Localized AL amyloidosis: A suicidal neoplasm?

Westermark, Per

doi:10.3109/03009734.2012.654861

Cited by 98 publications

(118 citation statements)

References 66 publications

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“…Localized amyloidosis (''amyloidoma'') involves more frequently single districts (such as larynx, ureter, skin, eyelid, and cardiac atria) and is characterized by the occurrence of foci of monoclonal plasma cells that synthesize amyloidogenic light chains. It is conceivable that giant cells are able to misfold these chains to AL amyloid fibrils [81]. Systemic amyloidosis, on the other hand, is structurally heterogeneous and includes three main types:…”

Section: Localized and Systemic Amyloidosismentioning

confidence: 99%

18F-FDG PET/CT: a review of diagnostic and prognostic features in multiple myeloma and related disorders

et al. 2014

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Conventional radiographic skeletal survey has been for many years the gold standard to detect the occurrence of osteolytic lesions in patients with multiple myeloma (MM). However, the introduction of more sensitive imaging procedures has resulted in an updated anatomic and functional Durie and Salmon "plus" staging system and has remarkably changed the diagnostic and prognostic approach to this tumor. It is now established that (18)fluorine-fluorodeoxyglucose ((18)F-FDG) positron-emission tomography (PET) combined with low-dose computed tomography (CT), shortly designated PET/CT, exhibits a higher screening and diagnostic sensitivity and specificity over the skeleton X-ray. In patients with monoclonal gammopathy of undetermined significance and in those with smoldering MM, PET/CT is consistently unable to detect focal and/or diffuse marrow abnormalities. Conversely, based on a systematic review of 18 studies comprising almost 800 MM patients, PET/CT was able to detect MM osteolytic lesions with a sensitivity of approximately 80-90% and a specificity of 80-100%. Importantly, a poor degree of concordance has also been emphasized between PET/CT and whole-body magnetic resonance imaging (WB-MRI) in that when both techniques were applied to the same patients, double-positive results were recorded in approximately 30% of the cases, but in the majority of them, a higher number of lesions were revealed with PET/CT than with MRI. Double-negative results, on the other hand, were found in about 22% of the patients. Because PET/CT is able to identify tumor foci throughout the body, it can be usefully applied to the study of solitary bone plasmacytoma and extra-medullary plasmacytoma: In both conditions, the detection of additional, previously overlooked sites of skeletal involvement would falsify the diagnosis of single-district disease, upstage the tumor, and therefore require a different therapeutic approach. In addition, although PET/CT is poorly sensitive to diffuse bone marrow infiltration, it can anticipate a site of impending fracture throughout the body and can discriminate old from new pathologic fractures. MRI should, however, be preferred when vertebral bodies are suspected to be involved and the risk of vertebral fracture is to be assessed. PET/CT is a sensitive and reliable procedure to evaluate the response to chemotherapy and/or radiotherapy, which is shown by a remarkable reduction and sometimes total disappearance of FDG accumulation in the involved bony structures, although these structures remain morphologically abnormal. Conversely, an increased focal uptake of FDG in apparent remission patients often precedes clinically overt relapse. PET/CT should be preferred to other imaging techniques to assess the remission status after autologous stem cell transplantation. In patients with primary and remission-induced non-secretory MM, the use of PET/CT may help to early detect single or multiple districts of focal non-secretory relapse. Osteonecrosis of the jaw, its location, and extent in MM patients receiving b...

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Section: Localized and Systemic Amyloidosismentioning

confidence: 99%

18F-FDG PET/CT: a review of diagnostic and prognostic features in multiple myeloma and related disorders

et al. 2014

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“…It has been proposed that the pathogenesis of localised AL amyloidosis may differ from that of the systemic type, as suggested by the fact that in localised amyloidosis κ light chains are more frequent than the λ form, in contrast to the systemic form, where λ chains constitute the overwhelming majority of cases. It is suggested that oligomeric assemblies of the produced immunoglobulin light chain are toxic to plasma cells [33]. Light chains that compose amyloid deposits are the same as those expressed by the lymphoma cells.…”

Section: Nodular Pulmonary Amyloidosismentioning

confidence: 99%

The lung in amyloidosis

et al. 2017

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Amyloidosis is a disorder caused by misfolding of autologous protein and its extracellular deposition as fibrils, resulting in vital organ dysfunction and eventually death. Pulmonary amyloidosis may be localised or part of systemic amyloidosis.Pulmonary interstitial amyloidosis is symptomatic only if the amyloid deposits severely affect gas exchange alveolar structure, thus resulting in serious respiratory impairment. Localised parenchymal involvement may be present as nodular amyloidosis or as amyloid deposits associated with localised lymphomas. Finally, tracheobronchial amyloidosis, which is usually not associated with evident clonal proliferation, may result in airway stenosis.Because the treatment options for amyloidosis are dependent on the fibril protein type, the workup of all new cases should include accurate determination of the amyloid protein. Most cases are asymptomatic and need only a careful follow-up. Diffuse alveolar-septal amyloidosis is treated according to the underlying systemic amyloidosis. Nodular pulmonary amyloidosis is usually localised, conservative excision is usually curative and the long-term prognosis is excellent. Tracheobronchial amyloidosis is usually treated with bronchoscopic interventions or external beam radiation therapy.

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“…Localized amyloidosis is typically seen in mucosal surfaces, such as the gastrointestinal tract (GIT), urinary tract, breasts, lungs and upper airways, as well as the skin [49]. It probably stems from a localized production of monoclonal LCs, and is thought to involve local amyloid phagocytic processing which provides the amyloidogenic properties of the LCs [50]. A diagnosis of localized AL amyloidosis is usually made after tissue sampling for symptoms induced by local deposition, although an incidental diagnosis can also occur.…”

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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Localized AL amyloidosis: A suicidal neoplasm?

Cited by 98 publications

References 66 publications

18F-FDG PET/CT: a review of diagnostic and prognostic features in multiple myeloma and related disorders

18F-FDG PET/CT: a review of diagnostic and prognostic features in multiple myeloma and related disorders

The lung in amyloidosis

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

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