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  • American Society of Hematology  (5)
  • 1
    Online Resource
    Online Resource
    Clinical Outcome of Cord Blood Transplantation by Age with Units Shipped from Tokyo Cord Blood Bank.
    American Society of Hematology ; 2004
    In:  Blood Vol. 104, No. 11 ( 2004-11-16), p. 5178-5178
    Details
    In: Blood, American Society of Hematology, Vol. 104, No. 11 ( 2004-11-16), p. 5178-5178
    Abstract: Recently, cord blood transplantation (CBT) for adult is rapidly increasing in number, Especially for the patients over 50 years of age. By the end of 2003, 224 units were shipped for the patients Group I: 〈 15 y.o. 39%, Group II: 15~50 y.o.40% and Group III : 〉 50 y.o.21%. We analyzed 152 patients with hematological malignancies including ALL, AML, CML, MDS, malignant lymphoma, myeloma and neuroblastoma reported from CBT center in the world by the end of 2003. Patients and Methods:Group I included 58 patients with 13 standard risk and 45 high risk patients and showed mean±SD of age; 5.3 ±4.1y.o.,BW; 20.5±13.4kg, CB volume at collection; 91.6±27.1ml, NC; 5.5±3.3x107/kg, CFC; 8.6±6.4x104/kg, CD34; 1.5±1.1x105/kg, Group II: 64 cases with 25 standard and 39 high risk patients and age; 30.6±10.3y.o., BW; 51.9±9.5kg, CB volume at collection;116.6±27.7ml, NC;2.6±0.7x107/kg, CFC;5.2±2.6x104/kg and CD34;0.8±0.5x105/kg, Group III: 30 cases with 9 standard and 21 high risk patients and age; 54.1±3.3y.o., BW;55.9±11.0kg, CB vol.at collection;118.8±24.7ml NC;2.4±0.4x107/kg, CFC;4.8±1.9x104/kg and CD34;0.8±0.4x105/kg. The patients who underwent CBT for graft failure (GF) of prior transplant were excluded. Conditioning regimen in Group I demonstrated 54 patients with full regimen and 4 with reduced intensity regimen (RIST); in Group II, 62 patients with full regimen and 2 RIST; and in Group III, 14 cases full regimen and 15 cases RIST. Results: Cumulative myeloid engraftment was seen 67.2% in Group I, 73.4% in Group II and 46.7% in Group III (*Group II vs. Group III: P 〈 0.05). Overall survival /EFS on day 100 showed 73.4%/59.4% in Group I, 74.0%/58.6% in Group II and 43.3%/34.5% in Group III (*Group III vs. others: P 〈 0.05). In Group III, the survival rate indicated 42.8% in full regimen group and 13.3% in RIST group at 1year after CBT. In Group I, four patients died of GF, 13 of relapse, 11 of Transplantation related disease (TRD); in Group II, 5 patients died of GF, 8 of TRD, 10 of relapse. In Group III, four patients died of TRD, 3 of GF and 3 of relapse in full regimen, while in RIST, six patients died of TRD, 1 of relapse and 1 of acute GVHD. Conclusion: The application of CBT has been expanded to the elderly patients ( 〉 50 y.o.), although the conditioning regimen and the special medical care for the complications in the early pahse after UCBT has remained to be discussed.
    Type of Medium: Online Resource
    ISSN: 0006-4971 , 1528-0020
    URL: Article
    DOI: 10.1182/blood.V104.11.5178.5178
    RVK:
    XA 33000
    RVK:
    XA 10000
    Language: English
    Publisher: American Society of Hematology
    Publication Date: 2004
    detail.hit.zdb_id: 1468538-3
    detail.hit.zdb_id: 80069-7
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  • 2
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    Online Resource
    Whole-Exome Analysis of Autoimmune Lymphoproliferative Syndrome-like Diseases
    American Society of Hematology ; 2015
    In:  Blood Vol. 126, No. 23 ( 2015-12-03), p. 1022-1022
    Details
    In: Blood, American Society of Hematology, Vol. 126, No. 23 ( 2015-12-03), p. 1022-1022
    Abstract: ALPS is characterized by chronic lymphoproliferation in combination with autoimmunity; mutations of molecules involved in FAS-dependent pathways play causative roles in this syndrome. The hallmarks of ALPS are an elevated CD4/CD8 double-negative T (DNT) cell count and attenuated induction of apoptosis by FAS stimulation. Autoimmune thrombocytopenia and/or hemolytic anemia are common in cases of ALPS; the combination of autoimmune thrombocytopenia and hemolytic anemia is referred to as Evans syndrome. ALPS is diagnosed in 47% of patients who present with Evans syndrome. Some patients with ALPS-like syndromes harbor mutations in RAS or PRKCD. In this study, we performed whole-exome analysis of undiagnosed patients exhibiting autoimmunity in combination with lymphoproliferation. Fifteen pediatric patients presenting with autoimmunity were enrolled in this study. Although not all of them fulfilled the diagnostic criteria of ALPS, most of them exhibited an ALPS-like phenotype, an autoimmune hematological disorder such as Evans syndrome, or immune thrombocytopenia with hepatosplenomegaly. Elevated counts of DNT cells among TCRαβ-positive cells were observed in some cases. All of the subjects screened negative for mutations in FAS, FASL, KRAS, and NRAS. These patients were subjected to whole-exome analysis, which revealed several mutations with known disease associations. One patient carried a mutation in CASP10, a causative gene for ALPS-CSAP (ALPS 2). This patient exhibited a typical ALPS phenotype with an elevated DNT cell count. In addition, we identified CTLA4 mutations in two patients, one of whom was described as ALPS type V (ALPS 5) and the other as CTLA4 haploinsufficiency with autoimmune infiltration (CHAI). A mutation in STAT3 was present in one patient. Dominant-negative mutations in the STAT3 gene result in hyper-IgE syndrome. Recently, an activating mutation of STAT3 was reported in infantile-onset multisystem autoimmune disease (ADMIO). It should be noted that an activating mutation of STAT3 results in a phenotype very similar to that of ALPS. Intriguingly, two autoinflammatory associated genes, PSTPIP1 and RNASEH2B were identified. Mutations in RNASEH2B causes Aicardi-Goutière syndrome (AGS), which phenotypically overlaps with SLE. Typical AGS develops as a results of biallelic mutations in causative genes. In this case, a heterozygous frame shift mutation was identified. Further evaluation is required to confirm whether this heterozygous frame shift mutation RNASEH2B really causes SLE or ALPS like symptoms. PSTPIP1 mutation causes pyogenic Arthritis, pyoderma gangrenosum and acne (PAPA) syndrome. The identified E250K mutation was one of the typical mutation in this disease. This patient exhibited SLE like symptom with hepatosplenomegaly without typical PAPA syndrome like symptoms, such as acne or pyoderma of the skin. Sequencing of the remaining patients yielded inconclusive results. The classical ALPS diagnostic procedure is well designed and suitable for identification of FAS-dependent apoptotic dysregulation. However, the measurement of FAS-dependent apoptosis requires technical skill, and the data quality is therefore dependent on the investigator. Moreover, RAS-associated ALPS-like disease (RALD, ALPS 3) and ALPS 5 (CHAI) cannot be identified by conventional ALPS diagnostic procedures. Therefore, the methods for diagnostic classification of these diseases need to be updated. It is interesting that mutations of autoinflammatory associated genes are involved in ALPS or SLE like patients. Our results reveal that a comprehensive genomic approach is a powerful tool for the characterization of ALPS or ALPS-like diseases. Together with recent progress in genome analysis in the PID field, our analyses provide an updated list of genes for use in differential diagnosis of ALPS-like diseases. This update will facilitate convenient genomic approaches such as comprehensive targeted sequencing focusing on genes involved in ALPS or ALPS-like diseases. This approach can be directly applied in the clinic to yield diagnostic benefits. Disclosures No relevant conflicts of interest to declare.
    Type of Medium: Online Resource
    ISSN: 0006-4971 , 1528-0020
    URL: Article
    DOI: 10.1182/blood.V126.23.1022.1022
    RVK:
    XA 33000
    RVK:
    XA 10000
    Language: English
    Publisher: American Society of Hematology
    Publication Date: 2015
    detail.hit.zdb_id: 1468538-3
    detail.hit.zdb_id: 80069-7
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  • 3
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    Online Resource
    Somatic mosaicism for oncogenic NRAS mutations in juvenile myelomonocytic leukemia
    American Society of Hematology ; 2012
    In:  Blood Vol. 120, No. 7 ( 2012-08-16), p. 1485-1488
    Details
    In: Blood, American Society of Hematology, Vol. 120, No. 7 ( 2012-08-16), p. 1485-1488
    Abstract: Juvenile myelomonocytic leukemia (JMML) is a rare pediatric myeloid neoplasm characterized by excessive proliferation of myelomonocytic cells. Somatic mutations in genes involved in GM-CSF signal transduction, such as NRAS, KRAS, PTPN11, NF1, and CBL, have been identified in more than 70% of children with JMML. In the present study, we report 2 patients with somatic mosaicism for oncogenic NRAS mutations (G12D and G12S) associated with the development of JMML. The mutated allele frequencies quantified by pyrosequencing were various and ranged from 3%-50% in BM and other somatic cells (ie, buccal smear cells, hair bulbs, or nails). Both patients experienced spontaneous improvement of clinical symptoms and leukocytosis due to JMML without hematopoietic stem cell transplantation. These patients are the first reported to have somatic mosaicism for oncogenic NRAS mutations. The clinical course of these patients suggests that NRAS mosaicism may be associated with a mild disease phenotype in JMML.
    Type of Medium: Online Resource
    ISSN: 0006-4971 , 1528-0020
    URL: Article
    DOI: 10.1182/blood-2012-02-406090
    RVK:
    XA 33000
    RVK:
    XA 10000
    Language: English
    Publisher: American Society of Hematology
    Publication Date: 2012
    detail.hit.zdb_id: 1468538-3
    detail.hit.zdb_id: 80069-7
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  • 4
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    Online Resource
    Spi-B is critical for plasmacytoid dendritic cell function and development
    American Society of Hematology ; 2012
    In:  Blood Vol. 120, No. 24 ( 2012-12-06), p. 4733-4743
    Details
    In: Blood, American Society of Hematology, Vol. 120, No. 24 ( 2012-12-06), p. 4733-4743
    Abstract: Plasmacytoid dendritic cells (pDCs), originating from hematopoietic progenitor cells in the BM, are a unique dendritic cell subset that can produce large amounts of type I IFNs by signaling through the nucleic acid–sensing TLR7 and TLR9 (TLR7/9). The molecular mechanisms for pDC function and development remain largely unknown. In the present study, we focused on an Ets family transcription factor, Spi-B, that is highly expressed in pDCs. Spi-B could transactivate the type I IFN promoters in synergy with IFN regulatory factor 7 (IRF-7), which is an essential transcription factor for TLR7/9-induced type I IFN production in pDCs. Spi-B–deficient pDCs and mice showed defects in TLR7/9-induced type I IFN production. Furthermore, in Spi-B–deficient mice, BM pDCs were decreased and showed attenuated expression of a set of pDC-specific genes whereas peripheral pDCs were increased; this uneven distribution was likely because of defective retainment of mature nondividing pDCs in the BM. The expression pattern of cell-surface molecules in Spi-B–deficient mice indicated the involvement of Spi-B in pDC development. The developmental defects of pDCs in Spi-B–deficient mice were more prominent in the BM than in the peripheral lymphoid organs and were intrinsic to pDCs. We conclude that Spi-B plays critical roles in pDC function and development.
    Type of Medium: Online Resource
    ISSN: 0006-4971 , 1528-0020
    URL: Article
    DOI: 10.1182/blood-2012-06-436527
    RVK:
    XA 33000
    RVK:
    XA 10000
    Language: English
    Publisher: American Society of Hematology
    Publication Date: 2012
    detail.hit.zdb_id: 1468538-3
    detail.hit.zdb_id: 80069-7
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  • 5
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    Online Resource
    Anti-Interleukin 6 Receptor Monoclonal Antibody Therapy Reduced Serum Interleukin 4 and IgE Production in Castleman’s Disease.
    American Society of Hematology ; 2005
    In:  Blood Vol. 106, No. 11 ( 2005-11-16), p. 1510-1510
    Details
    In: Blood, American Society of Hematology, Vol. 106, No. 11 ( 2005-11-16), p. 1510-1510
    Abstract: Multicentric Castleman’s disease (MCD) is an atypical lympho- proliferative disorder which is closely associated with dysregulated overproduction of interleukin-6 (IL-6). In the previous report, we showed that the humanized anti-IL-6 receptor monoclonal antibody, tocilizumab (formerly called MRA), was therapeutically effective for the patients with MCD and the safety profile was acceptable relative to the clinical benefit. We also found that serum IgE levels were elevated in some patients with Castleman’s disease, which also decreased by IL-6 blocking therapy, suggesting that IL-6 may be involved in IgE production in vivo. To examine whether or not IL-6 serves as a regulator for IL-4, a major class switching factor for IgE, in vivo, we analyzed serum IL-4 as well as IgE levels in patients with MCD before and after the blockade of IL-6 actions utilizing humanized anti-IL-6 receptor monoclonal antibody in the 4-month clinical trial. Twenty-eight patients with MCD were enrolled and received intravenously 8 mg/kg of tocilizumab every two weeks for a total of 4 months. In fifteen of twenty-eight patients, 5–20 mg/day of prednisolone was used 4 weeks prior to study dosing, but the dosage of each patient was not changed during treatment. Serum IL-4 and IgE levels as well as inflammatory markers were monitored. This study complied with all provisions of the Declaration of Helsinki and was conducted in accordance with Good Clinical Practice guidelines. All patients gave written informed consent before participating in this study. Tocilizumab treatment improved the systemic inflammatory manifestations and laboratory markers such as CRP and fibrinogen as we previously reported. Before tocilizumab treatment, serum IL-4 and IgE levels were elevated (median 14.9 pg/mL, range 2.8–228 pg/mL and median 995 IU/mL, range 12–8000 IU/mL, respectively, n=28). Serum IL-4 levels gradually decreased by tocilizumab treatment (median 8.0 pg/mL, range 2.0–257 pg/mL at 6 weeks, n=28, p 〈 0.005 vs. pretreatment; median 6.0 pg/mL, range 2.0–75.6 pg/mL at 16 weeks, n=28, p 〈 0.0001, respectively). Serum IgE levels also significantly decreased at 6 weeks but not at 16 weeks (median 590 IU/mL, range 6–7000 IU/mL at 6 weeks, p 〈 0.005; median 605 IU/mL, range 5.9–16000 IU/mL at 16 weeks, n.s, respectively). In association with the decrease in serum IL-4 and IgE levels, disease activity of atopic dermatitis improved in the patients with atopic dermatitis. IgE RAST scores for Japanese cedar also significantly decreased. IL-6 has been reportedly to direct the differentiation of IL-4-producing T helper cell 2 (Th2), in vitro, but the in vivo action of IL-6 has been controversial. Our findings clearly indicate that IL-6 regulates the IL-4 production, in vivo, and may serve as a regulator not only for B cell differentiation but also for class switching to IgE.
    Type of Medium: Online Resource
    ISSN: 0006-4971 , 1528-0020
    URL: Article
    DOI: 10.1182/blood.V106.11.1510.1510
    RVK:
    XA 33000
    RVK:
    XA 10000
    Language: English
    Publisher: American Society of Hematology
    Publication Date: 2005
    detail.hit.zdb_id: 1468538-3
    detail.hit.zdb_id: 80069-7
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