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Gastrointestinal Tract Amyloidosis Presenting With Pneumatosis Intestinalis
#MMPMID28611868
Raghunathan V
; Louis D
; Wirk B
J Clin Med Res
2017[Jul]; 9
(7
): 654-658
PMID28611868
show ga
Pneumatosis intestinalis is a radiographic finding of gas pockets within the
bowel wall. It can be associated with a range of diagnoses, but the most
life-threatening causes are mesenteric ischemia, bowel necrosis, and bowel
obstruction. Here we present the case of a patient with multiple myeloma who had
pneumatosis intestinalis due to gastrointestinal amyloidosis, which is a rare
manifestation of systemic amyloid disease. The patient had both transthyretin
(ATTR) amyloidosis and acquired apolipoprotein serum amyloid A (AA) amyloidosis
that are not usually seen in conjunction with multiple myeloma, which is most
commonly associated with light-chain (AL) amyloidosis. This case highlights the
importance of considering Congo red staining of bowel biopsies for amyloid
deposition in patients undergoing endoscopy for unexplained gastrointestinal
tract symptoms and even pneumatosis intestinalis, so as to avoid a delay in
diagnosis that is typically seen with amyloidosis. Since each subtype of
amyloidosis requires different therapy, amyloid subtyping is crucial, even with
co-existing multiple myeloma.
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