Detection of abnormal plasma cells in bone marrow contributes to the diagnosis of primary systemic light chain amyloidosis-review

Primary systemic light chain amyloidosis or immunoglobulin light-chain amyloidosis (AL) is the most common type of systemic amyloidosis.AL is a proteotoxic clonal plasma cell disease, a hematological malignancy, characterised by overproduction of immunoglobulin light chains that form characteristic abnormally folded and aggregated, insoluble fibrillar deposits in various organs, including kidneys, heart, liver, and autonomic and peripheral nerves, etc, these processes lead to organ dysfunction and death. Systemic amyloidosis have various types with different causes, thereby its clinical diagnosis and treatment are more difficult. Recent developments on studies that have significantly aided the management of patients with AL include diagnostic techniques for definitive typing of amyloid deposits by using flow cytometry and immunophenotype analysis. These methods can detect abnormalities of bone marrow plasma cell clones, such as CD38(+), CD138(+), CD56(+), CD19(-) in AL patients. The monitoring abnormal plasma cells with immunoglobulin light chain restriction and abnormal plasma cell phenotypic characteristics contributes to the early diagnosis of AL and detection of minimal residual disease after treatment, which greatly improved AL treatment and prognosis. In this review the diagnosis and typing, clinical characteristics, flow cytometry, immunophenotyping of bone marrow cells, immunoglobulin light chain restriction and phenotypic characteristics of abnormal plasma cells of AL are briefly summarized.PubMed051251-52