Clinical characteristics and prognostic factors in multiple myeloma patients with light chain deposition disease

Abstract

Light chain deposition disease (LCDD) is characterized by monotypic immunoglobulin depositions which will eventually lead to loss of organ function if left untreated. While the kidney is almost always affected, the presence and degree of LCDD in other organs vary. Ten to thirty percent of LCDD patients have underlying Multiple Myeloma (MM), yet outcome and prognostic markers in this particular patient group are still lacking. Here, we analyzed 69 patients with MM and biopsy proven LCDD and report on renal and extra-renal involvement and its impact on prognosis as well as renal response depending on hematologic response. Coexisting light chain diseases such as AL amyloid and cast nephropathy were found in 30% of patients; those with LCDD and concurrent amyloid tended to have shorter survival. Cardiac involvement by LCDD was seen in one-third of our patients and was associated with shorter overall survival; such patients also had a significantly higher risk of treatment-related mortality (TRM) after stem cell transplant (SCT) compared to LCDD patients without cardiac involvement. This study highlights that MM patients with LCDD present with different clinical features compared to previously reported LCDD cohorts. Rapid initiation of treatment is necessary to prevent progressive renal disease and worse outcome. Coexisting light chain diseases and cardiac involvement are more common than previously reported and confer worse clinical outcome, emphasizing the need for careful patient careful patient evaluation and treatment selection.

Long term outcomes in monoclonal gammopathy of renal significance

“…Haematological response was reported in the study using the borrowed 2006 International Myeloma Working Group (IMWG) response criteria for myeloma (Durie et al, 2006). Twelve individual patients achieved ≥VGPR during the study and although no effect of haematological response on RS was detected, previous studies have demonstrated that achieving excellent haematological response in MGRS patients is associated with an improved RS (Sayed et al, 2015;Kourelis et al, 2016;Mohan et al, 2017). Importantly, there are no existing MGRS response criteria and the multiple myeloma response criteria do not include renal response as part of the response measures.…”

Bacteremias following autologous stem cell transplantation for multiple myeloma: Risk factors and outcomes

“…High‐dose chemotherapy and ASCT still remains one of the frontline treatments of MM . ASCT is associated with a transplant‐related mortality of 1%‐10% . The risk of infection is highest during pre‐engraftment phase which is associated with neutropenia and mucositis …”

“…1 ASCT is associated with a transplant-related mortality of 1%-10%. 5 The risk of infection is highest during pre-engraftment phase which is associated with neutropenia and mucositis. 6 The microbial etiology of febrile neutropenia has changed over the past several decades.…”

Pulmonary Vascular Disease Due to Plasma Cell Dyscrasia

“…18 Patients with LCDD may have coexistent MM (10%-30%) and AL amyloidosis (17%). 19 Light chain deposition disease with cardiac involvement (33%) has overall poor survival and is related to increased transplant-related mortality. Only 18% of patients had LV ejection fraction less than 50%, and most frequent manifestations are atrial arrhythmias, sinus bradycardia, and diastolic dysfunction.…”