How I treat patients with indolent and smoldering mastocytosis (rare conditions but difficult to manage).

abstract：:Polycythemia vera (PV) is a clonal myeloproliferative disorder characterized by excessive erythrocyte production. Most patients with PV harbor an activating JAK2 mutation, but the molecular links between this mutation and erythrocyte overproduction are unknown. The interaction between death receptors and their ligands...

journal_title：Blood

authors： Zeuner A,Pedini F,Signore M,Ruscio G,Messina C,Tafuri A,Girelli G,Peschle C,De Maria R

更新日期：2006-05-01 00:00:00

abstract：:Ionophore A23187-induced 14C-serotonin secretion and thromboxane B2(TxB2) formation were found to be absent in citrated platelet-rich plasma (PRP) from thrombasthenic subjects and in normal PRP treated with glycoprotein (GP) IIb-IIIa complex-specific monoclonal antibodies. Both responses were restored to normal levels...

journal_title：Blood

authors： Lages B,Weiss HJ

更新日期：1994-05-01 00:00:00

abstract：:Monoclonal gammopathy of undetermined significance (MGUS) is present in ∼2% of individuals age >50 years. The increased risk of multiple myeloma (MM) in relatives of individuals with MGUS is consistent with MGUS being a marker of inherited genetic susceptibility to MM. Common single-nucleotide polymorphisms (SNPs) at ...

journal_title：Blood

authors： Weinhold N,Johnson DC,Rawstron AC,Försti A,Doughty C,Vijayakrishnan J,Broderick P,Dahir NB,Begum DB,Hosking FJ,Yong K,Walker BA,Hoffmann P,Mühleisen TW,Langer C,Dörner E,Jöckel KH,Eisele L,Nöthen MM,Hose D,Davies

更新日期：2014-04-17 00:00:00

abstract：:This study reports a lentiviral gene transfer protocol for efficient transduction of adult human peripheral blood (PB)-derived CD34+ NOD/SCID-repopulating cells (SRCs) using vesicular stomatitis virus-G protein (VSV-G)-pseudotyped lentiviruses encoding for enhanced green fluorescence protein (eGFP). Lentiviral stocks ...

journal_title：Blood

authors： Scherr M,Battmer K,Blömer U,Schiedlmeier B,Ganser A,Grez M,Eder M

更新日期：2002-01-15 00:00:00

abstract：:Biochemical and morphological evidence is presented to support the characterization of a rat bone-marrow-derived cell line (RPM) as an analog of the promegakaryoblast. The conditions for in vitro growth and maturation of the RPM line are described. Rapid proliferation of the RPM line is readily achieved when cultures ...

journal_title：Blood

authors： Weinstein R,Stemerman MB,MacIntyre DE,Steinberg HN,Maciag T

更新日期：1981-07-01 00:00:00

abstract：:Langerhans cell histiocytosis (LCH) is a rare histiocytic neoplasm associated with somatic mutations in the genes involved in the RAF/MEK/extracellular signal-regulated kinase (ERK) signaling pathway. Recently, oncogenic mutations in NRAS/KRAS, upstream regulators of the RAF/MEK/ERK pathway, have been reported in pulm...

journal_title：Blood

authors： Kamata T,Giblett S,Pritchard C

更新日期：2017-07-27 00:00:00

abstract：:Peripheral blood cells from nine patients with B-chronic lymphocytic leukemia (B-CLL) were treated in vitro with bryostatin 1 (a macrocyclic lactone derived from a marine invertebrate). Like the phorbol ester 12-0-tetradecanoyl-phorbol 13-acetate (TPA), bryostatin 1 activates protein kinase C (PKC), which plays a cent...

journal_title：Blood

authors： Drexler HG,Gignac SM,Jones RA,Scott CS,Pettit GR,Hoffbrand AV

更新日期：1989-10-01 00:00:00

abstract：:Human granulocyte colony-stimulating factor (G-CSF) is a regulatory glycoprotein that stimulates the production of neutrophilic granulocytes from committed hematopoietic progenitor cells both in vitro and in vivo. In this report, we show that biosynthetic (recombinant) human G-CSF enhances colony formation by normal h...

journal_title：Blood

authors： Avalos BR,Gasson JC,Hedvat C,Quan SG,Baldwin GC,Weisbart RH,Williams RE,Golde DW,DiPersio JF

更新日期：1990-02-15 00:00:00

abstract：:Stimulators of human erythroid burst-forming units (BFU-E) and multipotential colony-forming cells (CFU-GEMM) can be produced by a number of different cell types. A product of human peripheral blood monocytes, interleukin 1 (IL-1), was evaluated for its ability to stimulate fibroblast cultures to produce stimulators o...

journal_title：Blood

authors： Zucali JR,Broxmeyer HE,Dinarello CA,Gross MA,Weiner RS

更新日期：1987-01-01 00:00:00

abstract：:Ruxolitinib is JAK1/JAK2 inhibitor with established clinical benefit in myelofibrosis (MF). We analyzed long-term outcomes of 107 patients with intermediate-2 or high-risk MF receiving ruxolitinib at MD Anderson Cancer Center (MDACC) on phase 1/2 trial. After a median of 32 months of follow-up, 58 patients (54%) were ...

journal_title：Blood

authors： Verstovsek S,Kantarjian HM,Estrov Z,Cortes JE,Thomas DA,Kadia T,Pierce S,Jabbour E,Borthakur G,Rumi E,Pungolino E,Morra E,Caramazza D,Cazzola M,Passamonti F

更新日期：2012-08-09 00:00:00

abstract：:Patients with congenital T lymphocyte deficiency disorders received transplants with parental bone marrow depleted of mature T cells by the use of an anti-T cell monoclonal antibody (CT-2) and complement. Our results with 16 consecutive patients (20 transplants) showed rapid engraftment of donor cells; cytoreduction (...

journal_title：Blood

authors： Moen RC,Horowitz SD,Sondel PM,Borcherding WR,Trigg ME,Billing R,Hong R

更新日期：1987-09-01 00:00:00

abstract：:The periprocedural management of patients receiving long-term oral anticoagulant therapy remains a common but difficult clinical problem, with a lack of high-quality evidence to inform best practices. It is a patient's thromboembolic risk that drives the need for an aggressive periprocedural strategy, including the us...

journal_title：Blood

authors： Spyropoulos AC,Douketis JD

更新日期：2012-10-11 00:00:00

abstract：:To investigate genetic variants that affect iron concentrations in persons not affected by overt genetic disorders of iron metabolism, a genome-wide association study was conducted in the InCHIANTI Study (N = 1206) and the Baltimore Longitudinal Study of Aging (N = 713). The top 2 single-nucleotide polymorphisms were ...

journal_title：Blood

authors： Tanaka T,Roy CN,Yao W,Matteini A,Semba RD,Arking D,Walston JD,Fried LP,Singleton A,Guralnik J,Abecasis GR,Bandinelli S,Longo DL,Ferrucci L

更新日期：2010-01-07 00:00:00

abstract：:Previously, it was believed that megakaryocytopoiesis was regulated by two types of humoral factors: megakaryocyte colony-stimulating factor (MK-CSF), which acts on progenitors inducing their proliferation, and thrombopoietin (TPO), a megakaryocyte(s) (MK) maturational factor that induces platelet formation. The recen...

journal_title：Blood

authors： Debili N,Wendling F,Katz A,Guichard J,Breton-Gorius J,Hunt P,Vainchenker W

更新日期：1995-10-01 00:00:00

abstract：:Dendritic cells are the most potent antigen-presenting cells of the immune system. Although dendritic cells are likely to secrete selective cytokines that facilitate antigen presentation, the difficulty in isolating pure dendritic cells in sufficient numbers has made assessment of this function imprecise. In this stud...

journal_title：Blood

authors： Zhou LJ,Tedder TF

更新日期：1995-11-01 00:00:00

abstract：:An 81-year-old woman, who presented with sudden episodes of spontaneous bleeding, was found to have a specific inhibitor of factor XIII. Her fibrin clots had approximately 70% gamma-gamma and no alpha polymer formation, under conditions where normal fibrin was fully cross-linked; the patient's clots were soluble in ur...

journal_title：Blood

authors： Fukue H,Anderson K,McPhedran P,Clyne L,McDonagh J

更新日期：1992-01-01 00:00:00

abstract：:The myelodysplastic syndromes (MDSs) are hematologically diverse hematopoietic stem cell malignancies primarily affecting older individuals. The incidence of MDS in the United States is estimated at 3.3 per 100 000; however, evidence suggests underreporting of MDS to centralized cancer registries. Contrary to clinical...

journal_title：Blood

authors： Cogle CR,Craig BM,Rollison DE,List AF

更新日期：2011-06-30 00:00:00

abstract：:The rapid recovery of hematopoiesis after allogeneic blood stem cell transplantation has been attributed to the quality and quantity of hematopoietic progenitors in the blood stem cell grafts from filgrastim-stimulated donors. To determine whether further stimulation with filgrastim after transplantation would affect ...

journal_title：Blood

authors： Przepiorka D,Smith TL,Folloder J,Anderlini P,Chan KW,Körbling M,Lichtiger B,Norfleet F,Champlin R

更新日期：2001-06-01 00:00:00

abstract：:Growing evidence indicates antibody-dependent cellular cytotoxicity (ADCC) contributes to the clinical response to monoclonal antibody (mAb) therapy of lymphoma. Recent in vitro analysis suggests C3b can inhibit mAb-induced natural killer (NK)-cell activation and ADCC. Further studies were conducted to assess the effe...

journal_title：Blood

authors： Wang SY,Veeramani S,Racila E,Cagley J,Fritzinger DC,Vogel CW,St John W,Weiner GJ

更新日期：2009-12-17 00:00:00

abstract：:Fifty patients with Philadelphia chromosome-positive (Ph(+)) chronic myelogenous leukemia (CML) in early chronic phase received imatinib mesylate, 400 mg orally daily. After a median follow-up of 9 months, 49 patients (98%) achieved a complete hematologic response and 45 patients (90%) achieved a major cytogenetic res...

journal_title：Blood

authors： Kantarjian HM,Cortes JE,O'Brien S,Giles F,Garcia-Manero G,Faderl S,Thomas D,Jeha S,Rios MB,Letvak L,Bochinski K,Arlinghaus R,Talpaz M

更新日期：2003-01-01 00:00:00

abstract：:FTY720 is an immunosuppressant developed to prevent organ transplant rejection. Recent studies indicate an additional role for FTY720 in inducing cell apoptosis. We demonstrate here that FTY720 mediates toxic effects in cell lines representing different B-cell malignancies and primary B cells from patients with chroni...

journal_title：Blood

authors： Liu Q,Zhao X,Frissora F,Ma Y,Santhanam R,Jarjoura D,Lehman A,Perrotti D,Chen CS,Dalton JT,Muthusamy N,Byrd JC

更新日期：2008-01-01 00:00:00

abstract：:CTLA-4 is a critical inhibitory "checkpoint" molecule of immune activation. Several recent reports have described patients with immune dysregulation and lymphoproliferative disease resulting from 2 different genetic diseases that directly or indirectly cause CTLA-4 deficiency. Numerous articles have also been publishe...

journal_title：Blood

authors： Lo B,Fritz JM,Su HC,Uzel G,Jordan MB,Lenardo MJ

更新日期：2016-08-25 00:00:00

abstract：:The development of alloantibodies against von Willebrand factor (VWF) represents a rare but serious complication of treatment of von Willebrand disease (VWD), occurring in ~5% to 10% of type 3 VWD patients. Affected patients can present with a range of symptoms, including lack or loss of hemostatic response to infused...

journal_title：Blood

authors： James PD,Lillicrap D,Mannucci PM

更新日期：2013-08-01 00:00:00

abstract：:We recently demonstrated that immunoglobulin E (IgE), in the absence of cross-linking agents, activates signaling pathways in healthy murine bone marrow-derived mast cells (BMMCs) and that this activation enhances BMMC survival, at least in part, via secretion of autocrine-acting cytokines. We report herein that IgE a...

journal_title：Blood

authors： Lam V,Kalesnikoff J,Lee CW,Hernandez-Hansen V,Wilson BS,Oliver JM,Krystal G

更新日期：2003-08-15 00:00:00

abstract：:Signal transducer and activator of transcription 3 (STAT3) is considered a negative regulator of inflammation, as inhibition of STAT3 signaling enhances antitumor immunity. However, STAT3 activation is a key oncogenic pathway in natural killer (NK)-lineage large granular lymphomas, and we recently reported enhanced pr...

journal_title：Blood

authors： Zhu S,Phatarpekar PV,Denman CJ,Senyukov VV,Somanchi SS,Nguyen-Jackson HT,Mace EM,Freeman AF,Watowich SS,Orange JS,Holland SM,Lee DA

更新日期：2014-07-17 00:00:00

abstract：:Sardinian beta-thalassemia patients all are homozygotes for the same null allele in the beta-globin gene, but the clinical manifestations are extremely variable in severity. Previous studies have shown that the coinheritance of alpha-thalassemia or the presence of genetic variants that sustain fetal hemoglobin product...

journal_title：Blood

authors： Galanello R,Sanna S,Perseu L,Sollaino MC,Satta S,Lai ME,Barella S,Uda M,Usala G,Abecasis GR,Cao A

更新日期：2009-10-29 00:00:00

abstract：:Primary central nervous system lymphoma (PCNSL) is confined to the brain, eyes, and cerebrospinal fluid without evidence of systemic spread. Rarely, PCNSL occurs in the context of immunosuppression, e.g. post-transplant lymphoproliferative disorders (PTLD) or HIV (AIDS-related PCNSL). These cases are poorly characteri...

journal_title：Blood

authors： Gandhi MK,Hoang T,Law SC,Brosda S,O'Rourke K,Tobin JWD,Vari F,Murigneux V,Fink JL,Gunawardana J,Gould CM,Oey H,Bednarska K,Delecluse S,Trappe RU,de Long LM,Sabdia MB,Bhagat G,Hapgood G,Blyth E,Clancy LE,Wight J

更新日期：2020-11-17 00:00:00

abstract：:CXC ligand 12 (CXCL12; also known as stromal cell-derived factor 1alpha/SDF-1alpha) chemoattracts hematopoietic stem and progenitor cells (HSCs/HPCs) and is thought to play a crucial role in the mobilization of HSCs/HPCs from the bone marrow. CD26 (dipeptidylpeptidase IV [DPPIV]) is a membrane-bound extracellular pept...

journal_title：Blood

authors： Christopherson KW 2nd,Cooper S,Broxmeyer HE

更新日期：2003-06-15 00:00:00

abstract：:Chronic lymphocytic leukemia is characterized by relapse after treatment and chemotherapy resistance. Similarly, in other malignancies leukemia cells accumulate mutations during growth, forming heterogeneous cell populations that are subject to Darwinian selection and may respond differentially to treatment. There is ...

journal_title：Blood

authors： Schuh A,Becq J,Humphray S,Alexa A,Burns A,Clifford R,Feller SM,Grocock R,Henderson S,Khrebtukova I,Kingsbury Z,Luo S,McBride D,Murray L,Menju T,Timbs A,Ross M,Taylor J,Bentley D

更新日期：2012-11-15 00:00:00

abstract：:Fanconi anemia (FA) is a genetic disease characterized by congenital abnormalities, bone marrow failure, and susceptibility to leukemia and other cancers. FANCJ, one of 13 genes linked to FA, encodes a DNA helicase proposed to operate in homologous recombination repair and replicational stress response. The pathogenic...

journal_title：Blood

authors： Wu Y,Sommers JA,Suhasini AN,Leonard T,Deakyne JS,Mazin AV,Shin-Ya K,Kitao H,Brosh RM Jr

更新日期：2010-11-11 00:00:00