Clinical manifestations and outcomes of systemic light chain amyloidosis: Chiang Mai University Hospital experience

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Jomchai Luechoowong
EKARAT RATTARITTAMRONG
Pokpong Piriyakhuntorn
Thanawat Rattanathammethee
Sasinee Hantrakool
Chatree Chai-Adisaksopha
Adisak Tantiworawit
Lalita Norrasethada

Abstract

Objectives: The study’s primary objective aimed to investigate clinical manifestations including symptoms, signs and laboratory findings among patients with systemic light chain (AL) amyloidosis. The secondary objective was to study clinical outcomes among patients with AL amyloidosis.


Material and Methods: This study employed a retrospective design. We included all patients who had received a diagnosis of systemic AL amyloidosis and were treated in Chiang Mai University Hospital from January 2002 to October 2018. Data were obtained through the electronic database and clinical records including demographic data, clinical presentation, laboratory characteristics, treatment and outcomes.


Results: A total of 28 patients were identified. The median age at diagnosis was 63 years old (range 39 to 85 years old) and 71.4% were male. The most common initial clinical presentation was dyspnea on exertion (42.8%). The two most frequent organs involved among our patients were the heart (60.7%) and kidney (53.5%). Most patients underwent chemotherapy (85.7%), mainly a melphalan-based regimen (87.5%). Of the treated patients, 25% had a hematologic response (very good partial response 12.5% partial response 12.5%). The mortality rate was 82.1% with a median follow-up time of 11 months. Univariate and multivariable Cox regression analysis revealed that presence of Bence Jones protein in the urine was a poor prognostic factor for survival.


Conclusion: The most common organ involved in systemic AL amyloidosis was the heart. Overall survival of patients with AL amyloidosis was poor and most cases had a poor response to treatment.

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