Tirotta Daniela* and Durante Vittorio
Internal Medicine, Cervesi Hospital, Cattolica (AUSL Romagna), Italy
Received: 30 April, 2016; Accepted: 24 June, 2016; Published: 25 June, 2016
Daniela Tirotta, Medicina Interna, ospedale Cervesi di Cattolica (AUSL Romagna), Via Beethoven 1, 47841, Cattolica (AUSL Romagna), Italy, Tel: 0541/966291; Fax: 0541966290; E-mail:
Daniela T, Vittorio D (2016) Gastrointestinal Amyloidosis: An Unusual Case Where Protein-Losing Enteropathy is Associated to Malabsorption. Arch Clin Gastroenterol 2(1): 050-052. 10.17352/2455-2283.000020
© 2016 Daniela T, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Gastrointestinal AL amyloidosis; Enteropathy protein-losing; Malabsorption
Introduction: The systemic amyloidosis usually doesn’t save the digestive tract, but this involvement is present in approximately 60% of patients with AA Amyloidosis and only in 8% and 1% of patients with AL Amyloidosis (respectively through biopsy and clinically). Beside gastrointestinal involvement is rarely symptomatic and clinical events are not specific.
Enteropathy protein-losing is a rare condition caused by excessive loss of serum protein in the gastrointestinal tract, resulting in hypoproteinaemia, edema, and, sometimes, pleural/pericardial effusions. The diagnosis should be taken into account only when other causes have been excluded.
Gastrointestinal AL amyloidosis usually appears with constipation and mechanical obstruction/pseudobstruction, on the contrary enteropathy protein-losing and malabsorption are rare.
Case report: We report the case of a 59-year -old patient, with AL amyloidosis, who suffered from weight loss and ascites effusion related to an AL amyloidosis.
Discussion: The case is unusual, both because of the extension in the gastrointestinal tract and for its unusual clinical presentation as enteropathy protein-losing associated with malabsorption.
The systemic amyloidosis usually doesn’t save the digestive tract, but this involvement is present in approximately 60% of patients with AA Amyloidosis and only in 8% and 1% of patients with AL Amyloidosis (diagnosis respectively made through biopsy and clinically) . Beside gastrointestinal involvement is rarely symptomatic and clinical events are not specific .
Enteropathy protein-losing is a rare condition caused by excessive loss of serum protein in the gastrointestinal tract, resulting in hypoproteinaemia, edema, and, sometimes, pleural/pericardial effusions. The diagnosis should be taken into account only when other causes have been excluded (malnutrition, heavy proteinuria, and reduced protein synthesis due to liver diseases).
Gastrointestinal AL amyloidosis usually appears with constipation and mechanical obstruction/pseudobstruction, on the contrary enteropathy protein-losing and malabsorption are rare [1,2].
A 59 years old man was admitted for loss weight, taste perversion, diarrhea. Medical history:
A month earlier, due to an exertional dyspnea, he carried out an ECG (right ventricular hypertrophy and previous anterior septal ischemia)
He carried out an echocardiogram, positive for mild cardiac hypertrophy (apical prevalence), left atrial enlargement, and small pericardial effusion.
The Diagnosis. Physical examination showed basal pulmonary obtusity, abdomen US confirmed ascites and pleural effusion. Macroglossia was absent.
Blood tests showed low cholesterol levels (122 mg/dl total, 79 mg/dl LDL, 29 mg/dl HDL, 70 mg/dl), hypoproteinemia (54 g/l protein, 64.1% albumin); hypogammaglobulinemia (9.6% gamma-globulins).
Thoracoabdominal computed tomography confirmed modest ascites and lung interstitium thickening.
Because of gastrointestinal symptoms, ascites, hypoproteinemia and low cholesterol levels (in absence of malnutrition, proteinuria, and liver disease), we assumed the hypothesis of enteropathy protein-losing. The cardiopathy and the hypogammaglobulinemia encouraged us to think about lymphoproliferative disease.
Therefore, we planned an esophagogastroduodenoscophy and a colonoscopy, with evidence of widespread gastrointestinal amyloidosis.
In particular endoscopy showed erythematous striate in the gastric antrum, wall thickening, erythema and erosions in the second portion of the duodenum, extensive scarring of the whole circumference in the proximal transverse.
Histological examination showed the homogeneous eosinophilic material in duodenal and gastric withdrawals, in the vascular and in the lamina propria, with epithelial erosions, finally deposits in the vessel wall of submucosa of large intestine. No evidence of lymphoma. Positive staining with Congo red.
Blood tests revealed mild proteinuria (0.19 g/24 hours), urine Bence Jones positive, peripheral CD4/CD8 ratio inversion. Blood tests also showed high pro-BNP (4829 ng/l) and positive troponin on two points (63 ng/l). LDH and serum urea were normal.
Therefor it was planned a bone marrow biopsy, positive for monotypic kappa myeloma, first pathological stage associated to monotipicity for the light chain kappa immunoglobulin.
In the context of vascular walls there is a deposition of amorphous homogeneous substance and positive PAS: compatible with amyloid (Congo red staining positive) (Figures 1,2).
- Camilleri M, Friedman LS, Grover S (2015) Gastrointestinal amyloidosis: Clinical manifestations, diagnosis and management .
- Pavic M, Camus C, Pasquet F, Karkowski L, Galoo E, et al. (2011) [Seriousness of AL amyloidosis of the digestive system]. Rev Med Interne 32: e52- e54 .
- Milovic V, Grand RJ, Lamont JT (2013) Protein-losing gastroenteropathy. Up To Date .
- Béchade D, Carmoi T, Algayres JP (2008) Amylose digestive (foie exclu). EMC (Elsevier Masson SAS, Paris), Gastroentérologie 9-089-C-15 .
- James DG, Zuckerman GR, Sayuk GS, Wang HL, Prakash C (2007) Clinical recognition of AL type amyloidosis of the luminal gastrointestinal tract. Clin Gastroenterol Hepatol 5: 582–588 .
- Repiso A, Valle J, Rodriguez-Merlo R, Munoz-Rosas C, Bolano F, et al. (2003) Gastrointestinal bleeding and small bowel amyloidosis. Rev Esp Enferm Dig 95: 578–580 .
- Kawaguchi M1, Koizumi F, Shimao M, Hirose S (1993) Protein-losing enteropathy due to secondary amyloidosis of the gastrointestinal tract. Acta Pathol Jpn 43: 333-339 .
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