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Biomedical subjects

S Ohga

Publications and source records attributed to S Ohga.

At least 55 records · Page 3Linked to original sources
PubMed1997

Prolonged resolution of hemophagocytic lymphohistiocytosis following myeloablative chemotherapy and subsequent autologous peripheral blood stem cell transplantation.

A 30-month-old boy with hemophagocytic lymphohistiocytosis (HLH) received an autologous peripheral blood stem cell transplant (PBSCT) following high-dose chemotherapy. He presented with hemophagocytic syndrome (HPS) at 6 months of age, but relapsed despite the repeated administration of prednisolone, VP-16, cyclosporin A (CsA), and other cytotoxic agents. PBSC were obtained using combination chemotherapy with etoposide (VP16, 450 mg/m2), doxorubicin (70 mg/m2), vincristine (2 mg/m2) and cyclophosphamide (CY, 1200 mg/m2). 2.7 x 10(5)/kg CFU-GM PBSC were transplanted after similar high-dose VP16 preconditioning used for allogeneic BMT for HLH. The boy continues to remain in complete remission 30 months after PBSCT while receiving low-dose PSL/CsA therapy. High-dose chemotherapy followed by PBSCT may be an optional therapeutic approach for patients with HLH.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1997

Transfer of autoimmune thyroiditis and resolution of palmoplantar pustular psoriasis following allogeneic bone marrow transplantation.

We report an unusual case of a patient who was cured of one autoimmune disease (palmoplantar pustular psoriasis (PPP)) but developed another autoimmune disease (autoimmune thyroiditis) after allogeneic BMT. A 40-year-old man suffering from AML with PPP underwent allogeneic BMT from his HLA-identical sister for the treatment of AML. The patient experienced complete clearance of the cutaneous PPP despite the cessation of immunosuppressive therapy for over 2 years. However, he developed hyperthyroidism with anti-thyroglobulin antibodies 5 months after BMT, although he had showed normal thyroid functions without anti-thyroglobulin antibodies before BMT. The donor had no history of thyroid diseases and showed normal thyroid functions but was positive for anti-thyroglobulin antibodies. Thus, even when the donor is in a subclinical state, autoimmune thyroiditis may be transferred from donors to recipients by BMT.

Adoptive Transfer↗
PubMed1997

Hypogammaglobulinaemia in a patient with ring chromosome 21.

An 8 year old boy with ring chromosome 21 who was susceptible to sinorespiratory infections due to hypogammaglobulinaemia is reported. He presented with the characteristic features of monosomy 21 syndrome, such as psychomotor retardation, hypertonia, large saccular ears, prominent nasal bridge, micrognathia, thrombocytopenia, and patent ductus arteriosus. His serum IgG concentration was less than 1.5 g/l at 3 years and 6 months of age after repeated hospitalisations with pneumonia, otitis media, and convulsions. Regular replacement of intravenous gammaglobulin effectively reduced such infectious episodes. A predisposition to infection in patients with ring chromosome 21 may be explained by hypogammaglobulinaemia and merit treatment with gammaglobulin.

Agammaglobulinemia↗
PubMed1996

Molecular analysis of glucose phosphate isomerase deficiency associated with hereditary hemolytic anemia.

We report here two new cases of glucose phosphate isomerase (GPI) deficiency associated with hemolytic anemia and present the results of molecular analysis of the five Japanese GPI variants. A Japanese girl (GPI Fukuoka) had an episode of prolonged neonatal jaundice and at 3 years of age was admitted due to acute hemolytic crisis occurring with upper respiratory tract infection. Red blood cell (RBC) GPI activity was decreased to 11.8% of normal and the reduced glutathione (GSH) level of RBCs was slightly decreased. A 54-year-old Japanese man (GPI Iwate) was hospitalized due to chronic active hepatitis, and compensated hemolysis was noted. RBC GPI activity of the proband was decreased to 18.8%, and the GSH content was about half of the normal mean value. Sequencing of the reticulocyte GPIcDNA showed homozygous missense mutations 1028CAG-->CGG (343Gln-->Arg), 14ACC-->A7C (5Thr-->lle), 671ACG-->A7G (224Thr-->Met), and 1615GAC-->AAC (539Asp-->Asn) in GPI Narita, GPI Matsumoto, GPI Iwate, and GPI Fukuoka, respectively. We also identified GPI Kinki as a compound heterozygote of 1124ACA-->AGA(375Thr-->Arg)/ 1615GAC-->AAC(539Asp-->Asn). Our findings, together with the previous results of other investigators, showed that the GPI gene mutations so far identified were heterogeneous, although most GPI variants had common biochemical characteristics such as heat instability and normal kinetics. Several amino acid substitutions were identified in the proximity of the catalytically important amino acid residues such as Ser/Asp 159/160, Asp341, and Lys518, which have been identified in the structural analysis of the pig GPI. The molecular characterization of human GPI variants, therefore, may provide new insights into the genotype-phenotype correlation of GPI deficiency as well as the structure-function relationship of this enzyme.

Anemia, Hemolytic↗
PubMed1996

Fas ligand in human serum.

The Fas ligand (FasL), a member of the tumor necrosis factor family, induces apoptosis in Fas-bearing cells. The membrane-bound human FasL was found to be converted to a soluble form (sFasL) by the action of a matrix metalloproteinase-like enzyme. Two neutralizing monoclonal anti-human FasL antibodies were identified, and an enzyme-linked immunosorbent assay (ELISA) for sFasL in human sera was established. Sera from healthy persons did not contain a detectable level of sFasL, whereas those from patients with large granular lymphocytic (LGL) leukemia and natural killer (NK) cell lymphoma did. These malignant cells constitutively expressed FasL, whereas peripheral NK cells from healthy persons expressed FasL only on activation. These results suggested that the systemic tissue damage seen in most patients with LGL leukemia and NK-type lymphoma is due to sFasL produced by these malignant cells. Neutralizing anti-FasL antibodies or matrix metalloproteinase inhibitors may be of use in modulating such tissue damage.

Animals↗
PubMed1996

Chronic myelomonocytic leukaemia with t(8;9)(p11;q34) in childhood: an example of the 8p11 myeloproliferative disorder?

We describe the case of a 10-year-old girl with chronic myelomonocytic leukaemia with the chromosomal translocation t(8;9)(p11;q34), who had developed tonsillar lymphoma as extramedullary involvement at the initial presentation. The cytogenetic study of the cells in both bone marrow and tonsils demonstrated t(8;9)(p11;q34), despite no malignant features in the bone marrow specimens. She developed acute leukaemic transformation 8 months after diagnosis during chemotherapy for lymphoma. Although etoposide reduced the number of blasts, t(8;9)(p11;q34)-bearing cells were not eradicated. Complete remission was obtained following an unrelated bone marrow transplantation. The clinical characteristics of this patient are similar to those of the patients with t(8;9)(p11;q34 or q32) or t(8;13)(p11;q11 or q12) reported previously. The unusual progression of the disease might be associated with the presence of (8;9)(p11;q34), suggesting a part in the 8p11 myeloproliferative syndrome.

Child↗
PubMed1996

Granulocyte transfusion: stimulation of low dose granulocyte colony-stimulating factor in donors for leukapheresis.

In order to assess the clinical utility of granulocyte transfusions (GT), the stimulating effects of donor granulopoiesis for GT therapy were examined using either low dose recombinant human granulocyte colony-stimulating factor (rhG-CSF) or dexamethasone (DEX). The increment of leukocytes, polymorphonuclear cells (PMN) and monocytes in the subjects stimulated with rhG-CSF (0.7 microgram/kg SC) surpassed each increment in those with DEX alone (1 mg PO). The lymphocyte counts after DEX stimulation decreased in contrast to those after G-CSF stimulation. This dose of G-CSF did not enhance the priming effects on the superoxide release from PMN. The serum levels of lysozyme, but not of lactate dehydrogenase, in G-CSF stimulated donors were higher than those in DEX-treated donors. The serum macrophage/monocyte-colony stimulating factor (M-CSF) levels in DEX stimulation were lower than in either G-CSF stimulation or no stimulation. The net yield of the PMN in GT on G-CSF stimulation was practically larger than that on DEX stimulation. One of the two patients who received GT collected by DEX stimulation died of aspergillosis. Two of the five patients who received PMN mobilized by G-CSF died of fungal infections or necrotizing fasciitis, although two of the remaining patients overcame severe bacterial infections. These results suggest that low dose G-CSF effectively and safely mobilizes a sufficient quantity of PMN from GT-donors without excessive superoxide generation from the transfused cells. This low dose G-CSF stimulation may be substituted for conventional DEX stimulation for GT.

Adult↗
PubMed1995

An abnormal fibrinogen Fukuoka II (Gly-B beta 15-->Cys) characterized by defective fibrin lateral association and mixed disulfide formation.

A dysfibrinogenemia was attributable to a single amino acid substitution from glycine to cysteine at residue 15 of the B beta chain in a fibrinogen molecule designated as fibrinogen Fukuoka II. The fibrinogen Fukuoka II showed prolonged thrombin and reptilase times and impaired fibrinopeptide B release by thrombin, resulting in abolition of fibrin monomer repolymerization under physiological conditions. Repolymerization of the des-(B beta 1-42)-fibrin monomers, however, was not distinguished from the normal pattern of des-(B beta 1-42)-fibrin monomers, suggesting that no other abnormality existed in fibrinogen Fukuoka II. Although an additional cysteine was substituted at residue 15 of the B beta chain, fibrinogen Fukuoka II had no free sulfhydryl group within the molecule. Instead, fibrinogen Fukuoka II formed a disulfide bond with cysteine, albumin, another mutated B beta chain within the same molecule, or intermolecular dimeric fibrinogen Fukuoka II. The mutation in fibrinogen Fukuoka II was the same as that in fibrinogen Ise published previously (Yoshida, N., Wada, H., Morita, K., Hirata, H., Matsuda, M., Yamazumi, K., Asakura, S., and Shirakawa, S. (1991) Blood 77, 1958-1963). Fibrinogen Ise, however, has been described as having prolonged thrombin time but normal reptilase time. Reasons for the discrepancy were not clear. Analysis of the B beta 1-42 fragment showed that fibrinogen was heterogeneous at position 31 of the B beta chain with respect to proline or hydroxyproline.

Amino Acid Sequence↗
PubMed1995

Histiocytic haemophagocytosis in a patient with Kawasaki disease: changes in the hypercytokinaemic state.

UNLABELLED: A 32-month-old Japanese boy exhibited haemophagocytic syndrome (HPS) during the recurrent course of Kawasaki disease. Despite repeated gamma-globulin therapy, he developed cytopenia with marked hepatomegaly and evidence of histiocytic haemophagocytosis in the bone marrow. Serum levels of interferon-gamma and tumour necrosis factor, but not of interleukin-1 beta, increased in parallel with his symptoms. No confirmation was obtained of the association of toxic reactions to the used drugs. No coronary lesions remained as sequelae. CONCLUSION: Cytopenia in Kawasaki disease could herald HPS, and the hypercytokinaemia involved in the two febrile syndromes might be of distinct nature.

Child, Preschool↗
PubMed1995

Haptoglobin therapy for acute favism: a Japanese boy with glucose-6-phosphate dehydrogenase Guadalajara.

We report the case of a 2-year-old Japanese boy with acute favism who was treated with human haptoglobin products. He had been exhibiting chronic nonspherocytic haemolytic anaemia until the diagnosis of glucose-6-phosphate dehydrogenase (G6PD) deficiency when 14 months old. He suffered a favic crisis at 24 months of age, when the administration of haptoglobin was effective for relieving bilirubinaemia and haemoglobinuria. Serum-free Hb rapidly decreased to normal levels despite the sustained level of serum lactate dehydrogenase. His G6PD gene was G6PD Guadalajara. This is the first application of haptoglobin therapy for acute favism and the first reported case of Japanese G6PD deficiency with typical favic crisis. Haptoglobin treatment might be helpful for managing the haemolytic crisis in the disease.

Child, Preschool↗
PubMed1995

Recurrent pneumococcal meningitis in a patient with transient IgG subclass deficiency.

We report the case of a 3 year old boy who exhibited recurrent serious infections with a transient imbalance of IgG subclass in the second year of life. He suffered from pneumococcal meningitis at 3 months, hepatitis at 9 months, and purulent arthritis at 11 months of age. The second episode of pneumococcal meningitis occurred at 14 months. Serum IgG level was normal for age. Low level of IgG2, undetectable level of IgG4 and negligible level of pneumococcus-specific IgG1-G2 antibodies were found. No other primary immunodeficiency was apparent. Serum IgG2-G4 levels but not pneumococcus-specific IgG1-G2 titers increased by the age of 30 months. At that time, he was inoculated with a polyvalent pneumococcal vaccine along with acellular diphtheria-pertussis-tetanus vaccine. He acquired the immunity against these agents, and had no episodic infections in the following 2 years. This observation stresses the existence of transient IgG subclass deficiency associated with delayed development of the anti-polysaccharide antibody response.

Child, Preschool↗
PubMed1995

Granulocyte-colony stimulating factor levels in cord blood and neonatal peripheral blood.

We measured the granulocyte-colony stimulating factor (G-CSF) levels in cord blood and peripheral blood obtained from full-term or pre-term infants during the first 3 days of birth. The mean G-CSF level among cord blood (17.2 pg/mL) was similar to that of peripheral blood on day 0 (18.3 pg/mL) and day 1 (13.6 pg/mL), while that of peripheral blood on day 0 was significantly higher than on day 2 (10.9 pg/mL) and day 3 (8.8 pg/mL; both P < 0.05). There was no correlation between neutrophil counts and G-CSF levels. No difference was found in neutrophil counts or G-CSF levels between infants who weighed more or less than 2500 g at birth. These results suggest that the neonatal neutrophil count depends on regulatory factors other than G-CSF.

Fetal Blood↗
PubMed1995

Herpes zoster in children with bone marrow transplantation: Report from a single institution.

Herpes zoster (HZ) has been often observed after bone marrow transplantation (BMT) in childhood. The occurrence of HZ was reviewed in children who received BMT. The clinical features of HZ were reviewed in 44 children who underwent BMT at Kyushu Cancer Center. Among the 35 recipients with a history of varicella before BMT, several factors associated with BMT and the lymphocyte subsets were compared between the patients who developed HZ (HZ+ group) and those who did not (HZ- group). Twenty-two recipients (50%) developed HZ; in two-thirds of these cases (15/22: 68%), HZ occurred between 80 and 120 days after BMT (median 101 days). The recipients treated with busulfan had a higher occurrence of HZ than those treated without it. The patients with Grade II-IV acute graft-versus-host disease (GVHD) developed HZ more frequently. In the HZ+ group, the absolute number of lymphocytes, CD3+, CD4+ or CD8+ cells at 3 months was significantly lower than that observed at 12 months after BMT and the CD4/CD8 ratio was significantly lower at 1 month than after 3 months of BMT. In conclusion, recipients were susceptible to HZ at around 100 days after BMT. The development of HZ may be associated with unbalanced T lymphocytes at that time.

Adolescent↗
PubMed1995

Interferon-gamma therapy for infection control in chronic granulomatous disease.

Interferon (IFN)-gamma was subcutaneously administered to four patients with chronic granulomatous disease (CGD) in order to evaluate its effects in controlling infection. Patients 1 to 3 were all males, while patient 4 was female. In patients 2 and 4, the length of infection-associated hospitalization during the year of IFN therapy was significantly shorter than that during the whole observed period prior to IFN therapy. In patients 1 and 2, the length of hospitalization during a year of IFN therapy was shorter than that during 1 year prior to the therapy. Patient 3 exhibited no reduction in terms of the length of infectious disease during IFN therapy, because he suffered from a liver abscess before and during the therapy. As soon as the IFN therapy was stopped, patient 2 developed pneumonia and lymphadenitis, which were promptly relieved by readministering the agent. During 1 year of IFN therapy, patients 1, 2 and 4 showed no significant changes in either the nitroblue tetrazolium test, O2- production or the expression of NADPH oxidase components in neutrophils. On the other hand, the O2- generating ability of neutrophils from patient 3 slightly increased. Our limited observations suggest that IFN-gamma may be variably beneficial for infection control in CGD-patients, irrespective of the in vitro phagocyte functions. A longer follow-up time is needed to confirm the IFN response in CGD-patients.

Adolescent↗
PubMed1995

Treatment of young relapsed Hodgkin's disease patients with high-dose chemotherapy followed by peripheral blood stem cell transplantation.

Four patients less than 20 years of age with Hodgkin's disease, who had developed either second or third relapse, were treated with high-dose chemotherapy followed by peripheral blood stem cell transplantation (PBSCT). Peripheral blood stem cells (PBSC) were collected after the administration of high-dose cytosine arabinoside (HD-AraC, 1g/m2 x 4). The conditioning regimen for PBSCT contained cyclophosphamide (50mg/m2 x 4), etoposide (150mg/m2 x 6) and ranimustine (250mg/dose x 1) (CVM regimen). A significant tumor regression was thus obtained after HD-AraC in 3 cases. The amount of PBSC harvested ranged from 1.2 to 4.5 x 10(5)/kg CFU-GM. Several cycles of conventional therapy were performed prior to the transplantation. PBSCT was done only after achieving a complete remission (CR) in 3 cases, but during relapse in another case. All patients showed both a rapid and sustained hematological recovery without any critical side effects. After this treatment, the 3 patients who were in CR at transplantation maintained a CR for 27, 26, and 4 months, respectively, while another developed a bone relapse despite achieving a CR by PBSCT. These results suggest that a combination of HD-AraC and CVM followed by PBSCT is therefore considered to be beneficial in the treatment of relapsed Hodgkin's disease.

Adolescent↗
PubMed1995

Internalization of an anti-glycoprotein IIb/IIIa antibody by HEL cells.

Platelets are known to internalize monoclonal antibodies directed against the glycoprotein (GP) IIb/IIIa complex. We investigated whether an antibody directed against this complex (NNKY 2-11) was transported from the surface membrane to the intracellular pool in HEL cells. Flow cytometry showed that the percent binding of NNKY 2-11 to the surface membrane of HEL cells was decreased after incubation for 24 h compared with 1 h, while the binding of an anti-GPIb antibody (NNKY 5-5) did not change. It did not seem likely that the GP IIb/IIIa complex antibody was shed from the surface membrane of the HEL cells during incubation, because the medium conditioned by incubation with these cells for 24 h showed almost no binding to washed platelets. In addition, immunoelectron microscopy demonstrated that GP IIb/IIIa complex antibodies were incorporated into the intracellular pool of HEL cells and were associated with alpha granules. These findings indicated that an anti-GP IIb/IIIa antibody could be internalized by megakaryocytes, as has been previously shown with platelets, suggesting that megakaryocyte GP IIb/IIIa may act as a carrier for various adhesion proteins.

Antibodies, Monoclonal↗