Case report

Sun, Linge; Zhang, Lei; Hu, Wenlu; Li, Tian-Fang; Liu, Shengyun

doi:10.1097/md.0000000000008771

Cited by 7 publications

(2 citation statements)

References 18 publications

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“…High-risk patient populations such as MGUS patients should be closely monitored for relative markers such as NT-proBNP and albuminuria [29]. Moreover, cases of AL amyloidosis misdiagnosed as scleroderma [30] or jejunal mass [31] have been reported because of the nonspecific symptoms. In similar cases, diagnosis and therapy have been significantly delayed.…”

Section: Diagnostic Procedures and Differential Diagnosismentioning

confidence: 99%

Light-Chain Amyloidosis: The Great Impostor

Stefani,

Kouvata,

Vassilopoulos

2023

Life

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Light-chain amyloidosis (AL) is a disease of protean manifestations due to a wide spectrum of organs that can be affected. The disorder is caused by the deposition of an extracellular amorphous material, the amyloid, which is produced by malignant plasma cells. The latter usually reside in the bone marrow; plasma cell infiltration is often low, in sharp contrast to what we observe in multiple myeloma. The disease may run below the physician’s radar for a while before clinical suspicion is raised and targeted tests are performed. In this short review, we try to answer most of the questions that a practicing physician may ask in a relative clinical setting. The text is formed as a series of reader-friendly questions that cover the subject of AL amyloidosis from history to current therapy.

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Section: Diagnostic Procedures and Differential Diagnosismentioning

confidence: 99%

Light-Chain Amyloidosis: The Great Impostor

Stefani,

Kouvata,

Vassilopoulos

2023

Life

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“…При поражении слюнных и сальных желез пациентов бес покоят ксерофтальмия и ксеростомия, возможно раз витие дисгевзии (вкус соли во рту или другие нарушения чувствительности). Возможно развитие склероподоб ного синдрома и артропатии [31,32].…”

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Difficulties in diagnosis of primary AL-amyloidosis

et al. 2021

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Objective of the study: analysis of AL-amyloidosis (AL-A) diagnostics in real clinical practice and to determine the main approaches for the earlier detection of this disease.Materials and methods. A retrospective analysis of medical records of 34 patients with newly diagnosed AL-A.Results. The median time from first symptoms appearance to the diagnosis was more than 2 years. Most often, the pathological process in AL-A involves the kidneys, heart and gastrointestinal tract; moreover, at the time of diagnosis, most patients already have an injury of 2 or more organs. In half of the patients, a biopsy of the damaging organ was performed to verify the diagnosis; according to our data, histological examination of “easily accessible” locus not less informative. Symptoms characteristic of amyloidosis, such as periorbital purpura or macroglossia, are observed in a small part of patients and refer to late manifestations of the disease.Conclusion. The versatility of AL-A manifestations leads to a late diagnosis, which affects overall survival. The main signs were highlighted that should alert doctors in relation to this pathology.

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Significantly prolongedPTandAPTTcould indicate a wide spectrum of clinical manifestations in three patients with plasma cell disorders

Tang

Cao

et al. 2018

Int J Lab Hematology

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Case report

Cited by 7 publications

References 18 publications

Light-Chain Amyloidosis: The Great Impostor

Light-Chain Amyloidosis: The Great Impostor

Difficulties in diagnosis of primary AL-amyloidosis

Significantly prolongedPTandAPTTcould indicate a wide spectrum of clinical manifestations in three patients with plasma cell disorders

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