Amyloid light-chain amyloidosis presenting as abdominal bloating: a case report
نویسندگان
چکیده
BACKGROUND We present a case of amyloid light-chain amyloidosis with occult plasma cell dyscrasia, with the rare initial presentation of gastroparesis. While amyloidosis is known to affect the gastrointestinal system, rarely do patients present with gastrointestinal symptoms as their first symptom. To the best of our knowledge, this is the first such case reported with a definitive diagnosis made on gastroscopy. CASE PRESENTATION A 52-year-old Malay man with abdominal bloating, early satiety, and weight loss was found to have significant gastroparesis. He had a past medical history of stable non-ischemic cardiomyopathy. Results from initial screening were negative for common causes of gastroparesis, such as diabetes or offending medications. Gastroscopy did not show any mechanical gastric outlet obstruction. Our patient subsequently developed symptoms of postural giddiness, which then prompted further investigations for possible autonomic dysfunction. These finally revealed evidence of systemic involvement, including postural hypotension, speckled myocardium with infiltrative cardiomyopathy on a transthoracic echocardiogram, and multifocal motor neuropathy on nerve conduction studies, from which he had been relatively asymptomatic. These findings were collectively suggestive of infiltrative disease. Retrospective Congo red staining of a gastric biopsy specimen confirmed the diagnosis of gastric amyloidosis. The final diagnosis was amyloid light-chain amyloidosis secondary to plasma cell dyscrasia, which was confirmed by bone marrow examination. Our patients was started on chemotherapy and prokinetic agents, with some improvement in gastrointestinal symptoms on follow-up. CONCLUSION We present this case to highlight that, although rare, gastroparesis can be the initial sole presentation of amyloidosis. It is important for the internist, gastroenterologist, and hematologist to consider amyloidosis as a differential diagnosis in the investigation of gastroparesis and to be vigilant in monitoring for other systemic involvement.
منابع مشابه
Multiple Myeloma Associated Intestinal Amyloidosis: Intestinal Pseudoobstruction Falsely Considered as an Ascites.
INTRODUCTION Amyloidosis is a group of diseases pathohistologically diagnosed by characteristic extracellular deposition of an abnormal fibrillary protein (i.e. amyloid) into organs, leading to organ dysfunction secondary to destruction of normal tissue architecture. METHODS Case-report of a 44 year-old female, presenting with massive abdominal distension clinically suspected of ascites. RE...
متن کاملFamilial Amyloid Polyneuropathy Type IV (FINNISH) with Rapid Clinical Progression in an Iranian Woman: A Case Report
Familial amyloid polyneuropathy (FAP) type IV (FINNISH) is a rare clinical entity with challenging neuropathy and cosmetic deficits. Amyloidosis can affect peripheral sensory, motor, or autonomic nerves. Nerve lesions are induced by deposits of amyloid fibrils and treatment approaches for neuropathy are challenging. Involvement of cranial nerves and atrophy in facial muscles is a real concern i...
متن کاملDiffuse Peritoneal and Bowel Wall Infiltration by Light Chain-AL Amyloidosis with Omental Calcification Mimicking Abdominal Carcinomatosis – An Elderly Female with Incidental Finding of Light Chain Monoclonal Gammopathy of Undetermined Significance (LC-MGUS)
BACKGROUND Amyloidosis is the extracellular tissue deposition of plasma proteins, which after conformational changes, forms antiparallel beta pleated sheets of fibrils. Amyloid light-chain (AL) is a type of amyloidosis that is due to deposition of proteins derived from immunoglobulin (Ig) light chains. Gastrointestinal tract (GIT) involvement most often found in amyloid A (AA) amyloidosis type....
متن کاملMyocardial 99mTc-MDP uptake on the bone scintigraphy in the hemodialysis-associated amyloidosis: A case report [Persian]
Amyloidosis is characterized by an abnormal extracellular deposition of amyloid in different organs, where it usually causes some type of dysfunction. Its cause is unknown. Five different types of amyloidosis have been described according to the underlying disease; immunoglobulin amyloidosis, familial amyloidosis, senile systemic amyloidosis, secondary amyloidosis and hemodialysis-associa...
متن کاملگزارش یک مورد آمیلوییدوز سیستمیک در حنجره
Introduction: Amyloidosis is an idiopathic disorder characterized by amyloid deposition leading to tissue damage and disease. Laryngeal amyloidosis is usually a localized phenomenon that is rarely accompanied by systemic involvement. Hoarseness is its most common symptom and the clinical findings in laryngoscopy are variable, nonspecific and difficult to be distinguished from other laryngea...
متن کامل