Cover Image

Cover Image

A homology model of the ADAMTS13 metalloprotease (magenta) and disintegrin-like domains (cyan) is shown in the center, with the active site Zn2+ ion (green) and the three structural Ca2+ ions (grey) shown as spheres. ADAMTS13 is flanked by the native folded VWF A2 domain (top left) in which the target Tyr1605-Met1606 (red) bond is buried in the fourth strand of a beta sheet, and by a partially unfolded VWF A2 domain (bottom left) in which this bond is exposed to ADAMTS13. See the related article by Gao et al beginning on page 1713.
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Table of Contents

INSIDE BLOOD

VEGF-D: a modifier of embryonic lymphangiogenesis Free

Shear tango: dance of the ADAMTS13/VWF complex Free

Where does von Willebrand factor go? Free

Genomic complexity in chronic lymphocytic leukemia Free

Complement dances with T cells Free

Diamond-Blackfan anemia: a new facet Free

GASing up iron delivery via STAT5 Free

Blood group antigens reveal their maker Free

ASH 50TH ANNIVERSARY REVIEW

CD4 T cells: fates, functions, and faults Free

B lymphocytes: how they develop and function Free

BLOOD WORK

Target cells and hemoglobinopathy Free

PLENARY PAPERS

Abnormalities of the large ribosomal subunit protein, Rpl35a, in Diamond-Blackfan anemia Free

REVIEW ARTICLES

Bortezomib-induced peripheral neuropathy in multiple myeloma: a comprehensive review of the literature Free

European Organization for Research and Treatment of Cancer and International Society for Cutaneous Lymphoma consensus recommendations for the management of cutaneous B-cell lymphomas Free

CHEMOKINES, CYTOKINES, AND INTERLEUKINS

CC chemokine receptor 5 gene promoter activation by the cyclic AMP response element binding transcription factor Free

Increased serum levels of the chemokine CXCL13 and up-regulation of its gene expression are distinctive features of HCV-related cryoglobulinemia and correlate with active cutaneous vasculitis Free

CLINICAL TRIALS AND OBSERVATIONS

The natural history and treatment outcome of blast phase BCR-ABL− myeloproliferative neoplasms Free Clinical Trials & Observations

A randomized study of clofarabine versus clofarabine plus low-dose cytarabine as front-line therapy for patients aged 60 years and older with acute myeloid leukemia and high-risk myelodysplastic syndrome Free Clinical Trials & Observations

What determines the outcomes for adolescents and young adults with acute lymphoblastic leukemia treated on cooperative group protocols? A comparison of Children's Cancer Group and Cancer and Leukemia Group B studies Free Clinical Trials & Observations

Successful treatment of progressive cutaneous mastocytosis with imatinib in a 2-year-old boy carrying a somatic KIT mutation Free Brief Report

CNS Hodgkin lymphoma Free Brief Report

GENE THERAPY

Transient blockade of the inducible costimulator pathway generates long-term tolerance to factor VIII after nonviral gene transfer into hemophilia A mice Free

HEMATOPOIESIS AND STEM CELLS

Stem cell–specific epigenetic priming and B cell–specific transcriptional activation at the mouse Cd19 locus Free

Defective homing is associated with altered Cdc42 activity in cells from patients with Fanconi anemia group A Free Brief Report

HEMOSTASIS, THROMBOSIS, AND VASCULAR BIOLOGY

Hydroxychloroquine directly reduces the binding of antiphospholipid antibody–β2-glycoprotein I complexes to phospholipid bilayers Free

CalDAG-GEFI and protein kinase C represent alternative pathways leading to activation of integrin αIIbβ3 in platelets Free

Macrophages contribute to the cellular uptake of von Willebrand factor and factor VIII in vivo Free

Extensive contacts between ADAMTS13 exosites and von Willebrand factor domain A2 contribute to substrate specificity Free

Overexpression of delta-like 4 induces arterialization and attenuates vessel formation in developing mouse embryos Free

Tetraspanin family member CD9 inhibits Aggrus/podoplanin-induced platelet aggregation and suppresses pulmonary metastasis Free

Role of VEGF-D and VEGFR-3 in developmental lymphangiogenesis, a chemicogenetic study in Xenopus tadpoles Free

IMMUNOBIOLOGY

Defective innate immunity predisposes murine neonates to poor sepsis outcome but is reversed by TLR agonists Free

Locally produced C5a binds to T cell–expressed C5aR to enhance effector T-cell expansion by limiting antigen-induced apoptosis Free

Rac GTPase isoforms Rac1 and Rac2 play a redundant and crucial role in T-cell development Free

Human natural killer cells exposed to IL-2, IL-12, IL-18, or IL-4 differently modulate priming of naive T cells by monocyte-derived dendritic cells Free

STAT3 is required for IL-21–induced secretion of IgE from human naive B cells Free

The familial Mediterranean fever protein, pyrin, is cleaved by caspase-1 and activates NF-κB through its N-terminal fragment Free

Spi-B inhibits human plasma cell differentiation by repressing BLIMP1 and XBP-1 expression Free

Notch1 and TGFβ1 cooperatively regulate Foxp3 expression and the maintenance of peripheral regulatory T cells Free

Adenosine receptors in regulation of dendritic cell differentiation and function Free

STAT3- and STAT5-dependent pathways competitively regulate the pan-differentiation of CD34pos cells into tumor-competent dendritic cells Free

Targeting a single mismatched minor histocompatibility antigen with tumor-restricted expression eradicates human solid tumors Free

β2 integrin phosphorylation on Thr758 acts as a molecular switch to regulate 14-3-3 and filamin binding Free

A novel polymorphism of the human CD40 receptor with enhanced function Free

Detection of T lymphocytes with a second-site mutation in skin lesions of atypical X-linked severe combined immunodeficiency mimicking Omenn syndrome Free Brief Report

NEOPLASIA

Cytotoxic T lymphocytes directed to the preferentially expressed antigen of melanoma (PRAME) target chronic myeloid leukemia Free

HDAC6 inhibition enhances 17-AAG–mediated abrogation of hsp90 chaperone function in human leukemia cells Free

Analysis of clonotypic switch junctions reveals multiple myeloma originates from a single class switch event with ongoing mutation in the isotype-switched progeny Free

Dll4 activation of Notch signaling reduces tumor vascularity and inhibits tumor growth Free

Effective elimination of fludarabine-resistant CLL cells by PEITC through a redox-mediated mechanism Free

Relative value of ZAP-70, CD38, and immunoglobulin mutation status in predicting aggressive disease in chronic lymphocytic leukemia Free

Biologic and genetic characterization of the novel amyloidogenic lambda light chain–secreting human cell lines, ALMC-1 and ALMC-2 Free

Transcriptional repression of c-Myb and GATA-2 is involved in the biologic effects of C/EBPα in p210BCR/ABL-expressing cells Free

Sμ mutation patterns suggest different progression pathways in follicular lymphoma: early direct or late from FL progenitor cells Free

Absence of SKP2 expression attenuates BCR-ABL–induced myeloproliferative disease Free

Gossypol, a BH3 mimetic, induces apoptosis in chronic lymphocytic leukemia cells Free

Id1 is a common downstream target of oncogenic tyrosine kinases in leukemic cells Free

Genomic complexity identifies patients with aggressive chronic lymphocytic leukemia Free

Primary cystic lung light chain deposition disease: a clinicopathologic entity derived from unmutated B cells with a stereotyped IGHV4-34/IGKV1 receptor Free

DNA hypermethylation and epigenetic silencing of the tumor suppressor gene, SLC5A8, in acute myeloid leukemia with the MLL partial tandem duplication Free Brief Report

Leukemic transformation in mice expressing a NUP98-HOXD13 transgene is accompanied by spontaneous mutations in Nras, Kras, and Cbl Free Brief Report

mTOR inhibitors are synergistic with methotrexate: an effective combination to treat acute lymphoblastic leukemia Free Brief Report

Multidrug resistance gene (MDR1) polymorphisms are associated with major molecular responses to standard-dose imatinib in chronic myeloid leukemia Free Brief Report

PHAGOCYTES

Toll-like receptor–mediated activation of neutrophils by influenza A virus Free

Separable requirements for cytoplasmic domain of PSGL-1 in leukocyte rolling and signaling under flow Free

VEGF-D: a modifier of embryonic lymphangiogenesis

Shear tango: dance of the ADAMTS13/VWF complex

Where does von Willebrand factor go?

Genomic complexity in chronic lymphocytic leukemia

Complement dances with T cells

Diamond-Blackfan anemia: a new facet

GASing up iron delivery via STAT5

Blood group antigens reveal their maker

CD4 T cells: fates, functions, and faults

B lymphocytes: how they develop and function

Target cells and hemoglobinopathy

Abnormalities of the large ribosomal subunit protein, Rpl35a, in Diamond-Blackfan anemia

Bortezomib-induced peripheral neuropathy in multiple myeloma: a comprehensive review of the literature

European Organization for Research and Treatment of Cancer and International Society for Cutaneous Lymphoma consensus recommendations for the management of cutaneous B-cell lymphomas

CC chemokine receptor 5 gene promoter activation by the cyclic AMP response element binding transcription factor

Increased serum levels of the chemokine CXCL13 and up-regulation of its gene expression are distinctive features of HCV-related cryoglobulinemia and correlate with active cutaneous vasculitis

The natural history and treatment outcome of blast phase BCR-ABL⁻ myeloproliferative neoplasms
Clinical Trials & Observations

A randomized study of clofarabine versus clofarabine plus low-dose cytarabine as front-line therapy for patients aged 60 years and older with acute myeloid leukemia and high-risk myelodysplastic syndrome
Clinical Trials & Observations

What determines the outcomes for adolescents and young adults with acute lymphoblastic leukemia treated on cooperative group protocols? A comparison of Children's Cancer Group and Cancer and Leukemia Group B studies
Clinical Trials & Observations

Successful treatment of progressive cutaneous mastocytosis with imatinib in a 2-year-old boy carrying a somatic KIT mutation
Brief Report

CNS Hodgkin lymphoma
Brief Report

Transient blockade of the inducible costimulator pathway generates long-term tolerance to factor VIII after nonviral gene transfer into hemophilia A mice

Stem cell–specific epigenetic priming and B cell–specific transcriptional activation at the mouse Cd19 locus

Defective homing is associated with altered Cdc42 activity in cells from patients with Fanconi anemia group A
Brief Report

Hydroxychloroquine directly reduces the binding of antiphospholipid antibody–β2-glycoprotein I complexes to phospholipid bilayers

CalDAG-GEFI and protein kinase C represent alternative pathways leading to activation of integrin αIIbβ3 in platelets

Macrophages contribute to the cellular uptake of von Willebrand factor and factor VIII in vivo

Extensive contacts between ADAMTS13 exosites and von Willebrand factor domain A2 contribute to substrate specificity

Overexpression of delta-like 4 induces arterialization and attenuates vessel formation in developing mouse embryos

Tetraspanin family member CD9 inhibits Aggrus/podoplanin-induced platelet aggregation and suppresses pulmonary metastasis

Role of VEGF-D and VEGFR-3 in developmental lymphangiogenesis, a chemicogenetic study in Xenopus tadpoles

Defective innate immunity predisposes murine neonates to poor sepsis outcome but is reversed by TLR agonists

Locally produced C5a binds to T cell–expressed C5aR to enhance effector T-cell expansion by limiting antigen-induced apoptosis

Rac GTPase isoforms Rac1 and Rac2 play a redundant and crucial role in T-cell development

Human natural killer cells exposed to IL-2, IL-12, IL-18, or IL-4 differently modulate priming of naive T cells by monocyte-derived dendritic cells

STAT3 is required for IL-21–induced secretion of IgE from human naive B cells

The familial Mediterranean fever protein, pyrin, is cleaved by caspase-1 and activates NF-κB through its N-terminal fragment

Spi-B inhibits human plasma cell differentiation by repressing BLIMP1 and XBP-1 expression

Notch1 and TGFβ1 cooperatively regulate Foxp3 expression and the maintenance of peripheral regulatory T cells

Adenosine receptors in regulation of dendritic cell differentiation and function

STAT3- and STAT5-dependent pathways competitively regulate the pan-differentiation of CD34^pos cells into tumor-competent dendritic cells

Targeting a single mismatched minor histocompatibility antigen with tumor-restricted expression eradicates human solid tumors

β2 integrin phosphorylation on Thr758 acts as a molecular switch to regulate 14-3-3 and filamin binding

A novel polymorphism of the human CD40 receptor with enhanced function

Detection of T lymphocytes with a second-site mutation in skin lesions of atypical X-linked severe combined immunodeficiency mimicking Omenn syndrome
Brief Report

Cytotoxic T lymphocytes directed to the preferentially expressed antigen of melanoma (PRAME) target chronic myeloid leukemia

HDAC6 inhibition enhances 17-AAG–mediated abrogation of hsp90 chaperone function in human leukemia cells

Analysis of clonotypic switch junctions reveals multiple myeloma originates from a single class switch event with ongoing mutation in the isotype-switched progeny

Dll4 activation of Notch signaling reduces tumor vascularity and inhibits tumor growth

Effective elimination of fludarabine-resistant CLL cells by PEITC through a redox-mediated mechanism

Relative value of ZAP-70, CD38, and immunoglobulin mutation status in predicting aggressive disease in chronic lymphocytic leukemia

Biologic and genetic characterization of the novel amyloidogenic lambda light chain–secreting human cell lines, ALMC-1 and ALMC-2

Transcriptional repression of c-Myb and GATA-2 is involved in the biologic effects of C/EBPα in p210^BCR/ABL-expressing cells

Sμ mutation patterns suggest different progression pathways in follicular lymphoma: early direct or late from FL progenitor cells

Absence of SKP2 expression attenuates BCR-ABL–induced myeloproliferative disease

Gossypol, a BH3 mimetic, induces apoptosis in chronic lymphocytic leukemia cells

Id1 is a common downstream target of oncogenic tyrosine kinases in leukemic cells

Genomic complexity identifies patients with aggressive chronic lymphocytic leukemia

Primary cystic lung light chain deposition disease: a clinicopathologic entity derived from unmutated B cells with a stereotyped IGHV4-34/IGKV1 receptor

DNA hypermethylation and epigenetic silencing of the tumor suppressor gene, SLC5A8, in acute myeloid leukemia with the MLL partial tandem duplication
Brief Report

Leukemic transformation in mice expressing a NUP98-HOXD13 transgene is accompanied by spontaneous mutations in Nras, Kras, and Cbl
Brief Report

mTOR inhibitors are synergistic with methotrexate: an effective combination to treat acute lymphoblastic leukemia
Brief Report

Multidrug resistance gene (MDR1) polymorphisms are associated with major molecular responses to standard-dose imatinib in chronic myeloid leukemia
Brief Report

Toll-like receptor–mediated activation of neutrophils by influenza A virus

Separable requirements for cytoplasmic domain of PSGL-1 in leukocyte rolling and signaling under flow

Granulocyte colony-stimulating factor delays neutrophil apoptosis by inhibition of calpains upstream of caspase-3

A novel loss-of-function mutation in the proton-coupled folate transporter from a patient with hereditary folate malabsorption reveals that Arg 113 is crucial for function

HES1 is a novel interactor of the Fanconi anemia core complex

Hematopoietic-specific Stat5-null mice display microcytic hypochromic anemia associated with reduced transferrin receptor gene expression

Mutations in EKLF/KLF1 form the molecular basis of the rare blood group In(Lu) phenotype

Two nonsense mutations in the TMPRSS6 gene in a patient with microcytic anemia and iron deficiency
Brief Report

Donor demographic and laboratory predictors of allogeneic peripheral blood stem cell mobilization in an ethnically diverse population

Absence of donor Th17 leads to augmented Th1 differentiation and exacerbated acute graft-versus-host disease

Graft-versus-host disease causes failure of donor hematopoiesis and lymphopoiesis in interferon-γ receptor-deficient hosts

Mannose-binding lectin status is associated with risk of major infection following myeloablative sibling allogeneic hematopoietic stem cell transplantation

In vivo–activated CD103⁺CD4⁺ regulatory T cells ameliorate ongoing chronic graft-versus-host disease

Angiopoietin-2 predicts disease-free survival after allogeneic stem cell transplantation in patients with high-risk myeloid malignancies

Peripheral deletional tolerance of alloreactive CD8 but not CD4 T cells is dependent on the PD-1/PD-L1 pathway

A single nucleotide polymorphism at chromosome 2q21.3 (LCT −13910C>T) associates with clinical outcome after allogeneic hematopoietic stem cell transplantation
Brief Report

Expansion of donor-derived hematopoietic stem cells with PIGA mutation associated with late graft failure after allogeneic stem cell transplantation
Brief Report