232 A multidisciplinary case of multiple myeloma with cardiac and renal involvement: a look beyond amyloidosis

Aims Multiple myeloma (MM) is a malignant neoplasm with a frequent cardiac involvement characterized by restrictive cardiomyopathy. Kidney and thromboembolic complications are also described. Methods and results A 52-year-old woman with a history of kidney disease was admitted to nephrology department due to worsening of renal function and refractory hypertension. A bone marrow biopsy revealed the diagnosis of MM. A renal and fat pad biopsy with Congo red staining was performed but amyloid deposition was not observed. Increased cardiac enzymes suggested a cardiac involvement. Indeed, two intracardiac right-sided masses and massive pulmonary embolism were detected. Surgical cardiac intervention was promptly performed and a chemotherapy regimen was started. Meantime, a kidney biopsy revealed a light-chain deposition disease. Conclusions This case highlights that multiple organ involvement in patients with MM may result from a combination of paraprotein-dependent and -independent factors. Moreover, in patients with not acute massive pulmonary embolism and intracardiac right masses, surgical pulmonary embolectomy should be promptly performed to preserve RV function and to avoid prevent pulmonary hypertension development.

Case Report: Lenalidomide as a Second-Line Treatment for Bortezomib-Ineffective Nephrotic Syndrome Caused by LCDD: 2 Case Reports and a Literature Review

Background: Light-chain deposition disease (LCDD) is a rare systemic disorder characterized by the deposition of monoclonal light chains in organs. The kidney is a prominent target of light-chain deposition, with a median time to end-stage renal disease (ESRD) of 2.7 years and 5-year ESRD-free survival of 37%. The therapeutic management of LCDD remains ill-defined. In addition to bortezomib-based therapy as first-line therapy, the effect of lenalidomide on LCDD is rarely reported.Case Presentation: This study describes two male LCDD patients in their 60s with nephrotic syndrome and moderately impaired renal function. One patient had monoclonal IgGλ with underlying MGRS, and another had monoclonal IgGκ with underlying monoclonal gammopathy that developed into symptomatic MM during follow-up. The hallmarks of this disease were consistent with previous reports. Both patients initially received BCD therapy, but no hematological response was observed. Consequently, the nephrotic syndrome was refractory. Sequential Rd therapy was initiated, and partial hematological response and nephrotic remission were observed in the IgGλ patient but absent in the IgGκ patient.Conclusion: Limited reports have demonstrated the effect of lenalidomide in LCDD. We report the outcome of lenalidomide in two cases of bortezomib-resistant LCDD. This treatment might be a beneficial supplement for those unresponsive or intolerant to bortezomib in LCDD, but the effect should be prospectively investigated.