Systemic AL-k amyloidosis with lymph node, tracheobronchial, pulmonary and gastrointestinal involvement – a diagnostic and therapeutic challenge
Keywords:
Amyloidosis, Trachea, Bronchi, Lymph nodes, intestines, Diagnosis, TherapeuticsAbstract
Tracheobronchial and pulmonary involvement is uncommon in
amyloidosis and it is not usually associated to its systemic forms.
It is equally rare the presence of generalized lymphadenopathy
as a form of presentation.
We present the case of a 75-years-old man with anorexia,
weight loss and gastrointestinal complaints. At physical examination he had sparse rhonchi in pulmonary examination and an
abdominal mass. Image exams showed abdominal adenopathies
extending to the thorax and a micronodular pattern on both lung
fields. Transbronchial biopsy showed hyaline material compatible with amyloidosis; similar findings were made in a peripheral
adenopathy and in an abdominal fat pad biopsy. The bone marrow
aspirate and biopsy were normal, and there were no monoclonal
peaks in serum and urinary immune-electrophoresis. The immunohistochemistry revealed positivity for k light chains. Treatment was started with melphalan and prednisolone. The patient
died six months later due to mesenteric ischemia. Pathological
examination revealed interstitial and vascular amyloid deposits.
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