Amyloidosis - an unusual case of recurrent intestinal bleeding and sigmoid perforation: case report with review of the literature

Thaler, W.; Schatzer, G.; Eder, Piotr; Fichtel, Gertrud

doi:10.1007/s003840050232

Cited by 22 publications

(9 citation statements)

References 15 publications

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“…However, GI tract bleeding, both from the upper and lower GI tract, has been reported by several authors. 11,15,[20][21][22][23][24][25] A high incidence of GI tract bleeding as a presenting symptom (25%-45%) of amyloidosis has been described by some authors. 15,26 Levy et al 15 in a review from 1982 described 31 cases of GI tract bleeding associated with amyloidosis reported between 1948 and 1978.…”

Section: Tablementioning

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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Section: Tablementioning

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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“…3 Abnormal bleeding is frequently observed in patients with primary amyloid light-chain amyloidosis, 2 and severe life-threatening bleeding can occur. [4][5][6] In addition, some cases require surgical intervention for gastrointestinal bleeding, [17][18][19] and providing adequate hemostasis for surgical procedures can be challenging. 20 It has been previously reported that less than 5% of primary amyloidosis is associated with factor X deficiency, 15 but recent larger series of 368 and 337 patients demonstrated that the incidence is higher (8.7 % 1 and 14 %, 2 respectively).…”

Section: Case Reportmentioning

confidence: 99%

Successful perioperative management of factor X deficiency associated with primary amyloidosis

Takabe¹

2004

Journal of Gastrointestinal Surgery

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“…In cases of systemic disease, however, the only surgery justifiable is emergency surgery. More than half the patients in one series died postoperatively [35]. • Splenectomy may be beneficial in patients with factor X deficiency [36,37].…”

Section: Endoscopic Therapymentioning

confidence: 99%

Gastrointestinal amyloidosis: Approach to treatment

Poullos

Stollman

2003

Curr Treat Options Gastro

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The main treatment goals in amyloidosis are twofold: 1) to diagnose the underlying disease state accurately to guide effective primary therapy (if available) and 2) to ameliorate symptoms. The correct diagnosis is essential because disease-modifying therapies vary widely according to the underlying primary pathology. Primary treatment options remain limited. The best evidence is for high-dose chemotherapy, followed by autologous stem cell transplantation in patients with primary systemic amyloidosis. High-flux hemodialysis (HD) may prevent HD-related amyloidosis. Liver transplantation may be an option for patients with familial amyloidotic polyneuropathy. Several novel specific therapies are under investigation, including small molecule drugs and vaccines. Their efficacy and safety in humans remain to be demonstrated. In the absence of specific cures, symptom-directed therapy assumes a paramount role and can improve quality of life by mitigating diarrhea or pain, for example.

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Amyloidosis - an unusual case of recurrent intestinal bleeding and sigmoid perforation: case report with review of the literature

Cited by 22 publications

References 15 publications

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

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

Successful perioperative management of factor X deficiency associated with primary amyloidosis

Gastrointestinal amyloidosis: Approach to treatment

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