ABSTRACT

Monoclonal immunoglobulin deposits can be organized as fibrils, microtubules, crystals or casts, or be unorganized, as granular deposits. The two main prototypic conditions caused by highly organized fibrillar deposits or amorphous aggregates are light chain amyloidosis (AL) and light chain deposition disease (LCDD), respectively. Monoclonal immunoglobulin deposits can be organized as fibrils, microtubules, crystals or casts, or be unorganized, as granular deposits. The two main prototypic conditions caused by highly organized fibrillar deposits or amorphous aggregates are light chain amyloidosis (AL) and light chain deposition disease (LCDD), respectively. The overall survival of patients with AL and LCDD is similar, the median value being 45.3 and 49.2 months, respectively: 24% of AL and 31% of LCDD patients are alive at 8 years. Death in AL patients is most commonly due to heart involvement, while cachexia and heart involvement accounted for 44% and 11% of deaths, respectively, in LCDD.