Hematologie

λ-lichtketens en polyserositis zonder hypoproteïnemie wijzen op amyloïdose bij multipel myeloom

Een retrospectieve cohortstudie onder 359 patiënten met nieuw gediagnosticeerd multipel myeloom vergeleek klinische kenmerken van patiënten met lichte-ketenamyloïdose (AL) met patiënten zonder AL. Zeventien procent ontwikkelde AL, waarvan dertig procent cardiale amyloïdose.

Multivariaat analyse toonde aan dat λ-lichtketens (OR = 2,40) en polyserositis zonder hypoproteïnemie (OR = 7,66) onafhankelijke risicofactoren zijn voor AL, terwijl patiënten met cardiale betrokkenheid vaker congestief hartfalen, ernstige cardiovasculaire gebeurtenissen en verhoogde NT-proBNP- en hs-TnT-waarden vertoonden.

ECG-afwijkingen zoals lage voltage en pseudo-infarctpatronen waren specifiek voor de AL-groep en ondersteunen vroegtijdige screening op amyloïdose bij multipel myeloom.

Abstract (original)

OBJECTIVE: To analyze the clinical characteristics of patients with multiple myeloma (MM) complicated with light chain amyloidosis (AL), particularly those with cardiac amyloidosis (CA), in order to provide evidence for early identification. METHODS: Clinical data were retrospectively collected from 359 newly diagnosed MM patients at the First Affiliated Hospital of Soochow University from August 2017 to December 2023. Based on histopathological results, patients were grouped to compare the clinical characteristics between the multiple myeloma with amyloid light-chain (MM-AL) group and the multiple myeloma without amyloid light-chain (MM without AL) group, as well as between the cardiac involvement and non-cardiac involvement subgroups within the MM-AL cohort. RESULTS: MM-AL patients accounted for 19.5% (70/359), of whom 30.0% had cardiac involvement. Compared with the MM without AL group, the MM-AL group had a higher proportion of λ light chain type (65.7% vs. 45.0%), a higher proportion of frail patients (45.7% vs. 29.1%), a lower proportion of DS stage III (84.3% vs. 93.4%), fewer patients presenting with bone pain as the initial symptom (45.7% vs. 69.2%), and higher proportions of patients with heart failure (12.8% vs. 4.2%) and non-hypoproteinemia polyserositis (27.2% vs. 5.9%) (all P < 0.05). Electrocardiogram abnormalities (low voltage, pseudo-infarction) were observed only in the MM-AL group. Compared with those without cardiac involvement, MM-CA patients had a higher proportion of light chain type (47.6% vs. 18.4%), a higher proportion of congestive heart-failure at onset (33.3% vs. 4.1%), a higher incidence of major adverse cardiovascular events during treatment (33.3% vs. 2.0%), and significantly elevated levels of NT-proBNP and hs-TnT, as well as a higher incidence of electrocardiogram abnormalities (all P < 0.05). Multivariate analysis showed that non-hypoproteinemia polyserositis (OR =7.66, P < 0.001) and λ light chain type (OR =2.40, P =0.017) were independent risk factors for MM complicated with AL. In terms of cytogenetics, the proportion of high-risk cytogenetic abnormalities was lower in MM-CA patients compared with those without cardiac involvement (14.3% vs. 36.7%, P =0.047). CONCLUSION: Patients with MM complicated by AL present with distinct clinical and cytogenetic features. The presence of λ light chain type and non-hypoproteinemic polyserous effusions are independent risk factors. Electrocardiographic findings of low voltage and pseudoinfarction patterns are suggestive of early recognition of AL and cardiac involvement.

Dit artikel is een samenvatting van een publicatie in Zhongguo shi yan xue ye xue za zhi. Voor het volledige artikel, alle details en referenties verwijzen wij u naar de oorspronkelijke bron.

Lees het volledige artikel

DOI: 10.19746/j.cnki.issn1009-2137.2026.03.019