Efficacy and Safety of a Weekly Cyclophosphamide-Bortezomib-Dexamethasone Regimen as Induction Therapy Prior to Autologous Stem Cell Transplantation in Japanese Patients with Newly Diagnosed Multiple Myeloma: A Phase 2 Multicenter Trial.

We assessed the efficacy and safety of weekly cyclophosphamide-bortezomib-dexamethasone (CBD) induction prior to autologous stem cell transplantation (ASCT) in newly diagnosed Japanese patients with multiple myeloma (MM). This regimen consisted of four 28-day cycles of once-weekly oral cyclophosphamide (300 mg/m2), subcutaneous bortezomib (1.3 mg/m2), and oral dexamethasone (40 mg).

Final 3-year Results of the Dasatinib Discontinuation Trial in Patients With Chronic Myeloid Leukemia Who Received Dasatinib as a Second-line Treatment.

Introduction: We previously reported an interim analysis of the DADI (dasatinib discontinuation) trial. The results showed that 48% of patients with chronic myeloid leukemia in the chronic phase who maintained a deep molecular response (DMR) for ≥ 1 year could discontinue second- or subsequent-line dasatinib treatment safely at a median follow-up of 20 months. However, the results from longer follow-up periods would be much more useful from a clinical perspective.

Regulatory T cell inhibition by dasatinib is associated with natural killer cell differentiation and a favorable molecular response-The final results of the D-first study.

We evaluated the effects of regulatory T cell (Treg) inhibition during dasatinib treatment on the anticancer immune response, particularly on natural killer (NK) cells and cytotoxic T lymphocytes (CTLs). Fifty-two newly diagnosed Japanese patients with chronic myeloid leukemia (CML) in the chronic phase were enrolled in the D-first study; all received 100 mg of dasatinib once daily and were followed for at least 36 months. The cumulative deep molecular response (DMR, MR4) rate was 65% by 36 months; the 3-year overall survival was 96%.

Familial Dysalbuminemic Hyperthyroxinemia that was Inappropriately Treated with Thiamazole Due to Pseudo-thyrotoxic Symptoms.

We herein report the case of a Japanese woman with familial dysalbuminemic hyperthyroxinemia (FDH) who was initially diagnosed with Graves' disease. Direct genomic sequencing revealed a guanine to cytosine transition in the second nucleotide of codon 218 in exon 7 of the albumin gene, which then caused a proline to arginine substitution. She was finally diagnosed with FDH, which did not require treatment.

Dasatinib rapidly induces deep molecular response in chronic-phase chronic myeloid leukemia patients who achieved major molecular response with detectable levels of BCR-ABL1 transcripts by imatinib therapy.

Background: With the introduction of imatinib, a first-generation tyrosine kinase inhibitor (TKI) to inhibit BCR-ABL1 kinase, the outcome of chronic-phase chronic myeloid leukemia (CP-CML) has improved dramatically. However, only a small proportion of CP-CML patients subsequently achieve a deep molecular response (DMR) with imatinib. Dasatinib, a second-generation TKI, is more potent than imatinib in the inhibition of BCR-ABL1 tyrosine kinase in vitro and more effective in CP-CML patients who do not achieve an optimal response with imatinib treatment.

Association of pleural effusion with an early molecular response in patients with newly diagnosed chronic-phase chronic myeloid leukemia receiving dasatinib: Results of a D-First study.

Despite the efficacy and safety of dasatinib treatment for chronic-phase chronic myeloid leukemia (CML-CP), adverse effects such as pleural effusion (PE) are still a serious concern. We determined the clinical significance of PE incidence using patient data derived from the D-First clinical study. In the present study, chest radiography and quantification of specific lymphocyte subsets were performed routinely after initiation of dasatinib treatment.

Discontinuation of dasatinib in patients with chronic myeloid leukaemia who have maintained deep molecular response for longer than 1 year (DADI trial): a multicentre phase 2 trial.

Background: First-line imatinib treatment can be successfully discontinued in patients with chronic myeloid leukaemia after deep molecular response has been sustained for at least 2 years. We investigated the safety and efficacy of discontinuing second-line or subsequent dasatinib after at least 1 year of deep molecular response.

Methods: The Dasatinib Discontinuation trial was a prospective multicentre trial done in Japan.

Early cytotoxic lymphocyte expansion contributes to a deep molecular response to dasatinib in patients with newly diagnosed chronic myeloid leukemia in the chronic phase: results of the D-first study.

Dasatinib is one of the key treatment options for chronic myeloid leukemia (CML) patients. Increase in lymphocyte counts has been known to be predictive of a good treatment response under dasatinib treatment as a second line therapy. However, clinical significance of lymphocyte dynamics in the upfront setting has yet to be clarified.

Ectopic ACTH syndrome caused by desmopressin-responsive thymic neuroendocrine tumor.

A 32-year-old Chinese woman with rapid weight gain and progressive edema was found to have typical Cushingoid features. Her endocrine data were consistent with a diagnosis of ACTH-dependent Cushing's syndrome. To differentiate ectopic ACTH syndrome (EAS) from Cushing's disease (CD), various dynamic endocrine and imaging tests were performed.

[Myelofibrosis ameliorated after treatment for nontuberculous mycobacterial infection].

A 42-year-old female was admitted to our hospital because of continuous fever, anemia, and immature myeloid cells in peripheral blood. Bone marrow biopsy revealed severe myelofibrosis (MF). We performed computed tomography and identified several swollen mediastinal lymph nodes and nodules in the right upper lung.

Progression of bilateral internal carotid artery dissection during antiplatelet therapy.

Bilateral internal carotid artery dissection (ICAD) is a rare but important cause of stroke in young adults. Anticoagulant and/or antiplatelet agents are usually recommended for stroke prevention;however, such treatments remain highly controversial, and there are inadequate data to compare the efficacy of anticoagulation and antiplatelet therapy. We herein report the case of 30-year-old man presenting with progressive bilateral ICAD during antiplatelet treatment.

Relative increase in lymphocytes from as early as 1 month predicts improved response to dasatinib in chronic-phase chronic myelogenous leukemia.

Lymphocytosis in response to dasatinib for chronic myelogenous leukemia (CML) may be associated with favorable response. However, it occurs at varying times and in a limited subset of patients. To identify early clinical markers for favorable responses applicable to all patients with or without lymphocytosis, we prospectively analyzed lymphocyte profiles of 50 Japanese CML patients treated with dasatinib after intolerance/resistance to imatinib.

Usefulness of procalcitonin for severity assessment in patients with acute cholangitis.

Background: The aim of the present study was to examine the usability of procalcitonin (PCT) for severity assessment in patients with acute cholangitis (AC).

Methods: Serum PCT concentrations were measured on admission in patients with AC. Patients were classified with mild, moderate, or severe AC based on severity assessment guidelines.

Evaluation of indeterminate pulmonary nodules with dynamic MR imaging.

Purpose: We prospectively assessed whether enhancement characteristics on dynamic magnetic resonance (MR) imaging could distinguish indeterminate pulmonary nodules.

Methods: We evaluated 51 pulmonary nodules in 51 consecutive patients (11 female, 40 male; mean age, 64 years) using dynamic MR images acquired at 0, 10, 20, 30, 40, 50, 60, 70, 80, 90, 120, 150, 180, 210, 240, 360, 480, 600, 720, and 840 s following injection of contrast material. We prospectively evaluated morphologic enhancement patterns, peak rate, time to peak enhancement, steepest slope, and washout of nodules and analyzed statistics to determine any differences between MR parameters, patient age, tumor size, and final diagnosis.

[Subarachnoid hemorrhage due to a ruptured intracranial vertebral artery aneurysm associated with cerebrovascular fibromuscular dysplasia: case report].

Fibromuscular dysplasia (FMD) is comprised of a group of nonatherosclerotic and noninflammatory arterial diseases. Cerebrovascular FMD occurs more frequently in women, and the mean age at which it is diagnosed is 50 years. The most common angiographic pattern of cerebrovascular FMD is the "string-of-beads" deformity at the extracranial internal carotid artery.

Pulmonary emphysema: histopathologic correlation with minimum intensity projection imaging, high-resolution computed tomography, and pulmonary function test results.

Objective: To prospectively evaluate the use of minimum-intensity projection (minIP) imaging, high-resolution (HR) computed tomography (CT), and pulmonary function tests for quantifying emphysema with histopathologic examination.

Methods: MinIP and HRCT imaging data (n = 23) were obtained, and relative areas of the lung with attenuation values below thresholds from -940 to -1000 Hounsfield units (HU) and first to 13th percentiles were calculated for both data. Pulmonary function tests were performed before lung resection.

Can malignant and benign pulmonary nodules be differentiated with diffusion-weighted MRI?

Objective: The objective of our study was to evaluate whether diffusion-weighted imaging (DWI) with a high b factor can be used to differentiate malignancies from benign pulmonary nodules.

Materials And Methods: This study included 54 pulmonary nodules (>or= 5 mm in diameter) in 51 consecutive patients (37 men, 14 women; mean age, 65.7 years; age range, 31-88 years).

[Pure red cell aplasia successfully treated with rituximab in a patient with relapsed diffuse large B-cell lymphoma].

Malignant lymphoma is often accompanied by an autoimmune disorder. Here, we describe a 82-year-old woman with pure red cell aplasia (PRCA) complicated with relapsed diffuse large B-cell lymphoma. Primary treatment involving chemotherapy, continuous oral administration of prednisolone and a single infusion of rituximab was unsuccessful for both diseases, but the following treatments with 3 courses of rituximab alone for once a week dramatically improved the PRCA as well as the lymphoma.

Sibling cases of Addison's disease caused by DAX-1 gene mutations.

We report two sibling cases of Addison's disease without any evidence of sexual precocity, adrenal hyperplasia, or autoimmune disease. The diagnosis of primary adrenocortical insufficiency was made at the age of 5 in the younger brother and at the age of 18 in the elder brother. The younger brother had been inactive during infancy and had diffuse skin pigmentation without abnormal external genitalia, while the elder brother had been healthy until the age of 17 when he noticed skin pigmentation and small testes.

Icodextrin reduces mortality and the drop-out rate in Japanese peritoneal dialysis patients.

Although numerous reports have shown that the use of icodextrin solution, as compared with conventional dextrose solutions, provides various clinical benefits, data on the impact of icodextrin solution on mortality and drop-out are sparse. In the present retrospective study, we compared clinical outcomes in a large cohort of patients prescribed either icodextrin or dextrose solution for the long dwell. A total of 7808 patients across Japan who were using Baxter peritoneal dialysis (PD) solutions in 2004 were included in this cross-sectional analysis.

Sliding thin slab, minimum intensity projection imaging for objective analysis of emphysema.

Purpose: The aim of this study was to determine whether sliding thin slab, minimum intensity projection (STS-MinIP) imaging is more advantageous than thin-section computed tomography (CT) for detecting and assessing emphysema.

Materials And Methods: Objective quantification of emphysema by STS-MinIP and thin-section CT was defined as the percentage of area lower than the threshold in the lung section at the level of the aortic arch, tracheal carina, and 5 cm below the carina. Quantitative analysis in 100 subjects was performed and compared with pulmonary function test results.

Sliding thin slab, minimum intensity projection of the lung in asymptomatic subjects: lower limits of lung attenuation, without airways.

Purpose: A sliding thin slab, minimum intensity projection (STS-MinIP) is considered to be useful for detecting diseases that decrease lung attenuation. For evaluating these diseases, it would be useful to ascertain the lower limits of normal lung attenuation, allowing a division between normal and subnormal attenuation. However, normal lung attenuation may vary depending on respiratory status, anatomical position, and patient background factors.

[Case of hemophagocytic syndrome associated with active dermatomyositis].

We report a case of dermatomyositis (DM) with hemophagocytic syndrome (HPS). The patient is a 60 year old male admitted to our hospital with muscle weakness, high fever, weight loss and pancytopenia. On physical examination, proximal muscle weakness and skin rash on the back were noted.

[Anesthetic managements of the patients receiving catheterizable cecostomy].

To improve the quality of life of patients suffering from spinal cord injury, "Catheterizable cecostomy" operation have recently been carried out. In the procedure, cathetelizable stoma is opened and patients are able to control the excretion by injecting enema solution through the stoma. Although operations are usually performed under general anesthesia with tracheal intubation, special considerations sometimes need to be taken for airway management.

Reverse 'see-saw' relationship between Graves' disease and myasthenia gravis; clinical and immunological studies.

Although the association of two distinct autoimmune diseases, Graves' disease (GD) and myasthenia gravis (MG), is rare, the relationships of clinical and immunological activities between the two diseases remain unknown. In the present study, we investigated whether there exist any relationships between clinical and immunological activities of GD and MG as well as any common characteristics of their HLA antigens in five patients with concomitant association with GD and MG. The present study clearly showed positive relationships between the clinical activities of GD and MG in all five cases.