Successful bypass surgery for esophageal carcinoma under adequate factor XIII/13 replacement therapy in a case of intractable autoimmune hemorrhaphilia due to anti-Factor XIII/13 antibodies.

Autoimmune hemorrhaphilia due to anti-factor XIII (FXIII) antibodies (AH13) is a life-threatening disease associated with high risk of surgical bleeding. Since AH13 occurs mainly in the elderly, patients of AH13 tend to be complicated with other life-threatening diseases that may require surgical procedures. During our nation-wide survey on AH13, supported by the Japanese Ministry of Health, Labor, and Welfare, patients with unexplained bleeding were examined for FXIII-related parameters and anti-FXIII autoantibodies.

A case of clonally distinct relapse of Burkitt lymphoma 9 years after complete remission.

We report a case of HIV-negative Burkitt lymphoma (BL) that relapsed 9 years after complete remission. We performed a polymerase chain reaction analysis of three regions of the VDJ junction of the immunoglobulin heavy chain (IGH) gene and compared the clonality of the first and second BL lesions, which were found to be clonally distinct. The patient received the R-Hyper CVAD/R-MA regimen; however, leukoencephalopathy subsequently developed due to the effect of cytarabine, and the regimen was changed to R-IVAM.

Phase I Clinical Trial of Intravenous L-ascorbic Acid Following Salvage Chemotherapy for Relapsed B-cell non-Hodgkin's Lymphoma.

Purpose: To determine the safety and the appropriate dose of intravenous l-ascorbic acid (AA) in conjunction with chemotherapy for patients with relapsed lymphoma.

Patients And Methods: Patients with relapsed CD20-positive B-cell non-Hodgkin's lymphoma, who were going to receive the CHASER regimen as salvage therapy, were enrolled and treated with escalating doses of AA administered by drip infusion after the 2nd course of the CHASER regimen. The target plasma concentration immediately after AA administration was >15 mM (264 mg/dl).

Identification of a novel SEPT9-ABL1 fusion gene in a patient with T-cell prolymphocytic leukemia.

T-cell prolymphocytic leukemia (T-PLL), a rare type of peripheral T-cell leukemia, is characterized by marked splenomegaly with rapidly progressive lymphocytosis and a poor prognosis. Nine kinds of ABL1 chimeric genes have been identified in various kinds of hematological malignancies, such as chronic myeloid leukemia and B- or T-lymphoblastic leukemia. However, there have been no reports describing T-PLL cases with ABL1 rearrangements.

Phase 2 study of arsenic trioxide followed by autologous hematopoietic cell transplantation for relapsed acute promyelocytic leukemia.

The optimal treatments for relapsed acute promyelocytic leukemia (APL) remain equivocal. We conducted a phase 2 study to evaluate the efficacy and feasibility of a sequential treatment consisting of induction and consolidation with arsenic trioxide (ATO), peripheral blood stem cell (PBSC) harvest after high-dose cytarabine chemotherapy, and autologous hematopoietic cell transplantation (HCT). Between 2005 and 2009, 35 patients (26 with hematologic and 9 with molecular relapse) were enrolled.

A Phase I study to assess the safety, pharmacokinetics and efficacy of barasertib (AZD1152), an Aurora B kinase inhibitor, in Japanese patients with advanced acute myeloid leukemia.

Barasertib (AZD1152) is a highly potent and selective Aurora B kinase inhibitor. The safety, efficacy and pharmacokinetic (PK) profile of barasertib were investigated in Japanese patients with advanced acute myeloid leukemia. Barasertib (50-1200mg) was administered as a continuous 7-day intravenous infusion every 21 days.

Three cases of bortezomib-resistant multiple myeloma with extramedullary masses.

In some patients with multiple myeloma, extramedullary masses may be present at diagnosis or may develop during treatment. Recently, multiple myeloma has been treated using newer therapeutic regimens based on thalidomide and bortezomib. Using these drugs, positive responses to treatment, not found with conventional antineoplastic agents, have been reported along with an improvement in patient outcome.

Relationship between heart rate variability using Lorenz plot and sleep level.

In the present study, we propose a new technique for estimating depth of sleep over the whole night using electrocardiogram (ECG) RR intervals (RRIs). We produced a Lorenz plot (LP) using the RRIs recorded during all-night sleep and confirmed that changes in distribution on the LP occur based on changes in sleep stage. To evaluate the changes in these distributions, RRIs are projected a LP on a y = x axis, y = -x axis, and analyzed the shifting of the mean (center C) and standard deviation (area S) for each sleep stage.

Intensified consolidation therapy with dose-escalated doxorubicin did not improve the prognosis of adults with acute lymphoblastic leukemia: the JALSG-ALL97 study.

We designed a treatment protocol for newly diagnosed adult acute lymphoblastic leukemia (ALL) in the pre-imatinib era, employing intensified consolidation therapy with a total of 330 mg/m² doxorubicin and adopting slightly modified induction and maintenance regimen of the CALGB 8811 study. Of 404 eligible patients (median age 38 years, range 15-64 years), 298 (74%) achieved complete remission (CR). The 5-year overall survival (OS) rate was 32%, and the 5-year disease-free survival (DFS) rate was 33%.

[Granulocytic sarcoma of the prostate presenting with urinary obstruction which progressed to acute myeloid leukemia].

A 71-year-old man presented with progressive dysuria. Several imaging examinations indicated possibility of prostate tumor, therefore he underwent prostate biopsy. This resulted in a diagnosis of granulocytic sarcoma of the prostate.

Prognostic significance of FLT3 internal tandem duplication and NPM1 mutations in acute myeloid leukemia in an unselected patient population.

Mutations in the fms-like tyrosine kinase 3 (FLT3) gene containing an internal tandem duplication (FLT3/ITD) or mutations in the nucleophosmin 1 gene (NPM1) are thought to be prognostic indicators in acute myeloid leukemia (AML). Previous studies suggested that FLT3/ITD mutation indicates a poor prognosis and that NPM1 mutation indicates a more favorable one, but these studies were often performed with selected patient populations. We investigated the clinical significance of these mutations at our institution with an unselected group of patients with newly diagnosed AML.

Secondary pulmonary alveolar proteinosis in a patient with chronic myeloid leukemia in the accelerated phase.

Pulmonary alveolar proteinosis (PAP) is a rare respiratory disease the character of which is accumulation of protein consisting of surfactant in alveolar spaces. PAP sometimes complicates with hematological malignancies, especially myeloid leukemia. As one of the cause of PAP, impairment of alveolar macrophage is considered.

Preferential hypermethylation of the Dickkopf-1 promoter in core-binding factor leukaemia.

The Dickkopf-1 (DKK1) gene product is an extracellular Wnt inhibitor. Hypermethylation of the DKK1 promoter results in transcriptional silencing and may play an important role in cancer development. Here, we investigated hypermethylation of the DKK1 promoter in patients with acute myeloid leukaemia (AML), especially core-binding factor (CBF) leukaemia.

Infrequent hypermethylation of WIF-1 promoter in BCR/ABL-negative myeloproliferative disorders.

Objective: The Wnt/β;-catenin signaling pathway is important in the pathogenesis of hematological malignancies. Wnt inhibitory factor-1 (WIF-1) is a negative regulator of Wnt signaling that is frequently downregulated by hypermethylation of the WIF-1 promoter in acute promyelocytic leukemia (APL) and other malignancies. On the other hand, an acquired mutation in JAK2 tyrosine kinase involving a V617F amino-acid substitution shows a strong association with the pathogenesis of BCR/ABL-negative MPD.

[Refractory Evans syndrome complicated with transverse sinus thrombosis].

A 29-year-old woman was diagnosed as having Evans syndrome in 2002 and underwent a splenectomy for the refractory status of the disorder in May 2004. One and a half months after the operation, her platelet count again decreased due to relapse, and she was then prescribed with high dose dexamethasone (38 mg/day x 4 days). Five days after the medication, she complained of a severe headache and then fell into coma, even though her platelet count had risen to 8 x 10(4)/mm3.

Potential and origin of the hematopoietic population in human skeletal muscle.

While mononuclear cells isolated from murine skeletal muscle were shown to be capable of hematopoietic activity, similar hematopoietic cells (HC) recently were reported to exist in primate muscle. We investigated muscle-derived HC from young and adult human subjects. Although hematopoietic stem cells were rare in muscle, their frequency nonetheless was approximately four times greater than in peripheral blood.

Nonhematopoietic mesenchymal stem cells can be mobilized and differentiate into cardiomyocytes after myocardial infarction.

Bone marrow (BM) cells are reported to contribute to the process of regeneration following myocardial infarction. However, the responsible BM cells have not been fully identified. Here, we used 2 independent clonal studies to determine the origin of bone marrow (BM)-derived cardiomyocytes.