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Amyloidosis Toxic Megacolon: a Life-Threatening Complication of High-Dose Therapy and Autologous Stem Cell Transplantation Among Patients with AL Amyloidosis

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Amyloidosis Toxic Megacolon: a Life-Threatening Complication of High-Dose Therapy and Autologous Stem Cell Transplantation Among Patients with AL Amyloidosis Bone Marrow Transplantation (2002) 30, 279–285 2002 Nature Publishing Group All rights reserved 0268–3369/02 $25.00 www.nature.com/bmt Amyloidosis Toxic megacolon: a life-threatening complication of high-dose therapy and autologous stem cell transplantation among patients with AL amyloidosis BM Hayes-Lattin, PT Curtin, WH Fleming, JF Leis, DE Stepan, S Schubach and RT Maziarz Adult Bone Marrow Transplant Program, Division of Hematology and Medical Oncology, Oregon Health and Science University, Portland, OR, USA Summary: with autologous transplantation in patients with amylo- idosis and focuses on the complication of toxic megacolon. 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 Patients and methods with autologous stem cell transplantation for amylo- idosis suggest higher response rates and extended sur- Four patients received high-dose therapy and autologous vival compared to those seen with conventional chemo- stem cell transplantation at Oregon Health and Science Uni- therapy. However, substantial treatment-related toxicity versity. Two patients were treated for primary AL amylo- has been observed. This case series describes our insti- idosis, one for multiple myeloma with AL amyloidosis, and tutional experience with autologous transplantation in one patient for multiple myeloma who was later found to four patients with amyloidosis with an emphasis on have AL amyloidosis (see details in Table 1). unique gastrointestinal toxicities, including toxic mega- colon. Patient 1 Bone Marrow Transplantation (2002) 30, 279–285. doi:10.1038/sj.bmt.1703627 A 57-year-old man presented with 18 months of progress- Keywords: AL amyloidosis; high-dose chemotherapy; ive hand and finger swelling, nail changes, and skin fra- stem cell transplantation; toxic megacolon gility. 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 AL amyloidosis is monoclonal plasma cell disorder charac- monoclonal protein, but a bone marrow biopsy showed terized by tissue deposition of immunoglobulin light chains, 16% lambda-restricted plasma cells without evidence leading to dysfunction of organs including the heart, kid- of amyloid deposition and a 24 h urine collection showed neys, gastrointestinal (GI) tract, liver, soft tissues and 800 mg of Bence-Jones proteinuria. No lytic lesions were nerves. In one series of 474 patients, the median survival identified on bone survey. 1 after diagnosis was 13.2 months without treatment. Con- The patient was treated with vincristine, adriamycin, and ventional therapies aimed at the underlying plasma cell dis- dexamethasone (VAD) chemotherapy, followed by cyclo- order have produced modest gains, with response rates of phosphamide, etoposide and decadron (CED) chemo- 30% and an extension of median survival by only 6 therapy and G-CSF stem cell mobilization by which 37.3 2,3 months. Over the past several years, trials of high-dose ϫ 106 CD34+ cells/kg were collected and cryopreserved. therapy and autologous stem cell transplantation have Six months after the initiation of primary therapy, the reported response rates up to 55–100% and a median sur- patient received high-dose therapy with busulfan 4 mg/kg 4–16 vival exceeding 12 to 24 months. These data have led four times a day for 3 days, melphalan 50 mg/m2 daily for clinicians to increasingly consider AL amyloidosis as an 2 days, and thiotepa 250 mg/m2 daily for 2 days, followed indication for autologous transplantation. Unfortunately, by autologous transplant with 19.3 ϫ 106 CD34+ cells/kg. substantial treatment-related toxicity has been reported in The duration of severe neutropenia was 10 days, compli- patients with amyloidosis undergoing autologous transplan- cated by fever and grade 3 mucositis (NCI scale17). Within tation. This series describes our institutional experience 2 weeks of transplant, the patient had responded with near complete resolution of macroglossia and dermopathy. On day +14, he developed watery diarrhea, which responded Correspondence: Dr RT Maziarz, Oregon Health and Science University, to treatment with tincture of opium and pulsed steroids for 18 3181 SW Sam Jackson Park Road, Portland, OR 97201, USA possible autologous graft-versus-host disease. However, Received 18 December 2001; accepted 25 March 2002 on day +18, the diarrhea returned with frank hematochezia. Toxic megacolon after transplant for amyloidosis BM Hayes-Lattin et al 280 Table 1 Patient characteristics Age at Sex Transplant Amyloid Pre-transplant Stem cell Conditioning Outcome transplant diagnosis involvement therapy mobilization regimen 58 M Multiple myeloma Skin (biopsy), GI VAD ϫ 5 cycles CED with G-CSF Busulfan Toxic megacolon, with AL amyloidosis (macroglossia, Melphalan died day +81 biopsy), bone Thiotepa marrow (biopsy) 52 M AL amyloidosis Renal (biopsy), bone G-CSF, Melphalan Multi-organ system marrow (biopsy), G-CSF/GM-CSF failure, died day +20 liver (enlargement, alk phos), peripheral nerves (symptoms) 56 M Multiple myeloma GI (biopsy), bone Oral melphalan and CED with G-CSF Busulfan Toxic megacolon, marrow (biopsy) prednisone, VAD ϫ Thiotepa died 14 months post- 4 cycles, TBI transplant with cyclophosphamide ϫ relapse 3 cycles 65 M AL amyloidosis GI (macroglossia, VAD ϫ 3 cycles Cyclophosphamide Melphalan Mucositis, alive 20 oral biopsy), skin, with G-CSF months post- renal (proteinuria) transplant, partial remission Assays for ova and parasites, bacterial and viral pathogens, platelet transfusion support with gradual recovery, and was and C. difficile toxin were negative. Colonoscopy revealed discharged on day +25. ulceration and biopsies showed changes consistent with He was re-admitted on day +28 with fever and dehy- chronic ischemic colitis and focal amyloid deposition in dration. Blood cultures revealed a coagulase-negative vessel walls (Figure 1). The patient received red cell and Staphylococcal species, which was treated with intravenous antibiotics and removal of his central catheter. The fevers persisted and he developed abdominal cramping with wat- ery diarrhea and distention of the bowel of up to 8 cm on radiographs. He was treated with narcotic withdrawal, bowel rest, intravenous fluids, and additional empiric anti- biotics. A tagged WBC scan showed persistent tracer activity in the distal transverse colon, but a CT scan showed only mild ascites without visible abscess formation. Again, multiple stool cultures and C. difficile toxin assays were negative. Over 1 week, his diarrhea slowed and he remained afebrile after stopping the antibiotics. On day +33, the patient developed new lower abdominal pain with urinary symptoms and was found to have adenoviral cys- titis. With controlled diarrhea on bowel rest and total par- enteral nutrition, he was discharged from the hospital on day +50, but continued to suffer persistent nausea and vom- iting with any oral intake. On day +78, he developed acute jaundice and multiple new subcutaneous masses. He declined further treatment and died 81 days post transplant. A post-mortem examination was not performed. Patient 2 A 51-year-old man presented with hypertension and pro- teinuria, which were treated medically. One year later, he noted intermittent nausea and vomiting with anorexia and an intervening 62-pound weight loss. He also complained of persistent lower extremity edema, easy bruising, decreased exercise tolerance, paresthesias in his feet, and Figure 1 Colitis. Colonoscopy to evaluate bloody diarrhea 18 days after autologous transplantation for multiple myeloma with AL amyloidosis several episodes of diarrhea lasting several days at a time. revealed colitis with deep (a) and linear (b) ulcerations. Biopsies con- The serum creatinine was 1.9 mg/dl. A 24 h urine collection firmed presence of amyloid deposition. revealed a creatinine clearance of 80 ml/min and pro- Bone Marrow Transplantation Toxic megacolon after transplant for amyloidosis BM Hayes-Lattin et al 281 teinuria of 12.9 g. Renal biopsy showed negative Congo patient received therapy for multiple myeloma with oral red staining, but positive staining of mesangial deposits melphalan and prednisone. Three years later, after rising with anti-lambda light chain antibodies. Electron serum paraprotein levels, he was treated with VAD chemo- microscopy revealed fibrillary glomerular deposits with therapy and achieved only a partial response followed by ultrastructural features and dimensions consistent with three cycles of intermediate-dose cyclophosphamide, which amyloid. A bone marrow biopsy demonstrated normal reduced his bone marrow involvement to 11% plasma cells. cellularity and composition with aggregates of small He underwent hematopoietic stem cell mobilization using eosinophilic nodules which stained positively with Congo CED chemotherapy with G-CSF, and 8.3 ϫ 106 CD34+ red and were faintly birefringent, consistent with amyloid. cells/kg were harvested by leukopheresis. There was no detectable monoclonal protein in serum or Four years from initial diagnosis, he received high-dose urine. He had palpable hepatomegaly and an isolated elev- therapy with busulfan 4 mg/kg in divided daily doses for ation of the serum alkaline phosphatase. An echocardiog- 2 days, thiotepa 200 mg/m2 daily for 2 days, and total body ram showed normal myocardial wall thickness and normal irradiation
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