Systemic AL-amyloidosis with dominant clinical manifestation in digestive system

Czech version

Authors: R. Kroupa ¹; M. Dastych ¹; M. Šenkyřík ¹; J. Lata ¹; P. Dítě ¹; S. Hotárková ²; P. Hrobař ³
Authors‘ workplace: Interní gastroenterologická klinika Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta prof. MUDr. P. Dítě, DrSc. ¹; Patologicko-anatomický ústav Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta prof. J. Mačák, CSc. ²; Radiologická klinika Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta prof. MUDr. V. A. Válek, CSc. ³
Published in: Vnitř Lék 2005; 51(5): 588-592
Category: Case Reports

Overview

Paper describes a case of atypical manifestation of systemic AL-amyloidosis associated with multiple myeloma in 69 year old women with long-term dyspeptic complaint assigned to chronic gastroduodenal ulcer disease. She repeatedly underwent surgery for duodenal stenosis. Weight loss and laboratory signs of severe malnutrition gradually proceeded until amyloid deposits were demonstrated in biopsy in repeated sampling from chronic gastric ulceration. The condition proceeded and the patient died for associated infectious complications. The autopsy demonstrated massive amyloid infiltration of myocardium, lungs, stomach, bowel and kidneys. Plasmocytosis corresponding to multiple myeloma was found in bone marrow. The case was closed as a primary systemic amyloidosis of light chain type (AL) in multiple myeloma with clinically dominant infliction of digestive tract and malabsorption. Amyloidosis must be considered in differential diagnosis of all chronic inflammatory changes and gastric and intestinal infiltration. For definite assessment of diagnosis a finding of amyloid fibrils in histological examination is required.

Key words:
primary AL-amyloidosis – malabsorption syndrome – multiple myeloma

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