Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0026764 (multiple myeloma)
 36,148 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors deal with the clinicopathology of the renal, alterations in light-chain disease in connection with 6 cases. The disease was recognized by the monotype (in 5 cases kappa, in 1 case lambda) immunoreactivity of the light-chain paraprotein deposited in the basal membranes of the renal tissue. Electron microscopic examinations proved the fine-granulated, electrodense character of the paraprotein. Multiple myeloma was found in 3 cases and plasma cell dyscrasia of non-tumorous characteristic in 3 cases in the background of the deposition. The renal involvement appeared clinically in the picture of proteinuria without nephrosis syndrome and in progressing azotemia. Chronic renal insufficiency developed during some months in 5 patients. Morphologically renal impairment manifested in interstitial fibrosis, tubular atrophy and ateriolar hyalinosis was seen. These were associated with different glomerular alterations, for instance in 3 cases with nodular glomerulosclerosis. In 1 patient with plasma cell dyscrasia of non-tumorous characteristic nodular glomerulosclerosis and semilunar formation was observed in 56% of the glomeruli. In an other patient with myeloma the simultaneous existence of cylinder nephropathy and light-chain nephropathy was demonstrated. Both observations are unusual phenomena in plasma cell dyscrasia.
 ...
PMID:[Light-chain nephropathy]. 211 26

Thirty-two patients with primary amyloidosis (AL) either due to multiple myeloma or without apparent cause were studied. The most common manifestations were carpal tunnel syndrome and nephrosis. Rectal biopsy specimens showed amyloid infiltrates in 65% (15 of 23) of the patients studied, but virtually all biopsies of normal skin were negative. lambda Light chains were present in 75% (21 of 28) of patients with a monoclonal gammopathy. The median survival time was 14 months after histologic diagnosis, following a median delay of seven months from the recognition of disease by a physician. An earlier diagnosis would have been possible in most of the patients if the initial findings of amyloidosis had been recognized and evaluated definitively. The prognosis remains poor, because effective therapy is not available.
 ...
PMID:Amyloidosis in multiple myeloma or without apparent cause. 643 54

An eighty-one-year-old male with lower leg edema and proteinuria was diagnosed as having nephrotic syndrome and was hospitalized for a detailed examination. Kidney biopsy and immunostaining revealed nodular glomerulosclerosis and deposition of lambda chains, respectively. Because these findings indicate the occurrence of light chain deposition disease (LCDD), the underlying disease was found to be multiple myeloma BJ-lambda. After the administration of melphalan and prednisolone, followed by further addition of zoledronic acid, the patient's nephrosis remitted. However, renal dysfunction gradually deteriorated further and hemodialysis was instituted. He eventually died of gastrointestinal bleeding and biliary infection. The period of time from the initial diagnosis to death was thirty months. Autopsy revealed pervasive infiltration of plasma cells and light chain deposition in multiple organs. The uncontrollable gastrointestinal bleeding appears to have been caused by light chain deposition in the vascular walls of a bile duct. Although medical treatment for elderly LCDD cases depends on chemotherapy alone, it is difficult to obtain a complete remission with melphalan and prednisolone, according to the literature. Reports on the validities of biological agents, such as bortezomib, are beginning to appear, and accumulation of further therapeutic experience is eagerly awaited.
 ...
PMID:[Autopsy case of light chain deposition disease presented by nephrotic syndrome]. 2346 Dec 12

Cholemic nephrosis represents a spectrum of renal injury from proximal tubulopathy to intrarenal bile cast formation found in patients with severe liver dysfunction. However, the contribution of this diagnosis has been largely forgotten in the modern literature. To more precisely define this, we conducted a clinicopathologic study of 44 subjects (41 autopsies and 3 renal biopsies) from jaundiced patients at the University of Chicago. Of these, 24 patients had bile casts with involvement of distal nephron segments in 18 mild cases and extension to proximal tubules for 6 severe cases. Eleven of 13 patients with hepatorenal syndrome and all 10 with cirrhosis (due to alcoholism) had tubular bile casts. These casts significantly correlated with higher serum total and direct bilirubin levels, and a trend toward higher serum creatinine, AST, and ALT levels. Bile casts may contribute to the kidney injury of severely jaundiced patients by direct bile and bilirubin toxicity, and tubular obstruction. Both mechanisms are analogous to the injury by myeloma or myoglobin casts. Accounting for the presence of renal bile casts provides a more complete representation of the renal injury that can occur in this unique clinical setting. Thus, bile cast nephropathy is an appropriate term for the severe form of injury observed in the spectrum of cholemic nephrosis. Additional studies are needed to establish the significance of this parameter for patient management in different clinical settings.
...
PMID:Bile cast nephropathy is a common pathologic finding for kidney injury associated with severe liver dysfunction. 2458 95

Acute kidney injury (AKI) is a major complication in patients with acute liver failure and chronic liver disease. Hemodynamic changes appear to be the principal alterations in these conditions, therefore there should be no known structural abnormalities responsible for AKI. On the other hand, several authors have published data on structural changes known as bile cast nephropathy or cholemic nephrosis, which basically consist of the presence of bile casts in tubular lumen analogous to those observed in myeloma. Although these findings are well documented, there is a lack of reproducibility by other authors. This paper aims to discuss, through evidence-based medical literature, the role of biliary salts on kidney injury development.
...
PMID:Do Biliary Salts Have Role on Acute Kidney Injury Development? 2625 79

Here we report a rare case of Merkel cell carcinoma complicated with nephrosis and malignant lymphoma. A 79-year-old male, who had undergone rectectomy due to colorectal cancer about 10 years previously, was diagnosed as Merkel cell carcinoma of the left ear lobe with lymph node metastases. Tumor resection and lymph node dissection were performed. A year later, follow-up PET-CT revealed a small hot spot at the ileocecum without apparent tumor formation based on examination by colonoscopy. The patient received 56 Gy of radiation. Two months later, he developed new-onset nephrosis followed by renal failure, and was referred to our hospital (Cr 4.26 mg/dL, UA 13.5 mg/dL, Alb 2.1 g/dL). Further examination negated the possibility of vasculitis, collagen disease, or myeloma kidney. Since his renal function continued to decline, causing uremic symptoms, he was hospitalized and underwent hemodialysis soon after referral. Abdominal CT scan revealed an ileocecal mass with multiple abdominal lymphadenopathy, which was later diagnosed as diffuse large B-cell lymphoma (stage IV) by tumor biopsy. Corticosteroid therapy (prednisolone 60 mg/day) was soon initiated with no response. Local skin redness and blister formation at the left shoulder emerged gradually, which strongly suggested a local recurrence of Merkel cell carcinoma. Despite the use of rituximab, the patient's general condition deteriorated without any sign of recovery. Three months after the start of dialysis, we discontinued dialysis therapy due to his poor health status, and eventually he died of cachexia. Autopsy revealed triple cancers: rectal cancer, Merkel cell carcinoma, and malignant lymphoma. In addition to the case report, we will summarize and discuss former similar case reports in the literature.
 ...
PMID:[Merkel cell carcinoma of the ear lobe complicated with nephrosis syndrome and malignant lymphoma: a case report]. 2681 68