Multifocal primary amyloidosis of the airways: Case report and review of the literature

Lang, SM; Täuscher, D.; Fuller, Julie; Müller, Andréas; Schiffl, Helmut

doi:10.1016/j.rmcr.2015.05.004

Cited by 7 publications

(5 citation statements)

References 22 publications

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“…The other three patients with localised amyloidosis were treated with external beam radiation therapy. Patient #6 already got published as a case report [29].…”

Section: Patients Treated With Amyloidosis Targeting Therapymentioning

confidence: 99%

Local vs. systemic pulmonary amyloidosis—impact on diagnostics and clinical management

et al. 2018

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Immunoglobulin-derived light-chain (AL) amyloidosis of lungs and bronchi can appear as a systemic and a local form. While systemic AL amyloidosis may need haemato-oncological care, the localised form can be treated restrained. We re-evaluated 207 specimens of lungs and bronchi sent in for amyloid diagnostics. Amyloid was diagnosed by polarization microscopy using Congo red-stained tissue specimens and classified immunohistochemically. Histoanatomical amyloid distribution patterns were documented as well as additional histological findings. For 118 patients with AL amyloidosis, we retrieved clinical data. CT scan results were available from 59 patients. AL amyloidosis was the most common type (183 cases). ALλ was found in 141 and of ALκ in 27 cases. Fifteen cases were AL amyloid not otherwise specified. Twenty cases harboured transthyretin and three serum amyloid A derived amyloid. By correlation of histoanatomy, radiological and clinical data, amyloid was rarely in the initial differential diagnosis. Local AL amyloidosis often presented with a nodular pattern on CT scan and showed a significantly better disease-specific 10-year survival compared with systemic AL amyloidosis (96.0 vs. 51.9%). Localised and systemic pulmonary and bronchial AL amyloidosis are having a completely different prognosis. While CT scan might be indicative, histological and clinical assessment are mandatory to reach a proper diagnosis and guide patient care.

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“…The other three patients with localised amyloidosis were treated with external beam radiation therapy. Patient #6 already got published as a case report [29].…”

Section: Patients Treated With Amyloidosis Targeting Therapymentioning

confidence: 99%

Local vs. systemic pulmonary amyloidosis—impact on diagnostics and clinical management

et al. 2018

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“…The incidence of AL amyloidosis has been reported to be nine cases per million person-years [ 3 ]. However, respiratory tract involvement is rare [ 7 ]. More than 30 proteins have been identified, associated with varying amyloidosis subtypes [ 8 ].…”

Section: Discussionmentioning

confidence: 99%

Laryngotracheobronchial amyloidosis: A case report

Duc

et al. 2021

Respiratory Medicine Case Reports

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Primary laryngotracheobronchial amyloidosis is a rare pulmonary disease that can cause endobronchial stenosis. This disease has never previously been reported in Vietnam. We aimed to report a laryngotracheobronchial amyloidosis case in a 43-year-old female, which may be the first reported case in Vietnam. The patient had a 4-year history of progressive hoarseness, dyspnea, and hemoptysis. Multiple bronchial biopsies combined with detailed clinical information suggested an amyloidosis disease. Red congo staining was positive in bronchial samples, and a further workup found positive red congo staining in subcutaneous fatty tissue biopsy samples. Tracheostomy was performed due to severe dyspnea related to laryngeal stenosis. A multidisciplinary consultation was held, and chemotherapy with melphalan and dexamethasone were prescribed due to the systemic effects of the disease. After 2 cycles of chemotherapy, the patient showed improvement in dyspnea and cough. Due to the inexperience of both the clinicians and pathologists, this case was diagnosed quite late. In the future, if this diagnosis is considered in the differential diagnosis, an earlier diagnosis and better treatment outcome can be reached.

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“…Literature also supports multifocal or systemic involvement in patients of laryngeal amyloidosis. [15] A wide variety of studies have suggested different behavior of laryngeal amyloid. Clinical and laboratory assessment including a search for lymphadenopathy, radiographic imaging, urinary and digestive tract evaluation, ECG and blood count has been suggested to ascertain the true nature of the disease and rule out systemic disease.…”

Section: Discussionmentioning

confidence: 99%

Laryngeal Amyloidosis: Case Series Of An Unusual Cause Of Hoarseness

Kadam¹,

Kadam²,

Kutwal³

et al. 2017

AIMDR

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Background: Amyloid can be defined as in in-vivo deposited amorphous material which on electron microscopy is fibrillar in appearance and gives apple-green birefringence on Congo-red staining. Beta-pleated sheet structure observed on XRay Diffraction is the characteristic finding. Deposition of this amyloid material in the human body interfering in the normal physiological functions of the body is called amyloidosis. Amyloidosis can be divided into localized or systemic form depending upon whether it is arising from proteins expressed by cells at the local site or they get deposited at different organs after production at a local site. Though amyloidosis can affect any organ of the body the most commonly affected organs include kidneys, heart and liver. In head and neck region macroglossia is the most common manifestation of amyloidosis. Laryngotracheal system is also one of the commonly affected site for localized amyloidosis. It usually appears as nodule or polypoid lesion in the larynx and constitute 1% of all benign laryngeal lesions. We present here a case series of 6 cases of laryngeal amyloidosis who presented to ENT department of our medical college and a leading ENT hospital in an urban area. Aims: To study the clinical presentation, examination findings, management and outcome of patients with laryngeal amyloidosis. Methods: This is a case series of 6 patients who presented to us with gradual change in voice. Detailed history was noted and clinical examination was done. Laryngoscopy was done in all cases. Later microlaryngoscopy and CO2 laser excision of polypoidal masses was performed under general anesthesia. Diagnosis was made on haematoxylin and eosin stain while confirmation was done by Congo red stain. Patient was discharged postoperative day 2 with broad spectrum antibiotics, analgesics and oral methylprednisolone for one week and was advised to take voice rest for five days. All patients were followed on seventh day, 1month, 3 months and 6months interval and thereafter yearly. Results: Patients included 4 females and 2 males with female to male ratio 2:1 their ages ranged from 40 to 70 years with mean age at presentation of 55 yrs. The most common age group involved was found to be 50-60 years. In majority of the cases (5/6) a single lesion was found while in 1 patient the lesions were multiple involving larynx and epiglottis also. In majority of the patients (50%) the duration of hoarseness was 1-2 years. successful microlaryngoscopy and CO2 laser excision was done in all the cases. All the patients were followed up regularly for a period of 5years. One patient showed recurrence after 3 years after first surgery the patient who developed recurrent disease had polypoidal and diffuse amyloid deposits. Conclusion: Laryngeal amyloidosis is a rare benign condition affecting larynx and should be suspected in any patient presenting with gradually increasing hoarseness of voice. It is diagnosed by characteristic birefringence under polarized light. It can be effectively treated by laser excisi...

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Multifocal primary amyloidosis of the airways: Case report and review of the literature

Cited by 7 publications

References 22 publications

Local vs. systemic pulmonary amyloidosis—impact on diagnostics and clinical management

Local vs. systemic pulmonary amyloidosis—impact on diagnostics and clinical management

Laryngotracheobronchial amyloidosis: A case report

Laryngeal Amyloidosis: Case Series Of An Unusual Cause Of Hoarseness

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