|Year : 2019 | Volume
| Issue : 3 | Page : 134-136
Tracheal amyloidosis presenting with hemoptysis
Madhu Priya, Ranjan Kumar Das, Srabani Mukherjee
Department of Pulmonary Medicine, CMRI, Kolkata, West Bengal, India
|Date of Submission||30-Jul-2018|
|Date of Acceptance||27-Aug-2018|
|Date of Web Publication||23-Aug-2019|
Flat No. 8e, Block B, Ideal Towers, 27, Diamond Harbour Road, Khiddirpore, Kolkata, West Bengal
Source of Support: None, Conflict of Interest: None
Amyloidosis is a generic term for a heterogeneous group of disorders associated with deposition of protein in an abnormal fibrillar form. The diverse spectrum of amyloid-related diseases is now recognised to include Alzheimer's disease, Type II diabetes and transmissible spongiform encephalopathies. Amyloidosis can be hereditary or acquired, localised or systemic and potentially lethal or merely an incidental finding. Tracheal amyloidosis refers to the deposition of localised amyloid deposits in the trachea. Localised tracheal amyloidosis is a rare form of amyloidosis with only a few hundred cases reported so far. We report here a case of a 65-year-old male presenting with haemoptysis.
Keywords: Amyloidosis, Congo red, hemoptysis
|How to cite this article:|
Priya M, Das RK, Mukherjee S. Tracheal amyloidosis presenting with hemoptysis. Hamdan Med J 2019;12:134-6
| Introduction|| |
Amyloidosis is a disorder involving abnormal protein folding and deposition of insoluble amyloid fibril proteins accumulating in normal tissues. Tracheal amyloidosis refers to the deposition of localized amyloid deposits in the trachea. The patient may present with hemoptysis, stridor athough sometimes it may be asymptomatic.
| Case Report|| |
A 65-year-old hypertensive and diabetic gentleman, ex-smoker, presented to the Outpatient Department with chief complaints of haemoptysis (mild to moderate) on 3–4 occasions. There was no history of associated dyspnoea, fever or previous hospitalisation.
On physical examination, blood pressure was 130/70 mmHg, respiratory rate was 14/min and heart rate was 80/min in normal rhythm. There were no abnormal sounds on pulmonary auscultation. Routine laboratory test results were within normal limits. Forced expiratory volume in 1 s (FEV1) was 2.66 L (88% of predicted value), forced vital capacity (FVC) was 3.54 L (92% of predicted value) and FEV1/FVC ratio was 75% on his spirometry. Chest computed tomography (CT) scan revealed a non-enhancing mucosal thickening in the right anterolateral wall of the trachea at T2–T3 level of length 55 mm and thickness 16 mm [Figure 1]. Bronchoscopy was performed and showed a lobulated soft-tissue mass over the right wall of the trachea approximately 2.5-cm distal to vocal cord with hyperaemic irregular mucosa and nodularity above the carina [Figure 2]. Tracheal biopsy was taken and sent for further studies. Histopathological examination showed nodular masses and sheets of extracellular, eosinophilic, hyaline material consistent with amyloid [Figure 3] which was further confirmed by Congo red stain [Figure 4]. Histopathological evaluation revealed that the tumour was tracheobronchial amyloidosis (TBA).
|Figure 1: Chest computed tomography scan revealed a non-enhancing mucosal thickening in the right anterolateral wall of the trachea at T2–T3 level of length 55 mm and thickness 16 mm|
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|Figure 3: Amorphous eosinophilic appearance on haematoxylin and eosin staining|
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|Figure 4: Congo red stain under light microscope showing amorphous homogenous pink amyloid deposits beneath the tracheal epithelium|
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| Discussion|| |
There are two major types of amyloidosis: primary and secondary. Primary amyloidosis most commonly affects the respiratory system. Respiratory amyloidosis has been described in three forms: diffuse interstitial, nodular parenchymal and tracheobronchial. Amyloidosis occurring in the respiratory system may involve the larynx, trachea, bronchus, lung and mediastinal lymph nodes. Respiratory tract amyloidosis may occur in cases of systemic amyloidosis but more often occurs in isolation.
TBA is an uncommon localised form of amyloidosis, characterised by abnormal amyloid deposits in the trachea and main bronchi. Amyloidosis commonly affects males (2:1) in middle age (50–60 years) groups.
The clinical presentations of the TBA vary depending on the involved area. In the case of pulmonary involvement, the patients sometimes have no symptoms. However, common presenting symptoms include cough (74%), wheezing (70%), dyspnoea (60%), haemoptysis (50%) and stridor (30%).
Thorax CT is sensitive for detection of changes associated with amyloidosis. Submucosal wall thickening with irregular calcifications and luminal narrowing can be seen in the trachea and main bronchi. These conditions are seen like diffuse submucosal plaques on bronchoscopic examination. Only 14% of the cases have solitary deposits, and these deposits mimic endobronchial malignancy. Screening for symptoms of systemic amyloidosis should be performed in any patient with confirmed TBA. Studies include electrocardiogram, echocardiogram, serum and urine protein electrophoresis and creatinine level. Evaluation for multiple myeloma should be included in the workup given. Cardiac, renal and hepatic disease can range from asymptomatic to end-organ failure. Our patient was evaluated for all of the above, and there were no signs of systemic involvements.
Prognosis of TBA is variable depending on the degree of obstruction. No treatment with simple monitoring is proposed in asymptomatic patients, whereas more aggressive local and/or systemic therapy is undertaken when the obstruction is significant and the patient symptomatic.,, Bronchoscopic recanalization with neodymium: yttrium aluminium garnet and CO2 laser and mechanical resection have been documented to be successful in individual cases., There are reports of successful use of stents in recurrent stenotic tracheobronchial segments. Our patient has been kept on observation as he is not having any respiratory discomfort.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]