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Summary:AL amyloidosis is a plasma cell disorder in which tissue deposition of immunoglobulin light chains leads to organ dysfunction. Recent reports of high-dose therapy with autologous stem cell transplantation for amyloidosis suggest higher response rates and extended survival compared to those seen with conventional chemotherapy. However, substantial treatment-related toxicity has been observed. This case series describes our institutional experience with autologous transplantation in four patients with amyloidosis with an emphasis on unique gastrointestinal toxicities, including toxic megacolon. Bone Marrow Transplantation (2002) 30, 279-285. doi:10.1038/sj.bmt.1703627 Keywords: AL amyloidosis; high-dose chemotherapy; stem cell transplantation; toxic megacolon AL amyloidosis is monoclonal plasma cell disorder characterized by tissue deposition of immunoglobulin light chains, leading to dysfunction of organs including the heart, kidneys, gastrointestinal (GI) tract, liver, soft tissues and nerves. In one series of 474 patients, the median survival after diagnosis was 13.2 months without treatment.1 Conventional therapies aimed at the underlying plasma cell disorder have produced modest gains, with response rates of 30% and an extension of median survival by only 6 months.2,3 Over the past several years, trials of high-dose therapy and autologous stem cell transplantation have reported response rates up to 55-100% and a median survival exceeding 12 to 24 months. [4][5][6][7][8][9][10][11][12][13][14][15][16] These data have led clinicians to increasingly consider AL amyloidosis as an indication for autologous transplantation. Unfortunately, substantial treatment-related toxicity has been reported in patients with amyloidosis undergoing autologous transplantation. This series describes our institutional experience with autologous transplantation in patients with amyloidosis and focuses on the complication of toxic megacolon. Patients and methodsFour patients received high-dose therapy and autologous stem cell transplantation at Oregon Health and Science University. Two patients were treated for primary AL amyloidosis, one for multiple myeloma with AL amyloidosis, and one patient for multiple myeloma who was later found to have AL amyloidosis (see details in Table 1). Patient 1A 57-year-old man presented with 18 months of progressive hand and finger swelling, nail changes, and skin fragility. His history was also notable for macroglossia with obstructive sleep apnea, and bilateral carpal tunnel release surgeries. A skin biopsy confirmed AL amyloid deposition. Serum protein electrophoresis (SPEP) did not reveal a monoclonal protein, but a bone marrow biopsy showed 16% lambda-restricted plasma cells without evidence of amyloid deposition and a 24 h urine collection showed 800 mg of Bence-Jones proteinuria. No lytic lesions were identified on bone survey.The patient was treated with vincristine, adriamycin, and dexamethasone (VAD) chemotherapy, followed by cyclophosphamide, etoposide and decadron (CED) chemotherap...

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