[Clinical analysis of autologous stem cell transplantation for nine cases of cardiac amyloidosis].

Long-term survival remains poor for patients with cardiac amyloidosis. High-dose melphalan (MEL) with stem cell transplantation (HDM/SCT) is an effective treatment for AL amyloidosis, but patients with cardiac involvement are ineligible because of high therapy-related mortality. Here we report detailed HDM/SCT outcomes of 9 patients with cardiac failure. Their median age was 56 years (range, 45∼66). After a median follow-up of 15 months (range 9∼32), three died of multiorgan failure within the early phase after HDM/SCT, and the other six including poor risk patients are alive at present. Their symptoms of cardiac decompensation have improved. Decreases in interventricular septum thickness were confirmed in 4 patients 6∼12 months after HDM/SCT by echocardiography. One-year overall survival rate was 67%, longer than previously reported rates. HDM/SCT may lead to improvements in quality of life and extended survival in cardiac amyloidosis patients. Meanwhile, the median dosage of MEL in our procedure was 103 mg/m(2) (range 68∼180), less than the recommended dose, and patients were maintained on miscellaneous therapies. Further studies are required to clarify an effective MEL dose and to refine selection criteria for patients undergoing HDM/SCT.

[Increased level of KL-6 in a BJP-λ-type multiple myeloma patient with poor prognosis].

A 63-year-old female with BJP-multiple myeloma (Durie-Salmon stage III B, International Staging System III) showed an increased level of KL-6, a sialylated carbohydrate antigen that is a MUC1 molecule expressed in type II pneumocytes and reflects activity of interstitial pneumonia. At the time of diagnosis, KL-6 was as high as 22,030 U/ml; however, surfactant protein D (SP-D) was normal, and stroma-related pneumonia was not indicated on CT images. Expression of KL-6 in multiple myeloma cells was detected by immunostaining and the patient was diagnosed with KL-6-positive multiple myeloma. Usually, MUC1 is encoded by chromosome 1q21, but the karyotypic analysis of the patient's bone marrow cells lacked chromosome 1. KL-6 increased as the disease progressed. The patient did not respond to chemotherapy, including bortezomib, showed an increase of pleural effusion, and died. For this patient, multiple myeloma with high KL-6 was refractory to chemotherapy, suggesting that new treatment strategies, including transplantation of hematopoietic stem cells, are required.