Abstract：
:To investigate the value of genetic mutations in the pathogenesis and differential diagnosis of hemophagocytic lymphohistiocytosis (HLH), mutations related to blood and immune deficiency genes were analyzed in patients with HLH. Peripheral blood samples from 33 children diagnosed with HLH on the basis of the 2004 diagnostic criteria were collected, and 317 genes related to blood system diseases and 562 genes related to immunodeficiency were detected by second-generation targeted sequencing technology, bioinformatic analysis, and parental verification analysis. A total of 159 mutations related to blood system diseases and immunodeficiency were found in 33 patients, including 7 HLH-related gene mutations (UNC13D, XIAP, LYST, STX11, ITK, PRF1, and SRGN) in 12 patients. UNC13D was found in 6 patients, with the highest frequency. Two cases (6.1%, 2/33) were diagnosed as primary hemophagocytic lymphohistiocytosis (pHLH), and 6 cases (18.2%, 6/33) were diagnosed as primary immunodeficiency disease (PID) or hereditary hematopathy; the remainder were diagnosed as secondary hemophagocytic lymphohistiocytosis (sHLH). It is necessary to detect blood and immunodeficiency genes to exclude the possibility of pHLH, PID, or hereditary hematopathy associated with HLH for children.
journal_name
Exp Hematoljournal_title
Experimental hematologyauthors
Mo W,Wei W,Sun Y,Yang Y,Guan Z,Li M,Zhu P,Chi Zdoi
10.1016/j.exphem.2019.09.024subject
Has Abstractpub_date
2019-10-01 00:00:00pages
62-69eissn
0301-472Xissn
1873-2399pii
S0301-472X(19)31026-4journal_volume
78pub_type
临床试验,杂志文章abstract：OBJECTIVE:To study the early time course of engraftment of human mesenchymal stem cells in fetal sheep heart and determine the relative roles of proliferation and homing in formation of aggregates of human Purkinje fiber cells. METHODS:The human sheep xenograft model was utilized for these studies. Prior to injection ...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：10.1016/j.exphem.2006.04.019
更新日期：2006-07-01 00:00:00
abstract：:We have assessed the potential use of the mononuclear cell (MNC) content as the sole assessment of graft quality in 35 patients receiving BEAM (carmustine, etoposide, cytosine arabinoside, melphalan) chemotherapy and autologous peripheral blood progenitor cell (PBPC) transplantation for malignant lymphoma. PBPCs were ...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：
更新日期：1995-12-01 00:00:00
abstract：:Studies were undertaken to produce monoclonal antibodies directed against the murine receptor for macrophage colony-stimulating factor (M-CSF or CSF-1). Sprague-Dawley rats were injected with lysates prepared from a murine myelomonocytic cell line (RAW cell line) that has high levels of M-CSF receptors. Spleen cells f...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：
更新日期：1993-04-01 00:00:00
abstract：:Our previous studies have shown that a unique glycoprotease from Pasteurella haemolytica specifically cleaves only proteins containing sialylated O-linked glycans. The hematopoietic progenitor cell antigen, CD34, which is heavily glycosylated with both N- and O-linked glycans, is readily cleaved by this protease. In t...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：
更新日期：1992-06-01 00:00:00
abstract：:Induced pluripotent stem cells (iPS) derived from disease cells are expected to provide a new experimental material, especially for diseases from which samples are difficult to obtain. In this study, we generated iPS from samples from patients with primary and secondary myelofibrosis. The primary myelofibrosis cells h...
journal_title：Experimental hematology
pub_type： 临床试验,杂志文章
doi：10.1016/j.exphem.2014.03.010
更新日期：2014-09-01 00:00:00
abstract：:The aberrant overexpression of Wilms tumor-1 (WT1) in acute myeloid leukemia (AML) plays an important role in blast cell survival by enhancing proliferation and inhibiting apoptosis. However, the mechanism underlying the overexpression of WT1 remains unclear. Here, we identified miR-193a (miR-193a-5p) and miR-600 targ...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：10.1016/j.exphem.2018.02.001
更新日期：2018-05-01 00:00:00
abstract：OBJECTIVE:We studied two adult brothers with severe congenital thrombocytopenia in order to determine the genetic etiology of their inherited disorder. Despite the absence of eczema or immunodeficiency, a mutation of the Wiskott-Aldrich syndrome (WAS) gene was suspected because of the presence of microthrombocytes. MA...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：10.1016/s0301-472x(02)01023-8
更新日期：2003-02-01 00:00:00
abstract：:Human bone marrow contains plastic-adherent hemopoietic progenitor cells whose plating efficiency is increased by brief (2 h) exposure to methylprednisolone (MP). When subsequently covered with methylcellulose medium, they form colonies of monoblastoid cells. Colony size, but not number, and mature cell production are...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：
更新日期：1987-08-01 00:00:00
abstract：:The circadian and circannual rhythms of committed stem cells (CFUc) were investigated by using the agar colony technique. There was a marked circadian rhythmicity, with variations between maximum and minimum colony yields of 2-5.5 times; such variations could also be seen throughout the year. A phase shift was observe...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：
更新日期：1984-01-01 00:00:00
abstract：:Details are given of response and toxicity after high dose chemotherapy followed by autologous bone marrow infusion. High dose nitrosurea therapy (600-1000 mg/m2 BCNU) was predominantly associated with hematopoietic toxicity but recovery was within 4-5 weeks after BCNU therapy. High dose combination chemotherapy using...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：
更新日期：1979-01-01 00:00:00
abstract：:The tumor suppressor p53 is thought to play a role in megakaryocyte (MK) development. To assess the influence of the p53 regulatory pathway further, we studied the effect of RG7112, a small molecule MDM2 antagonist that activates p53 by preventing its interaction with MDM2, on normal megakaryocytopoiesis and platelet ...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：10.1016/j.exphem.2013.11.012
更新日期：2014-02-01 00:00:00
abstract：:Serum levels of monokines, including macrophage colony-stimulating factor (M-CSF), tumor necrosis factor-alpha (TNF-alpha), interleukin-1 alpha (IL-1 alpha) and IL-1 beta (IL-1 beta), were measured in patients with chronic renal failure in an attempt to clarify the kinetics of these cytokines in the course of renal an...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：
更新日期：1993-02-01 00:00:00
abstract：:An improved negative immunomagnetic selection strategy has been devised for the enrichment of primitive hemopoietic cells using the high proliferative potential colony-forming cell (HPP-CFC) assay as an index of stem cell purification. Immunomagnetic selection was carried out using goat anti-rat conjugated M-450 Dynab...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：
更新日期：1991-02-01 00:00:00
abstract：:The first blood cells observed in the embryo are large nucleated erythroblasts generated in blood islands of the extraembryonic yolk sac. These unique red cells have been termed primitive because of their resemblance to nucleated erythroblasts of nonmammalian species. It is now widely assumed that hematopoiesis in the...
journal_title：Experimental hematology
pub_type： 杂志文章,评审
doi：10.1016/j.exphem.2005.06.012
更新日期：2005-09-01 00:00:00
abstract：:An unusual case of graft failure following bone marrow transplantation for aplastic anemia is reported in which the patient had delayed and incomplete recovery of hematopoiesis despite documentation of sustained engraftment. The pathophysiology of graft failure following bone marrow transplantation is discussed and re...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：
更新日期：1984-10-01 00:00:00
abstract：:[3H]thymidine uptake by NFS-60 cells in microcultures was found to increase in a linear fashion with the increasing doses of purified recombinant human granulocyte colony-stimulating factor (rhG-CSF). Such increases were found neither with rhG-CSF samples pretreated with rabbit anti-rhG-CSF serum nor with other human ...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：
更新日期：1989-02-01 00:00:00
abstract：:The transcription factor RUNX1 is required in the embryo for formation of the adult hematopoietic system. Here, we describe the seminal findings that led to the discovery of RUNX1 and of its critical role in blood cell formation in the embryo from hemogenic endothelium (HE). We also present RNA-sequencing data demonst...
journal_title：Experimental hematology
pub_type： 杂志文章,评审
doi：10.1016/j.exphem.2018.10.009
更新日期：2018-12-01 00:00:00
abstract：OBJECTIVE:To assess the contribution of bone marrow (BM)-derived endothelial progenitor cells (EPCs) to the neovascularisation of cutaneous incisional wounds. METHODS:Lethally irradiated C57Bl/6 mice were transplanted with BM mononuclear cells from Tie2/lacZ mice, which constitutively overexpressed beta-galactosidase ...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：10.1016/j.exphem.2006.10.016
更新日期：2007-03-01 00:00:00
abstract：OBJECTIVE:Among Philadelphia chromosome-negative myeloproliferative neoplasms (Ph(-) MPN), essential thrombocythemia (ET) and the prefibrotic phase of primary myelofibrosis (PMF) represent two subtypes with considerable overlap. MATERIALS AND METHODS:In this study, histopathological classification of 490 MPN cases was...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：10.1016/j.exphem.2009.07.005
更新日期：2009-10-01 00:00:00
abstract：OBJECTIVE:To investigate whether Flk1(+)CD31(-)CD34(-) cells isolated from fetal bone marrow (BM) have characteristics of hemangioblasts, i.e., progenitors of endothelial and hematopoietic cells. MATERIALS AND METHODS:Mononuclear cells from fetal BM were negatively sorted by CD45, GlyA, and CD34 micromagnetic beads, t...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：10.1016/s0301-472x(03)00087-0
更新日期：2003-07-01 00:00:00
abstract：:The relative contributions of various organs to platelet production is controversial. In this study, serial histologic sections of bone marrow, spleen, liver, and lung from normal C57BL/6J mice and mice that had received three different agents which perturb normal murine thrombopoiesis (platelet antiserum, 5-fluoroura...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：
更新日期：1997-07-01 00:00:00
abstract：:The development of pluripotential hematopoietic stem cells (PHSC) requires the continuous support provided by the bone tissue and bone marrow (BM) stromal cells. The basic rule of spatial and temporal organization of the distinct stromal cells and differentiating hematopoietic cells in the course of development, regen...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：
更新日期：1995-04-01 00:00:00
abstract：:We have utilized two well-characterized human leukemia/lymphoma (LL) cell lines, UTMB-460 and CEM, to determine the role of integrin very late antigen-alpha 4 beta 1 (VLA-4) and its ligand vascular cell adhesion molecule-1 (VCAM-1) in the adherence of the LL cells to marrow stromal cells (MSC). Both these LL cell line...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：
更新日期：1993-03-01 00:00:00
abstract：:During chronic hypoxia, the number of splenic erythroid progenitor cells in mice, particularly CFU-E, increased dramatically but transiently. Since all three classes of erythroid progenitors in the femoral bone marrow were suppressed, a large part of this increase might be attributed to migration of CFU-E and/or their...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：
更新日期：1980-01-01 00:00:00
abstract：OBJECTIVE:Donor lymphocyte transfusions (DLT) are effective in the treatment of leukemia after allogeneic stem cell transplantation. Graft-vs-host-disease (GVHD) is the major risk factor of DLT. In dog leukocyte antigen (DLA)-identical littermate dogs, DLT given within 3 weeks after transplantation of marrow depleted o...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：10.1016/j.exphem.2009.05.001
更新日期：2009-08-01 00:00:00
abstract：OBJECTIVE:Generation and expansion of pluripotent unrestricted somatic stem cells (USSCs) and mesenchymal cells (MSCs) from human cord blood as well their functional characterization were assessed. METHODS:USSC generation was initiated from fresh and cryopreserved cord blood (CB). Culture conditions and functional ass...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：10.1016/j.exphem.2006.07.011
更新日期：2006-11-01 00:00:00
abstract：:Autotaxin (ATX) has been reported to act as a motility and growth factor in a variety of cancer cells. The ATX protein acts as a secreted lysophospholipase D by converting lysophosphatidylcholine (LPC) to lysophosphatidic acid (LPA), which signals via G-protein-coupled receptors and has important functions in cell mig...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：10.1016/j.exphem.2013.01.007
更新日期：2013-05-01 00:00:00
abstract：:The long-term effects of a maximum tolerated dose of doxorubicin (ADR) (10 mg/kg, LD10/60) on the recovery of the hematopoietic compartments of the femoral marrow from radiation (450 rad) were investigated over a 32-week interval using a mouse model. Comparative radiation response curves, estimating hematopoietic prol...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：
更新日期：1985-01-01 00:00:00
abstract：:Cell surface molecules aberrantly expressed or overexpressed by myeloid leukemic cells represent potential disease-specific therapeutic targets for antibodies. MUC1 is a polymorphic glycoprotein, the cleavage of which yields two unequal chains: a large extracellular α subunit containing a tandem repeat array bound in ...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：10.1016/j.exphem.2018.12.002
更新日期：2019-02-01 00:00:00
abstract：OBJECTIVE:Using transplantation of a clonal population of cells derived from a single hematopoietic stem cell (HSC) of transgenic enhanced green fluorescent protein (EGFP) mice, we have documented the hematopoietic origin of myofibroblasts, such as kidney mesangial cells and brain microglial cells. Because myofibroblas...
journal_title：Experimental hematology
pub_type： 杂志文章
doi：10.1016/j.exphem.2005.10.008
更新日期：2006-02-01 00:00:00
