Renal manifestations of light chain associated diseases - Epidemiology and prognosis
An impaired renal function in light chain associated disorders may be caused by myeloma cast nephropathy (MCN) but also by AL-amyloidosis (AL-A) and monoclonal immundeposition disease (MIDD).
Patients and methods:
In a monocentric, retrospective analysis, patients suffering from multiple myeloma (MM) (n = 392) requiring medical therapy, AL-A (n = 53) or MIDD (n = 12) diagnosed between 1996 and 2008 were evaluated for renal insufficiency. The different patient cohorts were compared in terms of their clinical course and outcome.
Renal insufficiency in MM-, AL-A- or MIDD-patients at the time of diagnosis was found in 45,5 % of the patients. MCN, AL-A and MIDD were found in 68, 25 and 6 %, respectively. Dialysis dependency was seen in 17 % of MCN, in 8 % of AL-A and in 50 % of MIDD patients. Signs of hypervolemia were the leading symptoms in MIDD/AL-A. The time between the occurence of first symptoms and diagnosis was as long as 52 weeks in patients with AL-A. Patients with renal involvement showed a reduced median survival of 17 compared with 77 months in patients with a normal renal function. Median survival was only 12 months in AL-A compared to 21 months in MCN. Stabilization of renal function after chemotherapy occurred only in MCN. Multivariate Cox regression analysis showed impaired renal function as independent risk factor (Hazard-Ratio 2,88 [2,06-4,0]. In terms of survival and kidney function, autologous stem cell transplantation (ASCT) was beneficial for patients with renal involvement.
Renal insufficiency is an independent risk factor in MM, AL-A and MIDD. Specific therapy, especially ASCT may improve prognosis in patients with renal insufficiency and could stabilize renal function in MCN-patients.
Available from: Birgit Federmann
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ABSTRACT: History and Course of disease: We report on a 55-year-old man presenting with progressive hepatopathy for 6 months and recurrent cardiovascular symptoms for 12 months. He presented with reduced general health and progressive jaundice since one week. Investigations: Faeces was discolored, the urine, however dark. Troponin levels were increased. In the spontaneous urine analysis there was a non-selective proteinuria, serum protein electrophoresis showed hypogammaglobulinemia. The chest X-ray showed a pleural effusion on the left side, echocardiography demonstrated hypertrophic cardiomyopathy. The liver was enlarged. During his stay the condition of the patient deteriorated rapidly and he developed progressive renal failure. Eight days after admission hemodialysis was initiated and 13 days after admission the patient was transferred to the medical intensive care unit where he died. Autopsy and diagnosis: In the autopsy, deposits of kongo-red amyloid were present in all organs. Serum amyloid-p-component and kappa light chains could be found. In the bone marrow biopsy plasma cells were not increased indicating primary immunglobulin light chain (AL) amyloidosis. Conclusion: Prognosis of amyloidosis depends on the type of amyloid and extension of disease. Therefore, an early as possible diagnosis and classification are essential for an adequate therapy.
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