Systemic AL-amyloidosis with dominant clinical manifestation in digestive system
Authors:
R. Kroupa 1; M. Dastych 1; M. Šenkyřík 1; J. Lata 1; Petr 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
Sources
1. Adam Z, Vorlíček J et al. Hematologie II. Přehled maligních hematologických nemocí. Praha: Grada Publishing 2001.
2. Araoz PA, Batts KP, Mac Carty RL. Amyloidosis of the alimentary canal: radiologic-pathologic correlation of CT findings. Abdominal Imaging 2000; 25: 38–44.
3. Brychta T, Pařenica J, Zatočil T et al. Restriktivní kardiomyopatie jako projev primární amyloidózy. Vnitř Lék 2004; 50: 66–71.
4. Falk RH, Comenzo RL, Skinner M. The systemic amyloidozes, review article. N Eng J Med 1997; 337: 898–909.
5. Hayman SR, Lacy MQ, Kyle RA et al. Primary systemic amyloidosis: a cause of malabsorption syndrome Am J Med 2001; 111: 535–540.
6. Hurlstone DP. Iron–deficiency anemia comlicating AL amyloidosis with recurrent small bowel pseudoobstruction and hindgut sparing. J Gastroenterol Hepatol 2002; 17: 623–624.
7. Husová L, Lata J, Hušek K et al. Primární amyloidóza jako neobvyklá příčina krvácení z trávicího ústrojí. Čes a Slov Gastroent 2000; 54: 112–116.
8. Chang SS, Lu CL, Tsay SH et al. Amyloidosis–induced gastrointestinal bleeding in a patient with multiple myeloma. J Clin Gastroenterol 2001; 32: 161–163.
9. Klener P et al. Vnitřní lékařství. Praha: Galén, 2. vydání, 2001.
10. Kyle R, Gertz MA. Primary systemic amyloidosis: clinical and laboratory features in 474 cases. Seminars in Hematology 1995; 32: 45–59.
11. Peny MO, Debongnie JC, Haot J et al. Localized amyloid tumor in small bowel. Dig Dis Sci 2000; 45: 1850–1853.
Labels
Diabetology Endocrinology Internal medicineArticle was published in
Internal Medicine
2005 Issue 5
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