Junji Tomiyama

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Upshaw-Schulman syndrome (USS) is a congenital thrombotic thrombocytopenic purpura (TTP) due to mutations in the gene that encodes for ADAMTS13 (ADAMTS13), but its clinical signs may be mild or absent during childhood. We have identified 37 patients with USS (24 females, 13 males) belonging to 32 families. The nine women from six families who were diagnosed(More)
Debate exists over whether CD7 expression indicates an unfavourable prognosis in de novo acute myeloid leukaemia (AML). Meanwhile, the type of cytogenetics is a strong prognostic factor in AML. We analysed 256 de novo adult AML cases and found that the proportion of CD7+ cases increased stepwise from the cases with favourable cytogenetics to the cases with(More)
We here report a rare case of dual antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in a 38-year-old Japanese woman previously diagnosed with mixed connective tissue disease. The patient was found to be positive for myeloperoxidase- and proteinase 3-ANCA, and was diagnosed with AAV following admission to hospital with fervescence,(More)
A 25-year-old female meeting all six criteria for Kawasaki disease is reported. A total of 22 reported cases of adult Kawasaki disease, including the present case, are reviewed. In adult Kawasaki disease, arthralgia, gastrointestinal complications and hepatic dysfunction are seen more frequently than in childhood cases. Cardiac complications are rarely seen(More)
Various prospective trials have been performed to assess the roles of allogeneic hematopoietic cell transplantation (allo-HCT) and chemotherapy in patients with acute myeloid leukemia (AML) in first complete remission (CR1). However, the results have not always been consistent, and there has been a limited evaluation of quality of life (QOL) in these(More)
Juzen-Taiho-To (JTT) is a Japanese herbal medicine that has been administered mainly to patients weakened by long illness. Currently, it has also been used for cancer patients and showed antitumor effects that have been reported as phagocytosis enhancement, cytokine induction and antibody production. In this study, we examined the effect of oral(More)
Turcot syndrome is characterized by an association of malignant brain tumors and colon cancer developing in the patient's teens. Since the mechanism of carcinogenesis in Turcot syndrome is still unclear, we analysed genetic changes in tumors from a Turcot patient with no family history of the condition. All tumors, including one astrocytoma, three colon(More)
We describe a 60-year-old woman who developed mixed connective tissue disease (MCTD) associated with multicentric Castleman's disease (MCD) and Crow-Fukase syndrome (CFS). She showed HLA DR-4 antigen and an abnormal X chromosome (47,XXX). The serum interleukin-6 (IL-6) level was markedly increased and IL-6 mRNA was detected in enlarged lymph node cells.(More)
To determine the antileukemic effect of N4-behenoyl-1-beta-D-arabinofuranosylcytosine (BH-AC), a synthetic masked compound of 1-beta-D-arabinofuranosylcytosine, the pharmacokinetics and suppressive effect on leukemic blast progenitors of BH-AC were studied. When BH-AC was added to the suspension culture of leukemic cells, BH-AC gradually decreased in(More)