He received conditioning with cytarabine (ara-C), BU/ fludarabine, and melphalan, followed by unrelated cord blood transplantation. fibrils predominantly show an antiparallel ?-pleated sheet conformation. Thus far, more than 30 proteins have been shown to form amyloid fibrils (1). The most frequent type of amyloidosis is amyloid light chain (AL) amyloidosis. AL amyloidosis is a clonal hematological disorder that arises when antibody-producing cells function incorrectly and produce abnormal protein fibers composed of immunoglobulin light chains which are deposited as amyloid fibrils. It is a systemic disorder that can present with various symptoms, including proteinuria, edema, unexplained heart failure, neuropathy, skin manifestations, and bleeding diathesis. Over the past decade, the median survival of patients with AL amyloidosis has nearly doubled (2). In 2004, the median survival time of patients with AL amyloidosis was only 2.2 years, and the estimated 4-year overall survival rate was 38% (3). The treatment of AL amyloidosis targets the clonal bone marrow plasma cells using the same methods that are employed for multiple myeloma (3). On 2004, as treatment for AL amyloidosis, induction chemotherapy with a combination of vincristine, adriamycin, and dexamethasone (VAD) prior to autologous stem cell transplantation with high-dose melphalan, autologous stem cell transplantation (HDM/SCT) was proposed by Perz et al. (4). A close association has been reported between AL amyloidosis and factor X deficiency. Systemic AL amyloidosis patients with reduced factor X levels is associated with increased hemorrhagic morbidity (5). In 2004, we initiated HDM/SCT therapy for a patient who had AL amyloidosis combined with severe factor X deficiency according to the report of Perz et al. (4). We herein report the patient’s long-term outcome in the 10 years after this therapy was administered. Case Report In 2004, a 55-year-old Japanese man was admitted to our hospital to undergo evaluation for proteinuria and weight loss. On admission, he was 168.0 cm tall and weighed 58.0 Lu AF21934 kg (he had lost 5 kg of body weight during the previous 5 months). His blood pressure was 100/70 mmHg, his pulse rate was 90 beats/min, and his body temperature was 36.6. Laboratory tests revealed the following: serum albumin, 2.5 g/dL; total protein, 5.4 g/dL; urea nitrogen (UN), 10 mg/dL; serum creatinine (Cre), 0.60 mg/dL; and estimated glomerular filtration rate (eGFR), 107.0 mL/min/1.73 m2) (Table). The patient’s C-reactive protein (CRP) level was 0.1 mg/dL, and his serum amyloid A protein (SAA) was 2.0 g/mL (normal: 13.0). Coagulation tests showed an activated partial thromboplastin time (APTT) of 51.3 seconds (normal range: 27.0-40.0 seconds), a prothrombin time of 21 seconds (normal range: 70 seconds), and a prothrombin time-international normalized ratio (PT-INR) of 1 1.77; however, clinical bleeding was not noted. Abnormal coagulation was observed because the patient’s factor X activity was reduced to 15.2% (normal range: 70-130%); his other coagulation factors were normal. An immunological evaluation revealed the following: IgG, 421 mg/dL (normal range: 870-1,700 mg/dL); IgA, 83.6 mg/dL (normal range: 110-410 mg/dL); and IgM, 47.4 mg/dL (normal range: 35-220 mg/dL). The patient was negative for all autoantibodies, including antinuclear antibodies and anti-cardiolipin-2 glycoprotein I complex antibodies. Immunofixation revealed that the patient was negative for serum M-protein. A urinalysis detected proteinuria of (5.36 g/day), while protein electrophoresis showed that albumin accounted for 57.5% and the patient was positive for lambda-type Bence-Jones protein (BJP). Table. Laboratory Tests before HDM/SCT and at 3, 5, 10, 11 and 13 Years Lu AF21934 after Transplantation. thead style=”border-top:solid thin; border-bottom:solid thin;” th valign=”middle” align=”left” rowspan=”1″ colspan=”1″ /th th valign=”middle” align=”center” rowspan=”1″ colspan=”1″ Before /th Lu AF21934 th valign=”middle” align=”center” rowspan=”1″ colspan=”1″ after 3 years /th th valign=”middle” align=”center” rowspan=”1″ colspan=”1″ after 5 years /th th valign=”middle” align=”center” rowspan=”1″ colspan=”1″ after 10 years /th th valign=”middle” align=”center” rowspan=”1″ colspan=”1″ after 11 years /th th valign=”middle” align=”center” rowspan=”1″ colspan=”1″ after 13 years /th th valign=”middle” align=”center” rowspan=”1″ colspan=”1″ normal range /th /thead Total protein, g/dL5.466., g/dL2., mg/dL1021212021248-20Creatinine, mg/dL0.61111.071.160.4-0.6eGFR10760.559.958.552.849.1 100White Kcnj12 blood cell /L6,5005,9005.44.26.16,6003,400-9,200Red blood cell, 104/L3.23.253.413.553.3400-566Hb, g/dL13.711.21212.612.210.313.0-17.0Platelet, 104/L37.113.813.910.912.815.514.1-32.7IgG, mg/dL421558965984904870-1,700IgA, mg/dL8415421249.5110-410IgM, mg/dL48711145335-220serum M protainnegativenegativenegativenegativenegativepositivenegativeAPTT, second51.33025.329.53327-40PT, second21114109.494.879.3 70INR1.80.941.921.13factors X activity, %12.910570-130urinary protain (g/day) 0.1urunary BJPpositivenegativenegativenegativenegativepositivenegativeLVIDd, mm5054464634-54IVST, mm159911.46-10PWT, mm1311109.26-10LVM, g276206148162122-174 Open in a separate window LVIDd: left ventricular internal diameter at end-diastole, IVST: interventricular septal thickness, PWT: posterior wall thickness, LVM: left ventricular mass, UN: urea nitrogen, eGFR: estimated glomerular filtration rate, APTT: activated partial thromboplastin time, PT: prothrombin?time, PT-INR: prothrombin?time-international normalized ratio, BJP: Bence-Jones protein Low voltage electrocardiography was performed. Echocardiography revealed generalized cardiac hypertrophy and the left ventricular mass was found to be 276 g by the formula of Devereux: 0.8[1.04(IVSTPWVLVIDd)3-LVIDd3]+0.6. IVST: interventricular septal thickness, PWV: posterior wall thickness, LVIDd: left ventricular internal diameter at end-diastole. These findings were consistent with cardiac amyloidosis. Renal biopsy was performed to evaluate the pathogenesis of nephrotic-range proteinuria. Renal biopsy A.