Stem cell transplantation in patients with autonomic neuropathy due to primary (AL) amyloidosis

Dingli, David; Tan, Tow Shung; Kumar, Shaji; Buadi, Francis K.; Dispenzieri, Angela; Hayman, Suzanne R.; Lacy, Martha Q.; Gastineau, Dennis A.; Hogan, William J.; Gertz, Morie A.

doi:10.1212/wnl.0b013e3181d55f4d

Cited by 30 publications

(19 citation statements)

References 22 publications

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“…Both liver measures can be abnormal in the presence of right heart failure. Basic testing for autonomic dysfunction includes orthostatic blood pressure measurement and evaluation for the loss of beat-to-beat variability on resting electrocardiogram [69]. …”

Section: Diagnosismentioning

confidence: 99%

Immunoglobulin Light-Chain Amyloidosis: From Basics to New Developments in Diagnosis, Prognosis and Therapy

et al. 2016

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Immunoglobulin amyloid light-chain (AL) amyloidosis is the most common form of systemic amyloidosis, where the culprit amyloidogenic protein is immunoglobulin light chains produced by marrow clonal plasma cells. AL amyloidosis is an infrequent disease, and since presentation is variable and often nonspecific, diagnosis is often delayed. This results in cumulative organ damage and has a negative prognostic effect. AL amyloidosis can also be challenging on the diagnostic level, especially when demonstration of Congo red-positive tissue is not readily obtained. Since as many as 31 known amyloidogenic proteins have been identified to date, determination of the amyloid type is required. While several typing methods are available, mass spectrometry has become the gold standard for amyloid typing. Upon confirming the diagnosis of amyloidosis, a pursuit for organ involvement is essential, with a focus on heart involvement, even in the absence of suggestive symptoms for involvement, as this has both prognostic and treatment implications. Details regarding initial treatment options, including stem cell transplantation, are provided in this review. AL amyloidosis management requires a multidisciplinary approach with careful patient monitoring, as organ impairment has a major effect on morbidity and treatment tolerability until a response to treatment is achieved and recovery emerges.

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Section: Diagnosismentioning

confidence: 99%

Immunoglobulin Light-Chain Amyloidosis: From Basics to New Developments in Diagnosis, Prognosis and Therapy

et al. 2016

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“…8,9 The extent of cardiac dysfunction is the main determinant of the prognosis of patients with AL amyloidosis, although symptomatic hepatic involvement and autonomic involvement also influence survival. 2,10 The epidemiology of AL amyloidosis in the United States has not been well characterized. A study by Kyle et al with data from 1950 to 1989 on the general population residing in Olmsted County, MN is the most comprehensive epidemiological study to date.…”

Section: Introductionmentioning

confidence: 99%

Epidemiology of AL amyloidosis: a real-world study using US claims data

et al. 2018

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“…23,24 This prospective observational study provides a platform upon which to base future studies of nutritional intervention in AL amyloidosis. It would be useful to determine whether nutritional status affects tolerability and toxicity of treatment, and hence treatment response.…”

Section: Discussionmentioning

confidence: 99%

A prospective study of nutritional status in immunoglobulin light chain amyloidosis

et al. 2012

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Stem cell transplantation in patients with autonomic neuropathy due to primary (AL) amyloidosis

Abstract: Patients with autonomic neuropathy secondary to AL amyloidosis can undergo autologous stem cell transplantation with relative safety. Autonomic neuropathy is an independent, adverse determinant of survival in these patients.

Cited by 30 publications

References 22 publications

Immunoglobulin Light-Chain Amyloidosis: From Basics to New Developments in Diagnosis, Prognosis and Therapy

Immunoglobulin Light-Chain Amyloidosis: From Basics to New Developments in Diagnosis, Prognosis and Therapy

Epidemiology of AL amyloidosis: a real-world study using US claims data

A prospective study of nutritional status in immunoglobulin light chain amyloidosis

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