Faculty Profiles - MURAMATSU, Hideki

Papers - MURAMATSU, Hideki

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Natural history of transfusion-independent non-severe aplastic anemia in children Reviewed

Nishio N., Yagasaki H., Takahashi Y., Muramatsu H., Hama A., Yoshida N., Kudo K., Kojima S.

International Journal of Hematology Vol. 89 ( 4 ) page： 409 - 413 2009.5

Language：Japanese Publisher：International Journal of Hematology

Although the known clinical courses of non-severe aplastic anemia (NSAA) in children comprise spontaneous resolution, persistent NSAA, or progression to severe aplastic anemia (SAA), only a few published reports have indicated the outcome of transfusion-independent NSAA. We retrospectively evaluated the incidence and time of progression from transfusion-independent to transfusion-dependent NSAA or SAA. We reviewed the records of 70 children with acquired AA who were referred to our hospital between 1986 and 2006, and among them we found 22 patients who had transfusion-independent NSAA at diagnosis and were treated with supportive care alone until progression to transfusion-dependent AA. 22 patients were followed up for a median of 86 months (range, 11-198 months). The Kaplan-Meier estimates for progression-free survival were 62 ± 12 and 22 ± 13% at 60 and 120 months after diagnosis, respectively. None of the patients treated with supportive care alone improved hematologically. In conclusion, because the incidence of disease progression was high in patients with NSAA, a prospective randomized trial of early intervention with IST or observation alone until disease progression to SAA, followed by IST when the patients become transfusion-dependent is warranted. © 2009 The Japanese Society of Hematology.

DOI： 10.1007/s12185-009-0302-9

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Late-onset non-infectious pulmonary complications following allogeneic hematopoietic stem cell transplantation in children Reviewed

Nishio N., Yagasaki H., Takahashi Y., Muramatsu H., Hama A., Tanaka M., Yoshida N., Watanabe N., Kudo K., Yoshimi A., Kojima S.

Bone Marrow Transplantation Vol. 44 ( 5 ) page： 303 - 308 2009.4

Language：Japanese Publisher：Bone Marrow Transplantation

Late-onset non-infectious pulmonary complications (LONIPCs) that arise beyond 3 months after allogeneic hematopoietic SCT include bronchiolitis obliterans (BO), bronchiolitis obliterans with organizing pneumonia (BOOP) and idiopathic pneumonia syndrome (IPS). We retrospectively analyzed the incidence and outcome of LONIPCs among pediatric hematopoietic SCT recipients. We included 97 patients who survived for more than 3 months among the 114 who underwent allogeneic hematopoietic SCT between April 1997 and May 2007. Of the 97 enrolled patients, 10 (10.3%) developed LONIPCs at a median of 187 days after hematopoietic SCT (range, 123-826 days). Of the 10 patients with LONIPCs, eight had BO and two had IPS. Multivariate analysis showed that the onset of LONIPCs was associated with high-risk underlying disease and extensive chronic GVHD (hazard ratio, 5.42 (95% confidence interval, 1.36-21.7) and hazard ratio, 11.7 (95% confidence interval, 2.40-57.1), respectively). Only two patients responded to therapy with steroids and six of the 10 patients died. The 5-year OS rate was significantly lower among patients with, than without LONIPCs (28.0 vs 87.2%, P = 0.000). Considering that we are lacking optimal therapies for LONIPCs, strategies aimed at the prevention of LONIPCs should be attempted.

DOI： 10.1038/bmt.2009.33

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Correlation of clinical features with the mutational status of GM-CSF signaling pathway-related genes in juvenile myelomonocytic leukemia Reviewed

Yoshida N., Yagasaki H., Xu Y., Matsuda K., Yoshimi A., Takahashi Y., Hama A., Nishio N., Muramatsu H., Watanabe N., Matsumoto K., Kato K., Ueyama J., Inada H., Goto H., Yabe M., Kudo K., Mimaya J., Kikuchi A., Manabe A., Koike K., Kojima S.

Pediatric Research Vol. 65 ( 3 ) page： 334 - 340 2009.3

Language：Japanese Publisher：Pediatric Research

Mutations in RAS, neurofibromatosis type 1 (NF1), and PTPN11, constituents of the granulocyte-macrophage colony-stimulating factor signaling pathway, have been recognized in patients with juvenile myelomonocytic leukemia (JMML). We assessed 71 children with JMML for NRAS, KRAS, and PTPN11 mutations and evaluated their clinical significance. Of the 71 patients, three had been clinically diagnosed with neurofibromatosis type 1, and PTPN11 and NRAS/KRAS mutations were found in 32 (45%) and 13 (18%) patients, respectively. No simultaneous aberrations were found. Compared with patients with RAS mutation or without any aberrations, patients with PTPN11 mutation were significantly older at diagnosis and had higher fetal Hb levels, both of which have been recognized as poor prognostic factors. As was expected, overall survival was lower for patients with the PTPN11 mutation than for those without (25 versus 64%; p = 0.0029). In an analysis of 48 patients who received hematopoietic stem cell transplantation, PTPN11 mutations were also associated with poor prognosis for survival. Mutation in PTPN11 was the only unfavorable factor for relapse after hematopoietic stem cell transplantation (p = 0.001). All patients who died after relapse had PTPN11 mutation. These results suggest that JMML with PTPN11 mutation might be a distinct subgroup with specific clinical characteristics and poor outcome. Copyright © 2009 International Pediatric Research Foundation, Inc.

DOI： 10.1203/PDR.0b013e3181961d2a

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Primary infection of human herpesvirus-6 in an infant who received cord blood SCT Reviewed

Muramatsu H., Watanabe N., Matsumoto K., Ito M., Yoshikawa T., Kato K., Kojima S.

Bone Marrow Transplantation Vol. 43 ( 1 ) page： 83 - 84 2009

Congenital erythroid and myeloid hypoplasia terminating myelodysplastic syndrome Reviewed

Nishio N., Yagasaki H., Takahashi Y., Muramatsu H., Hama A., Xu Y., Villalobos I.B., Kojima S.

Journal of Pediatric Hematology/Oncology Vol. 30 ( 9 ) page： 692 - 695 2008.9

Language：Japanese Publisher：Journal of Pediatric Hematology/Oncology

We describe a 4-month-old infant girl with congenital erythroid and myeloid hypoplasia who developed myelodysplastic syndrome. Bone marrow examination showed severe erythroid and myeloid hypoplasia without dysplastic morphology. Flow cytometry detected autoantibodies to myeloid cells, indicating a diagnosis of Diamond-Blackfan anemia with autoimmune neutropenia. The patient was administered prednisolone and rituximab, which brought the neutrophil count and hemoglobin level to within the normal range. However, bicytopenia recurred at the age of 22 months. She was diagnosed with myelodysplastic syndrome because of trilineage dysplasia and the clonal abnormality of 46,XX,dup(1)(q21;q32) in bone marrow. She was transplanted with cord blood from an unrelated human leukocyte antigen-matched donor and has since remained in complete remission for 20 months. © 2008 by Lippincott Williams & Wilkins.

DOI： 10.1097/MPH.0b013e31816e23a1

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Risk factors for early death in neonates with Down syndrome and transient leukaemia Reviewed

Muramatsu H., Kato K., Watanabe N., Matsumoto K., Nakamura T., Horikoshi Y., Mimaya J., Suzuki C., Hayakawa M., Kojima S.

British Journal of Haematology Vol. 142 ( 4 ) page： 610 - 615 2008.8

Language：Japanese Publisher：British Journal of Haematology

Transient leukaemia (TL) in neonates with Down syndrome (DS) is characterized by the transient appearance of blast cells in the peripheral blood that resolves spontaneously. Some TL patients die at an early age due to organ failure. Seventy DS patients with TL were studied retrospectively to identify clinical and laboratory characteristics associated with early death (<6 months of age). Sixteen of 70 patients (22.9%) died early. The main causes of death were organ failure, particularly hepatic and cardiopulmonary failure. On univariate analysis, early gestational age (EGA), high white blood cell (WBC) count (≥100 × 109/l), percentage of peripheral blasts, elevated aspartate transaminase (AST), elevated direct bilirubin (DB), and low Apgar score were significantly associated with poor survival. On multivariate analysis, EGA, WBC count, and DB were independent predictors of poor outcome. A simple risk stratification system combining EGA and WBC count was devised to predict poor outcome. Term infants (EGA ≥ 37 weeks) whose WBC count was lower than 100 × 109/l had the best outcome [7.7% (3/39) died early], while preterm infants (EGA < 37 weeks) whose WBC count was higher than 100 × 109/l had the worst outcome [54.5% (6/11) died early]. This stratification system may be useful for identifying high-risk patients who need early therapeutic interventions. © 2008 The Authors.

DOI： 10.1111/j.1365-2141.2008.07231.x

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Acute megakaryoblastic leukaemia (AMKL) in children: A comparison of AMKL with and without Down syndrome Reviewed

Hama A., Yagasaki H., Takahashi Y., Nishio N., Muramatsu H., Yoshida N., Tanaka M., Hidaka H., Watanabe N., Yoshimi A., Matsumoto K., Kudo K., Kato K., Horibe K., Kojima S.

British Journal of Haematology Vol. 140 ( 5 ) page： 552 - 561 2008.3

Language：Japanese Publisher：British Journal of Haematology

To characterize childhood acute megakaryoblastic leukaemia (AMKL), we reviewed 45 children with AMKL diagnosed between 1986 and 2005 at Nagoya University Hospital and Japanese Red Cross Nagoya First Hospital. Twenty-four patients (53%) had AMKL associated with Down syndrome (DS-AMKL) and 21 (47%) had non-DS-AMKL. The median age of the DS-AMKL patients was 21 months (range, 8-38 months) and that of non-DS-AMKL patients was 15 months (range, 2-185 months). The morphology of blast cells was categorized into three groups according to the stage of megakaryocyte maturation. The blast cells were more immature in DS-AMKL than in non-DS-AMKL in terms of morphology and immunophenotyping. Cytogenetic abnormalities of leukaemic cells were classified into seven categories: normal karyotype including constitutional trisomy 21 in DS-AMKL; numerical abnormalities only; t(1;22)(p13;q13); 3q21q26 abnormalities; t(16;21)(p11;q22); -5/del(5q) and/or -7/del(7q); and other structural changes. The outcome of children with either DS-AMKL or non-DS-AMKL is excellent. The 10-year overall survival estimate was 79% [95% confidence interval (CI): 54-90] for DS-AMKL and 76% (95% CI: 58-91) for non-DS-AMKL (P = 0.81) with a median follow-up of 78 months (range, 20-243 months). Our study shows the diverse heterogeneity of childhood AMKL and the need for subclassification according to cytogenetic and morphological features. © 2008 The Authors.

DOI： 10.1111/j.1365-2141.2007.06971.x

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Anemia in children Reviewed

Muramatsu H., Kojima S.

Nippon rinsho. Japanese journal of clinical medicine Vol. 66 ( 3 ) page： 544 - 547 2008.3

Language：Japanese Publisher：Nippon rinsho. Japanese journal of clinical medicine

Anemia is one of the most common symptoms in children caused by numerous underlying diseases. In majority of patients, these diseases can be correctly diagnosed through physical examination, history taking, and routine laboratory tests. Bone marrow failure syndromes associated with several genetic diseases are rare causes of anemia in childhood. We reviewed the recent progress of molecular mechanisms in bone marrow failure syndromes, such as Shwachman-Diamond syndrome (SDS), Diamond-Blackfan anemia (DBA), and dyskeratosis congenita (DC), which are all predicted to involve defective ribosome synthesis. Delineation of the precise role of each gene product in ribosomal biogenesis and hematopoiesis may have both therapeutic and prognostic significance.

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[Anemia in children]. Reviewed

Muramatsu H, Kojima S

Nihon rinsho. Japanese journal of clinical medicine Vol. 66 ( 3 ) page： 544 - 7 2008.3

Wiskott-Aldrich syndrome is an important differential diagnosis in male infants with juvenile myelomonocytic leukemialike features Reviewed

Watanabe N., Yoshimi A., Kamachi Y., Kawabe T., Muramatsu H., Matsumoto K., Manabe A., Kojima S., Kato K.

Journal of Pediatric Hematology/Oncology Vol. 29 ( 12 ) page： 836 - 838 2007.12

Language：Japanese Publisher：Journal of Pediatric Hematology/Oncology

A newborn presented with thrombocytopenia at birth and subsequently developed leukocytosis, monocytosis, and mild hepatomegaly. The bone marrow was normocellular with dysplasia and spontaneous granulocyte-monocyte colony formation was demonstrated. These findings fulfilled the diagnostic criteria of juvenile myelomonocytic leukemia. Then he developed atopic dermatitislike eczema, which led to the consideration of Wiskott-Aldrich syndrome (WAS). Lack of intracellular WASP expression and WASP gene mutation confirmed the diagnosis of WAS. After stem cell transplantation, he is alive in good condition with normal WASP expression. WAS should be considered as a differential diagnosis in male infants with juvenile myelomonocytic leukemialike features. © 2007 Lippincott Williams & Wilkins, Inc.

DOI： 10.1097/MPH.0b013e3181580ec5

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Spatially and temporally regulated expression of N-acetylglucosamine-6-O-sulfotransferase during mouse embryogenesis Reviewed

Fan Q.W., Uchimura K., Yuzawa Y., Matsuo S., Mitsuoka C., Kannagi R., Muramatsu H., Kadomatsu K., Muramatsu T.

Glycobiology Vol. 9 ( 9 ) page： 947 - 955 1999.9

Language：Japanese Publisher：Glycobiology

GlcNAc-6-O-sulfotransferase is involved in formation of 6-sulfo-N-acetyllactosamine-containing structures such as 6-sulfo sialyl Lewis x. We investigated the mode of expression of GlcNAc-6-O-sulfotransferase during postimplantation embryogenesis in the mouse by in situ hybridization. Sulfotransferase mRNA was not detected on embryonic day (E) 6.5, while on E7.5 it was detected in the mesoderm, ectoderm, and ectoplacental cone. On E10.5, the sulfotransferase signals were mainly observed in the nervous tissue. On E12.5 and 13.5, various tissues in the process of differentiation expressed this mRNA. Several epithelial and mesenchymal tissues undergoing epithelial-mesenchymal interactions strongly expressed the mRNA. For example, in the developing tooth strong sulfotransferase mRNA expression was found only in the condensing mesenchyme on E13.5. On E13.5 and 15.5, the sites showing intense expression of the sulfotransferase again became restricted. In the brain, sulfotransferase mRNA was frequently found as discrete signals in narrow regions. These results suggest that 6-sulfo-N-acetyllactosamine structures have important roles in development. On E13.5 and 15.5, G152 (6-sulfo sialyl Lewis x antigen) was expressed in the neocortex, and AG223 (6-sulfo Lewis x antigen) in the thalamus and neocortex where the sulfotransferase signal was detected. However, in other organs, expression of these antigens did not correlate with the sulfotransferase mRNA, implicating complex nature of regulation of expression of the fucosyl 6-sulfo antigens.

DOI： 10.1093/glycob/9.9.947

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Human N-acetylglucosamine-6-O-sulfotransferase involved in the biosynthesis of 6-sulfo sialyl Lewis X: Molecular cloning, chromosomal mapping, and expression in various organs and tumor cells Reviewed

Uchimura K., Muramatsu H., Kaname T., Ogawa H., Yamakawa T., Fan Q.W., Mitsuoka C., Kannagi R., Habuchi O., Yokoyama I., Yamamura K.I., Ozaki T., Nakagawara A., Kadomatsu K., Muramatsu T.

Journal of Biochemistry Vol. 124 ( 3 ) page： 670 - 678 1998.9

Language：Japanese Publisher：Journal of Biochemistry

N-Acetylglucosamine-6-O-sulfotransferase catalyzes the transfer of sulfate from 3'-phosphoadenosine 5'-phosphosulfate to position 6 of a non-reducing N-acetylglucosamine (GlcNAc) residue. We have cloned human GlcNAc-6-O-sulfotransferase cDNA, based on the sequence homology to cloned cDNA of mouse GlcNAc-6-O-sulfotransferase. The predicted protein sequence of the human enzyme was highly homologous to that of the mouse enzyme; in the 363 amino acid stretch of the catalytic region, the two proteins were nearly identical except for conservative changes in 3 amino acid residues. The expressed enzyme transferred sulfate to GlcNAcβ1-3Galβ1-4GlcNAcβ1-3Galβ1-4GlcNAc. Co-transfection of the enzyme cDNA and fucosyltransferase VII cDNA into COS-7 cells resulted in cell surface expression of 6-sulfo sialyl Lewis X. Fluorescence in situ hybridization analysis revealed that the GlcNAc-6-O-sulfotransferase gene is located on human chromosome 7q31. mRNA of the human enzyme was strongly expressed in the bone marrow, peripheral blood leukocytes, spleen, brain, spinal cord, ovary, and placenta, and moderate levels of expression were observed in many organs including lymph nodes and thymus. In situ hybridization with the mouse system showed that the transcript was localized in specific regions of the brain, i.e. pyramidal cells in the CA3 subregion of the hippocampus, cerebellar nucleus and Purkinje cells. Among human tumor cells, strong expression of the mRNA was found in MOLT-4 and Jarkat lymphoblastic leukemia cells, Raji lymphoma cells, K-562 chronic myelogeneous leukemia cells, U251 glioma cells, and G361 melanoma cells. Carbohydrate structures synthesized by the sulfotransferase may be involved in various aspects of the differentiation and behavior of blood cells, their progenitor cells, and neurons in the central nervous system.

DOI： 10.1093/oxfordjournals.jbchem.a022164

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Molecular cloning and characterization of an N-acetylglucosamine-6-O- sulfotransferase Reviewed

Uchimura K., Muramatsu H., Kadomatsu K., Fan Q.W., Kurosawa N., Mitsuoka C., Kannagi R., Habuchi O., Muramatsu T.

Journal of Biological Chemistry Vol. 273 ( 35 ) page： 22577 - 22583 1998.8

Language：Japanese Publisher：Journal of Biological Chemistry

We isolated a cDNA clone encoding mouse N-acetyl-glucosamine-6-O- sulfotransferase based on sequence homology to the previously cloned mouse chondroitin 6-sulfotransferase. The cDNA clone contained an open reading frame that predicts a type II transmembrane protein composed of 483 amino acid residues. The expressed enzyme transferred sulfate to the 6 position of nonreducing GlcNAc in GlcNAβ1-31-3Galβ1-4GlcNAc. Galβ1-4GlcNAcβ1-3Galβ1- 4GlcNAc and various glycosaminoglycans did not serve as acceptors. Expression of the cDNA in COS-7 cells resulted in production of a cell-surface antigen, the epitope of which was NeuAcα2-3Galβ1-4(SO4-6)GlcNAc; double transfection with fucosyltransferase IV yielded Galβl-4(Fucal-3)(SO4- 6)Glc-NAc antigen. The sulfotransferase mRNA was strongly expressed in the cerebrum, cerebellum, eye, pancreas, and lung of adult mice. In situ hybridization revealed that the mRNA was localized in high endothelial venules of mesenteric lymph nodes. The sulfotransferase was concluded to be involved in biosynthesis of glycoconjugates bearing the 6-sulfo N- acetyllactosamine structure such as 6-sulfo sialyl Lewis X. The products of the sulfotransferase probably include glycoconjugates with intercellular recognition signals; one candidate of such a glycoconjugate is an L-selectin ligand.

DOI： 10.1074/jbc.273.35.22577

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Mouse chondroitin 6-sulfotransferase: Molecular cloning, characterization and chromosomal mapping Reviewed

Uchimura K., Kadomatsu K., Fan Q.W., Muramatsu H., Kurosawa N., Kaname T., Yamamura K.I., Fukuta M., Habuchi O., Muramatsu T.

Glycobiology Vol. 8 ( 5 ) page： 489 - 496 1998.5

Language：Japanese Publisher：Glycobiology

Chondroitin 6-sulfotransferase (C6ST) catalyzes the transfer or sulfate from 3'-phosphoadenosine 5'-phosphosulfate to position 6 of the N-aceytylgalactosamine residue of chondroitin. Using chick C6ST cDNA as a probe, we cloned the cDNA of mouse C6ST. The mouse enzyme was predicted to be composed of 472 amino acids, and exhibited 71% sequence identity with the chicken enzyme. The mouse and chicken catalytic domains exposed to the luminal side exhibited 81% identity while the homology of the remaining regions was less. Transfection and expression of the mouse cDNA in COS-7 cells yielded C6ST activity. Keratan sulfate sulfotransferase activity which was simultaneously expressed, amounted to 3% of the C6ST activity, this value being significantly lower than that observed in the case of the chicken enzyme. Mouse C6ST mRNA was strongly expressed in the spleen, lung, and eye. In situ hybridization revealed that the transcript was localized in stromal cells in the marginal zone and red pulp of the spleen, and stromal cells in the bone marrow. Fluorescence in situ hybridization analysis revealed the gene is located in mouse chromosome 9.

DOI： 10.1093/glycob/8.5.489

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Papers - MURAMATSU, Hideki