathies, simple clotting tests ensure the diagnosis of acquired von Willebrand's syndrome.