Kappa L-chain primary nodular lung amyloidosis: a case report

Moscatelli, Barbara; Gb, Capoccetta; Anselmi, Pasquale; M, Di Girolamo; Campagna, Domenico

doi:10.3109/13506129.2011.574354045

Cited by 2 publications

(4 citation statements)

References 10 publications

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“…In primary AL amyloidosis, amyloid light-chain protein derived from monoclonal gammaglobulin light chains (either lambda or kappa) is deposited. This type is caused by preexisting multiple myeloma, Waldenström macroglobulinemia or progresses to one [ 1 , 2 , 4 , 5 , 12 ]. Regardless of the specific fibril type, amyloidosis can be divided into localized and systemic forms.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary amyloidosis mimicking interstitial lung disease and malignancy - A case series with a review of a pulmonary patterns

Zimna¹,

Sobiecka²,

Langfort³

et al. 2021

Respiratory Medicine Case Reports

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Background Amyloidosis is an uncommon condition, which results from accumulation of misfolded extracellular insoluble protein in tissues and organs of the body, causing its damage and dysfunction. Histologically, after staining with Congo red, the amyloid deposits show an apple-green birefringence under polarized light microscope. Amyloidosis can affect all organ systems and is classified into hereditary or acquired, localized or systemic. Respiratory involvement occurs in 50% of the patients with amyloidosis and it may take tracheobronchial, nodular parenchymal, diffuse alveolar septal and lymphatic forms. Methods We report four cases of pulmonary amyloidosis. A female patient with localized form of tracheobronchial and nodular parenchymal pulmonary amyloidosis, which was initially misdiagnosed as sarcoidosis. A male patient who was referred to our department for further evaluation of multiple tumors in lungs accompanied by mediastinal lymphadenopathy, liver and peritoneal tumors. A male patient with suspect of lung malignancy. A male patient with diagnosed idiopathic pulmonary fibrosis and the possibility of malignancy. Results All the diagnoses were established by demonstration of amyloid protein in tissue specimens obtained in transbronchial or open lung biopsies. Conclusions Due to its nonspecific clinical and radiological findings, amyloidosis can often mimic other diseases and should be considered as one of the differential diagnoses. In order to confirm the diagnosis, proving the presence of amyloid deposition with positive Congo red staining in respiratory specimen is mandatory.

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Section: Discussionmentioning

confidence: 99%

Pulmonary amyloidosis mimicking interstitial lung disease and malignancy - A case series with a review of a pulmonary patterns

Zimna¹,

Sobiecka²,

Langfort³

et al. 2021

Respiratory Medicine Case Reports

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“…In most cases, the detection of typical regular round lesions in nodular pulmonary amyloidosis is an accidental finding of (particularly) imaging methods (mostly chest radiography) when the lungs are being examined for a different reason. It is also frequently discovered by chance during an autopsy [ 8 , 12 , 13 ]. Since pulmonary amyloidosis cannot be distinguished solely by the appearance of nodules provided by imaging techniques, a biopsy is required to accurately determine the pathological changes.…”

Section: Discussionmentioning

confidence: 99%

“…The detection of apple green birefringence in polarised light after Congo red staining is the most notable and typical finding. Further analysis is performed, primarily using immunohistochemical analyses, to accurately determine the type of amyloidosis (based on the composition of protein fibrils) [ 4 , 12 , 13 ]. The AL type is the most common finding in the nodular pulmonary form of amyloidosis.…”

Section: Discussionmentioning

confidence: 99%

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A unique case of AH-dominant type nodular pulmonary amyloidosis presenting as a spontaneous pneumothorax: a case report and review of the literature

Skopelidou,

Hurník,

Tulinský

et al. 2023

Pathol. Oncol. Res.

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Amyloidosis is a rare metabolic disorder primarily brought on by misfolding of an autologous protein, which causes its local or systemic deposition in an aberrant fibrillar form. It is quite rare for pulmonary tissue to be impacted by amyloidosis; of the three forms it can take when involving pulmonary tissue, nodular pulmonary amyloidosis is the most uncommon. Nodular pulmonary amyloidosis rarely induces clinical symptoms, and most often, it is discovered accidentally during an autopsy or via imaging techniques. Only one case of nodular pulmonary amyloidosis, which manifested as a spontaneous pneumothorax, was found in the literature. In terms of more precise subtyping, nodular amyloidosis is typically AL or mixed AL/AH type. No publications on AH-dominant type of nodular amyloidosis were found in the literature. We present a case of an 81 years-old male with nodular pulmonary AH-dominant type amyloidosis who presented with spontaneous pneumothorax. For a deeper understanding of the subject, this study also provides a review of the literature on cases with nodular pulmonary amyloidosis in relation to precise amyloid fibril subtyping. Since it is often a difficult process, accurate amyloid type identification is rarely accomplished. However, this information is very helpful for identifying the underlying disease process (if any) and outlining the subsequent diagnostic and treatment steps. Even so, it is crucial to be aware of this unit and make sure it is taken into consideration when making a differential diagnosis of pulmonary lesions.

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Kappa L-chain primary nodular lung amyloidosis: a case report

Cited by 2 publications

References 10 publications

Pulmonary amyloidosis mimicking interstitial lung disease and malignancy - A case series with a review of a pulmonary patterns

Pulmonary amyloidosis mimicking interstitial lung disease and malignancy - A case series with a review of a pulmonary patterns

A unique case of AH-dominant type nodular pulmonary amyloidosis presenting as a spontaneous pneumothorax: a case report and review of the literature

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