American Journal of Hematology 81:281–283 (2006)
Successful Reduced Intensity Allogeneic Stem Cell Transplantation for Systemic AL Amyloidosis Tomoyuki Imamura,1* Masao Ogata,1 Kazuhiro Kohno,1 Tadashi Tomo,1 Eiichi Ohtsuka,1 Hiroshi Kikuchi,2 and Jun-ichi Kadota1 1
Division of Pathogenesis and Disease Control, Department of Infectious Diseases, Oita, Japan 2 Blood Transfusion Center, Oita University Faculty of Medicine, Oita, Japan
No established treatments for systemic AL amyloidosis have been determined, and only four reports have described allogeneic stem cell transplantation for this disease. We report the case of a patient with orthostatic hypotension, diarrhea, nephrotic syndrome, and cardiac amyloidosis due to systemic AL amyloidosis. Reduced intensity allogeneic stem cell transplantation (RIST) was performed using a conditioning regimen comprising fludarabine 125 mg/m2 and melphalan 90 mg/m2. Hematologically complete remission and symptomatic improvement were obtained without severe transplantation-related complications. RIST may thus offer a useful treatment strategy for systemic AL amyloidosis complicated by cardiac C 2006 Wiley-Liss, Inc. amyloidosis. Am. J. Hematol. 81:281–283, 2006. V Key words: systemic AL amyloidosis; reduced intensity allogeneic stem cell transplantation
INTRODUCTION
Systemic AL amyloidosis is a protein conformation disorder associated with clonal plasma cell dyscrasia, resulting in multisystem failure and death [1]. Less than 5% of all patients with systemic AL amyloidosis survive 10 years from the time of diagnosis. Several recent studies have reported the efficacy of autologous peripheral blood stem cell transplantation (PBSCT) for patients with systemic AL amyloidosis [2–7]. However, PBSCT can only be offered to a small proportion of patients, due to the high transplantrelated mortality (TRM), particularly in patients with cardiac amyloidosis. Only four reports have described allogeneic stem cell transplantation (SCT) for patients with systemic AL amyloidosis [8–11], and only one of the patients in these reports displayed cardiac amyloidosis. We report herein successful reduced intensity allogeneic stem cell transplantation (RIST) for a patient with both systemic AL amyloidosis and cardiac amyloidosis.
in November 2000, followed by diarrhea and oligohydrosis in August 2002. Light-headedness and fainting on standing began in November 2002 and progressed. Orthostatic hypotension was severe enough that, in a standing position, blood pressure could not be measured and the patient would faint. He was unable to move from bed. Blood counts revealed a leukocyte count of 5,000/lL; hemoglobin concentration, 11.1 g/ dL; erythrocyte count, 347 104/lL; and platelet count, 26.5 104/lL. Blood chemistry indicated total protein, 5.05 g/dL; albumin, 3.50 g/dL; b2-microglobulin, 2.4 mg/L; and C-reactive protein (CRP) was 0.30 mg/dL. Serum c-globulins were decreased (IgG, 675.9 mg/dL; IgA, 57.2 mg/dL; IgM, 20.6 mg/dL) with an IgD- monoclonal component (210 mg/dL) and without free light chain by immunoelectrophoresis.
*Correspondence to: Tomoyuki Imamura, M.D., Division of Pathogenesis and Disease Control, Department of Infectious Diseases, Oita University Faculty of Medicine, Hasama-machi, Oita 879-5593, Japan. E-mail:
[email protected]
CASE REPORT
Received for publication 14 March 2005; Accepted 22 August 2005
A 43-year-old Japanese man presented in April 2003 with orthostatic hypotension. He had noted impotence
Published online in Wiley InterScience (www.interscience.wiley.com). DOI: 10.1002/ajh.20544
C 2006 Wiley-Liss, Inc. V
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Case Report: Imamura et al.
TABLE I. Course of Laboratory Findings Days pre-RIST
Serum creatinine (mg/dL) Creatinine clearance (mL/min) Serum total protein (g/dL) Serum albumin (g/dL) Proteinuria (g/day) IgD In serum In urine BJP-l In serum In urine BNP (pg/mL)
Days after-RIST
190
100
0
100
200
300
400
500
600
1.03 87.7 5.05 3.5 1.17
0.71 73.3 3.81 2.35 3.21
0.61 100.6 3.48 2.37 5.4
0.89 63.4 3.43 2.15 2.37
0.89 48.6 3.69 2.44 2.44
1.02 62.5 3.67 2.34 3.77
0.83 67.3 3.56 2.43 2.34
0.96 107.7 4.68 3.16 2.05
0.99 72.4 6.11 3.41 0.89
21.0
49.5
41.8
12.7
