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Open Journal of Hematology

ISSN: 2075-907X
Volume 8, 2017



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EMBASE

Open Journal of Hematology, 2014, 5-3 [Case Report]

Patient with primary systemic amyloidosis presenting with intractable diarrhoea

Hemant Saha1, Ahmed Twahir1, Fatima Juma1, Ruchika Kohli2, Malkit S. Riyat1

1 The Aga Khan University Hospital, Nairobi, Kenya
2 Pathologists Lancet Kenya, Nairobi, Kenya

Corresponding Author & Address:

Ruchika Kohli *
P O Box 46203-00100, Nairobi, Kenya; Email: ruchikakohli@hotmail.com

Article History:
Published: 26th May, 2014   Accepted: 26th May, 2014
Received: 12th January, 2014      

© Kohli et al.; licensee Ross Science Publishers

ROSS Open Access articles will be distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided that the original work will always be cited properly.

Abstract:

Introduction:
Primary systemic amyloidosis is the most common form of systemic amyloidosis. Clinical presentation commonly involves organs such as the kidney and heart. We report on a patient with systemic amyloidosis presenting as intractable diarrhoea. Numerous investigations had to be done before the diagnosis could be made highlighting the challenge in making a diagnosis due to the slow progression of the disease. This report is to increase awareness among physicians of this diagnosis and to emphasise the importance of identifying patients quickly.

Case Presentation:
A 63 year old male was admitted with a six months history of intractable, watery diarrhoea, anorexia and progressive renal failure. He had been investigated for proteinuria and deranged renal function prior to admission but no therapy was suggested and the diagnosis at that time was inconclusive. Extensive laboratory and radiological investigations were done. The diagnosis of systemic amyloidosis was made on serum free light chains which showed excess lambda chains and a terminal iliac and colonic biopsy which revealed eosinophilic thickening of the blood vessels with positive Congo red stain. Unfortunately the patient died prior to definitive management for the amyloidosis.

Conclusion:
This case emphasises the importance of early recognition of systemic amyloidosis so that management can be instituted.



©2017 Ross Science Publishers