Gastrointestinal Manifestations of Amyloidosis

Lee, J. G.; Wilson, J. A. P.; Gottfried, Marcia R.

doi:10.1097/00007611-199402000-00019

Cited by 36 publications

(9 citation statements)

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“…The gastrointestinal tract is most commonly involved in all types of systemic amyloidosis, manifesting uncontrollable diarrhea, constipation, malabsorption syndrome and pseudointestinal obstruction, but fatal hemorrhagic accidents appear to be rare (1)(2)(3)(4)(5).…”

Section: Introductionmentioning

confidence: 99%

Recurrent Gastric Hemorrhaging with Large Submucosal Hematomas in a Patient with Primary AL Systemic Amyloidosis: Endoscopic and Histopathological Findings

et al. 2004

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A 64-year-old woman who suffered intractable gastric ulcers with hemorrhaging showed huge submucosal hematomas in her stomach on the endoscopic examination. Since gastric mucosal biopsy revealed amyloid deposition and IgG type M protein was detectable in her serum, she was diagnosed as having primary AL systemic amyloidosis. The gastric hemorrhages did not improve despite intensive medication, so total gastrectomy was performed, resulting in an unfavorable outcome. Massive deposition of amyloid with A immunoreactivity was seen on the submucosal vessels in her stomach. This is a rare primary AL systemic amyloidosis case showing recurrent and fatal gastric submucosal hematomas.

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Section: Introductionmentioning

confidence: 99%

Recurrent Gastric Hemorrhaging with Large Submucosal Hematomas in a Patient with Primary AL Systemic Amyloidosis: Endoscopic and Histopathological Findings

et al. 2004

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“…Autopsy studies show deposition of amyloid in the GI tract in 70% to 100% of patients. 11,12 GI tract amyloidosis usually takes the form of malabsorption syndromes and nonspecific GI tract symptoms, 1,13,14 but GI tract bleeding as a manifestation has been reported by several authors. 11,[15][16][17] The structural involvement of the GI tract is often compounded by coagulation abnormalities secondary to factor deficiencies, 18 leading to episodes of GI tract bleeding.…”

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confidence: 99%

High incidence of gastrointestinal tract bleeding after autologous stem cell transplant for primary systemic amyloidosis

Kumar

Dispenzieri

Lacy

et al. 2001

Bone Marrow Transplant

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Summary:Peripheral blood stem cell transplants have been associated with better response rates than conventional chemotherapy in patients with primary systemic amyloidosis. A higher incidence of gastrointestinal (GI) tract bleeding has been observed among amyloidosis patients undergoing peripheral stem cell transplantation. We retrospectively reviewed the medical records of such patients to identify those who had GI tract bleeding in the post-transplant period. Forty-five patients were studied. Nine patients had GI tract bleeding in the posttransplant period. The median post-transplant duration to onset of bleeding was 9.5 days (range 1 to 48 days). Three patients had lower GI tract bleeding, two had upper GI tract bleeding, and four had both. Diffuse esophagitis and gastritis were the most common findings on endoscopy. There were no correlations among the age, platelet nadir, or CD34 count of the graft and the risk of bleeding. Women were more likely to have GI tract bleeding (P = 0.015), as were patients with slow platelet engraftment (P = 0.02). Patients with multiorgan involvement and those on hemodialysis appeared to be at a higher risk of GI tract bleeding. The mean posttransplant hospital stay for those with GI tract bleeding was 37 days compared with 14.5 days for those who did not have GI tract bleeding (P = 0.0047). Bone Marrow Transplantation (2001) 28, 381-385. Keywords: GI bleeding; high-dose chemotherapy; stem cell transplantation; systemic amyloidosis Primary systemic amyloidosis is a rare plasma cell proliferative disorder with an incidence of nearly eight per million per year. The prognosis for patients diagnosed with the disorder remains poor, with a median survival of only 13 months for those seen within 1 month of diagnosis.1 In those with congestive cardiac failure, the median survival is only 4 months, and only 5% survive more than 10 years. Currently available therapy has a minimal effect on the natural history of the disease and includes combination chemotherapy with melphalan and prednisone.2-4 Addition of other drugs, namely vincristine, BCNU, and cyclophosphamide to melphalan and prednisone (VBMCP), has not improved the results over melphalan and prednisone alone.

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“…This is most probably due to the fact that AL amyloidosis is the most common form of this disease. In fact, from the 1990s onwards, the AA/AL ratio was 1/17 to 1/38 due to fewer chronic infections and increasing recognition of AL amyloidosis [19, 20]. …”

Section: Discussionmentioning

confidence: 99%