Objective:
To present the clinicopathologic features and assess the factors related to the survival in light- chain amyloidosis (AL) patients.
Method:
All the patients with AL diagnosis being followed-up in the hematology department were recruited in the study. Clinicopathologic data were obtained. Factors related with overall survival (OS) including systemic inflammatory response markers were analyzed.
Results:
In 16 AL patients, the estimated OS was 58.6±10.8 months, with a-5-year- survival rate of 52.1%. While, 43.8% of the patients died during the study period. Gastrointestinal and respiratory complaints were the most frequent symptoms. Myocardial and renal biopsies were amyloid positive in 31.3% and 25% of the patients respectively. Myeloma was diagnosed in 18.8% and amyloid was positive in 31.3% of the bone marrow biopsies. There was no difference between surviving and deceased patients with respect to laboratory findings including systemic inflammatory markers. Only immunoglobulin M was significantly lower in the deceased patients and IgM was found to be the only factor independently associated with OS. Lower IgM levels were associated with decreased OS. An IgM value of 75.4 mg/dL was found as a cut-off value with a sensitivity and specificity of 71.4% and 66.7% respectively for the prediction of survival status.
Conclusion:
AL is a rare, progressive, systemic disease with a wide spectrum of clinical presentations. The disease most commonly presents with gastrointestinal and respiratory complaints. IgM level seems to be an independent predictor of survival and may be used as a prognostic marker.
Keywords: light chain amyloidosis, AL, Immunoglobulin M, neutrophil- to- lymphocyte ratio, platelet -to- lymphocyte ratio, systemic inflammatory response markers