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Biomedical subjects

S Hibi

Publications and source records attributed to S Hibi.

At least 73 records · Page 4Linked to original sources
PubMed1997

Hypercalcemia in children presenting with acute lymphoblastic leukemia.

Although hypercalcemia is a well-recognized complication in malignant disorders, neither the incidence and prognostic significance of hypercalcemia, nor the role of parathormone related peptide (PTHrP) in pediatric acute lymphoblastic leukemia (ALL) have been clarified. Of 83 newly diagnosed pediatric ALL patients with early pre-B cell phenotype treated at our hospital during the last 8 years, four patients were diagnosed as having hypercalcemia (> 14 mg/dl). In these 4 hypercalcemic ALL patients at onset, serum calcium levels ranged from 14.6 to 20.8 mg/dl (normal 7.4-9.0 mg/dl), and serum PTHrP levels were markedly elevated to 112-240 pmol/l (normal range: 17.6-61.2 pmol/l). Unlike patients with ordinary ALL in childhood, gastrointestinal symptoms (nausea, vomiting, abdominal pain) and skeletal symptoms (bone pain, gait disturbance) were the chief complaints. Because of these characteristic symptoms, bone marrow aspiration was carried out in two patients in an attempt to diagnose ALL before leukemic cells appeared in peripheral blood. Serum calcium levels were promptly normalized by induction chemotherapy. The four patients have been in complete remission from 35+ to 125+ months. Based on these results, the incidence of hypercalcemia in pediatric ALL patients with early pre-B cell phenotype at our institute is calculated to be about 4.8%. Gastrointestinal and skeletal problems are the characteristic initial symptoms, and hypercalcemia does not seem to be significant in the prognosis of these patients.

Child↗
PubMed1997

Unsuccessful CTL transfusion in a case of post-BMT Epstein-Barr virus-associated lymphoproliferative disorder (EBV-LPD).

A patient with AML (FAB M4Eo) developed EBV-LPD 1.5 months after allogeneic BMT from his one locus-mismatched mother, the diagnosis being confirmed on day +82. Attempts to eradicate the monoclonally proliferating LPD using chemotherapy (VP16/dexamethasone) followed by two doses of EBV-specific CTL and one dose of unstimulated donor leukocytes were not successful. We assume delay of infusions (day +100, +107) and insufficient CTL cell doses (total 9.2 x 10(6)) may have been responsible for the poor outcome in this case.

Bone Marrow Transplantation↗
PubMed1997

Chimerism analysis on mononuclear cells in the CSF after allogeneic bone marrow transplantation.

To evaluate the chimeric status of mononuclear cells in the CSF after allogeneic BMT, cells were analyzed by FISH using satellite DNA probes for human X and Y chromosomes. CSF cells were obtained from five pediatric ALL patients who received BMT from sex-mismatched donors. All patients received TBI-containing conditioning regimens. We found that CSF cells showed complete donor type in 19-97 days after BMT, when complete donor type hematopoiesis was observed. The rapid entry of the donor leukocytes into the brain may exert beneficial effects to eradicate the residual CNS leukemic cells and prevent a CNS relapse in ALL patients after BMT.

Bone Marrow Transplantation↗
PubMed1997

Haemophagocytic lymphohistiocytosis in association with granular lymphocyte proliferative disorders in early childhood: characteristic bone marrow morphology.

Five paediatric cases of haemophagocytic lymphohistiocytosis (HLH) which showed proliferation of granular atypical lymphocytoid cells in bone marrow are reported. All cases were girls aged 8 months to 4 years who had marked hepatosplenomegaly. Marker analysis on peripheral blood mononuclear cells revealed an increase in the CD3+HLADR+ subset in three cases and the CD3- CD56+ subset in one case. An Epstein-Barr virus genome was detected in three cases, and monoclonality was confirmed in two cases. A characteristic morphology of large granular lymphocytes (LGL) was identified, with elongated bizarre features that resembled horsetail-, tadpole-, cucumber- or shooting star-type configurations on the bone marrow smear. Serum concentrations of soluble interleukin-2 receptor and interferon-gamma were elevated in all cases. All five cases required multi-agent chemotherapy which resulted in two complete remission, two partial remissions and one no response. Refinement of treatment is required for these paediatric GLPD cases which probably comprise a specific high-risk subgroup among secondary HLH patients which had previously escaped notice.

Bone Marrow Diseases↗
PubMed1997

Effects of thrombopoietin (c-mpl ligand) on growth of blast cells from patients with transient abnormal myelopoiesis and acute myeloblastic leukemia.

Thrombopoietin (TPO) is a ligand for c-mpl that promotes both proliferation and differentiation of megakaryocytes in vivo and in vitro. We investigated the expression of c-mpl transcripts and the effects of recombinant human TPO (rhTPO) on the proliferation and differentiation of human leukemic cell lines or fresh samples obtained from 32 patients with transient abnormal myelopoiesis (TAM) or acute myeloblastic leukemia (AML). Cells were cultured with TPO alone or combined with rh interleukin-3 (IL-3) or stem cell factor (SCF). Expression of c-mpl was verified in 6 of 13 cases tested. All but one of the cases that showed c-mpl expression responded to TPO. Blasts from all cases of TAM or French-American-British (FAB) subtype M7 showed growth responses to TPO with higher sensitivity than cells of other FAB subtypes and these responses were increased by addition of rhIL-3 or rhSCF in some cases. Responses of cells of other FAB subtypes varied. In addition, increased expression of platelet-specific surface antigens on MO7E cells after incubation with rhTPO was observed. These data suggest that TPO may be involved in the abnormal proliferation and differentiation of human leukemic cells, especially of M7 and TAM cells, considered to be of megakaryocytic lineage.

Adult↗
PubMed1997

High levels of circulating interleukin-4 and interleukin-10 in Kawasaki disease.

For analysis of the cytokine network in Kawasaki disease (KD), we measured over time the plasma levels of interferon (IFN)-gamma, interleukin (IL)-4 and IL-10 in patients with KD. Fifteen patients with KD were studied. Eight healthy children were selected as control subjects. Circulating IFN-gamma levels were measured by immunoradiometric assay, and IL-4 and IL-10 levels were measured by enzyme-linked immunosorbent assay. The results were as follows: (1) The plasma levels of IFN-gamma in KD patients in the acute phase were significantly higher than the levels of patients in the convalescent phase (p < 0.05) and those of the control children (p < 0.05). (2) The plasma levels of IL-4 in the KD patients in the acute phase were significantly higher than the levels of the patients in the convalescent phase (p = 0.001) and those of the control children (p = 0.001). (3) The plasma levels of IL-10 in the KD patients in the acute phase were significantly higher than the levels of the patients in the convalescent phase (p < 0.03) and those of the control children (p < 0.005). (4) The investigation of the relationship between the IL-4 and IFN-gamma levels during the acute phase of KD demonstrated a significant reciprocal relationship (p < 0.05). (5) There was no significant relationship between the IL-4 and IL-10 levels during the acute phase. However, plasma IL-10 levels were low in the patients with high levels of plasma IL-4, and the patients with high levels of IL-10 revealed low levels of plasma IL-4. The above results suggested that a variety of patterns of cytokine production was present in the acute phase of this disease, and that the key cytokine, which might regulate the cytokine network, was IL-4.

Case-Control Studies↗
PubMed1997

[Transient inhibitors developed in two cases of hemophilia A].

This report describes the transient development of inhibitors in two cases of hemophilia A. Case 1 was an 8-month-old boy who suffered from subcutaneous hematoma with extremely low level (1.5%) of plasma factor VIII activity (F VIII:C). He showed an inhibitor titer of 2.5 Bethesda units (BU)/ml when he suffered from measles at age 24 months with poor clinical response to factor VIII. This inhibitor elevated to 11.6 BU/ml at 29 months, but completely disappeared at 32 months. Case 2 was a 5-month-old boy who suffered from subcutaneous hematoma with extremely low level (< 1%) of plasma F VIII:C. At 8 months his inhibitor titer peaked at 19 BU/ml but subsequently declined to 11.5 BU/ml at 12 months and completely disappeared at 14 months. A review of the literature revealed that the median (range) titer of transient inhibitor initially developed at 2 (0.5-19) BU/ml on 225 (79-448) days, immediately peaked at 3.6 (0.5-60) BU/ml and disappeared by 665 (282-1140) days, suggesting that careful monitoring of F VIII inhibitors is required in the treatment of hemophilia A.

Autoantibodies↗
PubMed1996

Structure-activity relationships of (E)-3-(1,4-benzoquinonyl)-2-[(3-pyridyl)-alkyl]-2-propenoic acid derivatives that inhibit both 5-lipoxygenase and thromboxane A2 synthetase.

As part of our research for the development of novel antiinflammatory drug candidates, we have designed and synthesized a series of (E)-3-(1,4-benzoquinonyl)-2-[(3-pyridyl)alkyl]-2-propenoic acid derivatives as dual inhibitors of 5-lipoxygenase (5-LO) and thromboxane (TX) A2 synthetase. In order to increase the absorption after oral administration, we introduced a carboxylic acid moiety into the 1,4-benzoquinone skeleton, which has 5-LO-inhibitory character. Introduction of a 3-pyridylalkyl group at the double bond of the 1,4-benzoquinonyl propenoic acid moiety afforded good to moderate inhibitory activities against the production of leukotriene (LT) B4 and TXA2 while not significantly inhibiting that of prostaglandin E2 by glycogen-induced peritoneal cells of rat (in vitro). The length of the methylene chain of the 3-pyridylalkyl group influenced the inhibition of LTB4 and TXB2 production. An increase of lipophilicity by introducing a more lipophilic alkoxy group did not markedly increase the inhibitory activity on LTB4 production. The position of alkoxy group on the 1,4-benzoquinone skeleton played an important role in TXA2 synthetase inhibition. Compounds such as 20c (E6700) with an appropriate alkoxy group and proper length of methylene side chain, together with a polar substituent (carboxylic acid), showed good inhibition of both 5-LO and TXA2 synthetase and possess a variety of pharmacologically beneficial effects.

Animals↗
PubMed1996

Secondary lymphoid malignancy in two children with neuroblastoma.

Secondary lymphoid malignancy, particularly acute lymphoblastic leukemia (ALL), is rare. We report one case of ALL and another case of mediastinal lymphoblastic lymphoma developed after treatment for neuroblastoma. The secondary ALL characterized by short latency period and an 11q23 translocation apparently was induced by etoposide treatment. The pathogenesis of the secondary lymphoma is less certain and may be related to previous treatment with cyclophosphamide and radiotherapy, host susceptibility, or chance occurrence. One child died of progressive lymphoma and the other remains in remission 1 year following allogeneic bone marrow transplantation. Additional studies are needed to determine the risk, pathogenesis, and optimal treatment for secondary lymphoid malignancy.

Child↗
PubMed1996

Hyper-interleukin (IL)-6-naemia in haemophagocytic lymphohistiocytosis.

Clinical features in patients with haemophagocytic lymphohistiocytosis (HLH) have been demonstrated to be characterized by hypercytokinaemia. Previously, we reported the impact of high serum levels of interferon (IFN)-gamma and soluble IL-2 receptor (sIL-2R) on patient outcome; however, it was not known if serum levels of interleukin (IL)-6 also could be a prognostic factor. In a study during the active phase of disease in 25 cases of HLH in children and young adults (median age 3 years, range 0.1-23 years), we noted 12 cases which showed serum IL-6 > 100 (normal < 4.0) pg/ml. Five of these cases showed hyper-IL-6-naemia alone without hyper-IFN-gamma-naemia (group A) whereas seven cases showed both hyper-IL-6- and IFN-gamma-naemia (group B). Patient outcome did not differ between the patients with IL-6 > 100 pg/ml and those with IL-6 < 100 pg/ml, suggesting that high serum concentrations of IL-6 alone do not necessarily indicate poor prognosis in patients with HLH. Among the cases with hyper-IL-6-naemia ( > 100 pg/ml), underlying disorders causing haemophagocytosis were found to be different between groups A and B.

Adolescent↗
PubMed1996

[Plasma interleukin-10 levels in Kawasaki disease].

Time course of interleukin-10 (IL-10) in plasma of patients with Kawasaki disease was measured. The results are as follow: (1) IL-10 concentration in plasma of patients in acute phase of Kawasaki disease (median 37.8 pg/ml) was high, compared with that in convalescent phase (5.77 pg/ml) or that of control children (10.2 pg/ml). (2) From the examination of IL-10 concentration in plasma collected by following its time course, it was revealed that most of cases with this disease showed transiently high figure in the acute phase, then the figure diminished promptly afterwards.

Acute-Phase Reaction↗
PubMed1996

[Chemotherapy for B lymphoid malignancy in childhood--results in BLK88 protocol].

Ten cases of newly diagnosed pediatric B cell non-Hodgkin's lymphoma or acute lymphoblastic leukemia (B-NHL, stage I & II 6 cases, stage III & IV 2 cases/ALL 2 cases) experienced during the last 7 years (1987-1994) were treated by BLK88 protocol, which consisted of HD-CPM (1,200 mg/m2), and HD-MTX (1,000 mg/m2) with VCR, ADR, and/or AraC combination, and CNS prophylaxis by triple intrathecal injection. The therapy duration was 24 weeks for B-NHL (36 weeks for B-ALL). The results showed that while one of the six cases in stage I & II relapsed, and other 4 cases of stage III & IV B-NHL/ALL remained in complete remission. On the other hand, all of the four cases in stage III & IV in historical controls had relapsed. Neutropenia and liver dysfunction were observed during therapy, but they were tolerable. We conclude that BLK88 is a very useful protocol for B-NHL/ALL in childhood.

Adolescent↗
PubMed1996

[Circulating interleukin-4 levels in acute phase Kawasaki disease].

We have measured the plasma levels of IL-4 in patients with Kawasaki disease, serially. The results were as follows: The plasma levels of IL-4 from patients with Kawasaki disease in acute phase (median 17 pg/ml) were significantly higher than those from the patients in convalescent phase (median 1.96 pg/ ml, p = 0.001) or from control children (median 7.64 pg/ml, p = 0.001). These findings imply that Th2 (or Tho) cells may be vigorously stimulated at an acute phase of this disease, and that IL-4 produced by these cells may possibly modulate the pathological conditions of this disease.

Acute Disease↗