Abbas, Zaigham

Abid, Shahab

Pervez, Shahid

Rehman, Naim -ur-

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eCommons@AKUDepartment of Pathology and Laboratory Medicine Medical College, PakistanOctober 1995Primary Amyloidosis of Gut presenting withDysphagiaZaigham AbbasBaqai University HospitalShahab AbidBaqai University HospitalNaim -ur- RehmanAga Khan UniversityShahid PervezAga Khan University, shahid.pervez@aku.eduFollow this and additional works at: http://ecommons.aku.edu/pakistan_fhs_mc_pathol_microbiolPart of the Oncology Commons, and the Pathology CommonsRecommended CitationAbbas, Z., Abid, S., Rehman, N. -., Pervez, S. (1995). Primary Amyloidosis of Gut presenting with Dysphagia. Journal of PakistanMedical Association, 45(10), 274-275.Available at: http://ecommons.aku.edu/pakistan_fhs_mc_pathol_microbiol/310Primary Amyloidosis of Gut Presenting with DysphagiaPages with reference to book, From 274 To 275 Zaigham Abbas, Shahab Abid ( Dept. of Medicine, Baqai University Hospital, Karachi. ) Naim-ur-Rehman ( Dept. of Medicine, Aga Khan University Hospital, Karachi. ) Shahid Pervez ( Dept. of Pathology, Aga Khan University Hospital, Karachi. ) Introduction The gut may be involved in the primaiy (AL) amyloidosis1. It is associated with dysmotility andmalabsorption2. Endoscopic examination of these cases reveals fine granular appearance of mucosa,polypoid protrusions, erosions, ulcerations and mucosal friability3. We present here a case of primaryamyloidosis of gut who presented with dysphagia.Case report A sixty-five year old lady presented with increasing dysphagia forboth solids and liquids. Her initialsymptom was feeling of food sticking in the retrostemal region followed by vomiting of food, anorexiaand constipation. She became dependent on laxatives to move her bowels on alternate days. She lostabout 10 Kg of body weight and became completely bedridden. There was a past histor of breastcarcinoma operatedoneyearback, whichwas stage I disease. Post-operatively radiotherapy wasadministered and presently tainoxifen was in use. On examination, the patient was afebnle, looked paleand had bilateral xanthelasma. Pulse rate was 80 per minute and blood pressure 90/60 mmHg in supineposition and there was mild pedal odema. Systemic examination revealed no abnonnality. Haemoglobinwas 9.4 gram/dl with nomiochromic normocytic picture, total leucocyte count 10400/uf, ESR 8 mm 1sthr., serum creatinine 1.2 mg/dl and serum sodium 128 mmol/1. Transaminases were normal. Albuminwas 2.0 gfdl and globulins 1.7 g/dl. Protein electrophoresis showed an increase in alpha one fractionwith a decrease in beta and gamma fractions of globulin. She was mildly hypothyroid with a TSH of8.29 uU/ml (0.3 2-5.0) and T4 of 4.0 ug/dI (4.5-12.0). Urine DR and Chest X-rays were normal.Barium studies could not be done as patient was bedridden. Upper gastrointestinal endoscopy wasperformed. There was paucity of primary penstaltic waves inoesophagus. A ring of contraction wasseen in the distal oesophagus 3 cm above the Z line. The ‘ring’ relaxed intermittently for brief periods.Lower oesophageal sphincter was gaping. The oesophageal mucosa was erythematous with finegranularity and erosions near the Z line. The gastric lake was full of greenish biliary fluid. Pylorus waswide open and gastric and duodenal mucosa were erythematous. Biopsies were taken from oesophagus,stomach and duodenum. Later rectal biopsy was also taken. Histopathology report revealedhomogenous thickening of blood vessel walls in all the specimens with areas exhibitingnodulesofeosinophilichomogenous material in the lamina propria. Congo red staining with greenbirefringence on polarisation confirmed the amyloid deposits in these areas and vessel walls (Figures 1and 2).Pretreatment with modified pennanganate stain followed by standard Congo red stain did not abolishthe green birefringence in the polarized microscope confirming the AL type of amyloid (primaryamyloidosis).Discussion Amyloidosis of oesophagus may present with achalasia4-6, oesophageal spasms7, non-specific motilitydisorder8 or decreased lower oesophageal sphincter pressure9. The manometric abnormalities observedare consistent with depositibn of amyloid in smooth and striated muscle as well as in the entire nervoussystem9,10. Though the manometric studies were not done in our case, severe motility dysfunction wasapparent on endoscopic examination. Paucity of any propulsive waves and presence of a ring ofcontraction in the lower oesophagus could explain her symptoms of sticking of food and dysphagia.There was also endoscopic evidence of gastroparesis and bile reflux. Dysmotility had affected herbowel habits and she was experiencing increasing constipation, alteration in the bowel habits andintestinal pseudo-obstruction have been reported in amyloidosis of gut11. Our patient had AL type ofamyloid deposits. In one study abdominal distension, nausea and vomiting were more frequent inpatients of AL type whereas, incidence of diarrhoea and gastrointestinal bleeding was high in AAtype12. Duodenum is involved in almost all cases of gastrointestinal amyloidosis as compared to rectumwhich may be involved in 86-91% cases3,12. So duodenal biopsy should replace the rectal biopsy toincrease the diagnostic yield.References 1. Case records of the Massachusetts General Hospital. Case 43-1985. N. Engl. J. Med.,1985;313:1070-9.2. Kyle, R. A. and Greipp, P.R. Amyloidosis (AL). Clinical and laboratory features in 229 cases. MayoClin. Proc., 1983;58 :665-83.3. Tada, S., lida, M., Iwashita, A. et al. Endoscopic and biopsy findings of the upper digestivetract inpatients with amyloidosis. Gastrointest. Endosc., 1990;36: 10-144. Costigan, D. J. and Clouse, R. E. Achalasia - like oesophagus from amyloidosis Successful treatmentwith pneumatic bag dilatation. Dig. Dis. Sci., 1983;28:763-5.5. Lctkowitz, J. R., Brand, D. L., Schuffler, M. D. et al. Amyloidosis mimics achalasia’s effect on loweresophageal sphincter. Dig. Dis. Sci., 1989;34:630-5.6. Suns, X., Moya, F., Panes, J. et al. Achalasia of the esophagus in secondary amyloidosis. Am. J.Gastroenterol., 1993;88: 1959-60.7. Funch - Jensen, P., Foged, E., Ravnsback, J. et al. A case ofesophageal spasm in amytoidosis. ActaMed. Scand., 1986;219:229- 33.8. Bjerle, P, Ek, B., Linderholm, H. et al. Oesophageal dysfunction in familial amyloidosis withpolyneuropathy. Clin. Physiol., 1993; 13:57-69.9. Burakoff. R., Rubinow. A. and Cohen, A. S. Esophageal manometry in familial polyneuropathy. Am.J. Med., 1985;79:85-9.10. Rubinow, A., Burakofl R., Cohen, A. S. et at. Esophageal manometry in systemic amyloidosis. Astudy of 30 patients. Am. J. Med., 1983;75:951-6.11. Lea, J. G., Wilson, J. A. and Gottfried, M. R. Gastrointestinal manifestations of amytoidosis. SouthMed. J., 1994;87:243-7.12. Tada, S. Diagnosis of gastrointestinal amytoidosis with special reference to the relationship withamyloid fibril protein. Fukuoka Igaku Zasshi, 1991 ;82:624-47.

Primary Amyloidosis of Gut presenting with Dysphagia

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Primary Amyloidosis of Gut presenting with Dysphagia

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