- Mutations in GATA2 are associated with the autosomal dominant and sporadic monocytopenia and mycobacterial infection (MonoMAC) syndrome
[作者:Hsu, AP; Sampaio, EP; Khan, J; Calvo, KR; Lemieux, JE; Patel, SY; Frucht, DM; Vinh, DC; Auth, RD; Freeman, AF; Olivier, KN; Uzel, G; Zerbe, CS; Spalding, C; Pittaluga, S; Raffeld, M; Kuhns, DB; Ding, L; Paulson, ML; Marciano, BE; Gea-Banacloche, JC; Orange, JS; Cuellar-Rodriguez, J; Hickstein, DD; Holland, SM,期刊:Blood, 页码:2653-2655 , 文章类型: Article,,卷期:2011年118-10]
- The syndrome of monocytopenia, B-cell and NK-cell lymphopenia, and mycobacterial, fungal, and viral infections is associated with myelodysplasia, cytogenetic abnormalities, pulmonary alveolar proteinosis, and myeloid leu...
- Exome sequencing identifies GATA-2 mutation as the cause of dendritic cell, monocyte, B and NK lymphoid deficiency
[作者:Dickinson, RE; Griffin, H; Bigley, V; Reynard, LN; Hussain, R; Haniffa, M; Lakey, JH; Rahman, T; Wang, XN; McGovern, N; Pagan, S; Cookson, S; McDonald, D; Chua, I; Wallis, J; Cant, A; Wright, M; Keavney, B; Chinnery, PF; Loughlin, J; Hambleton, S; Santibanez-Koref, M; Collin, M,期刊:Blood, 页码:2656-2658 , 文章类型: Article,,卷期:2011年118-10]
- The human syndrome of dendritic cell, monocyte, B and natural killer lymphoid deficiency presents as a sporadic or autosomal dominant trait causing susceptibility to mycobacterial and other infections, predisposition to ...
- Thrombophilia and outcomes of assisted reproduction technologies: a systematic review and meta-analysis
[作者:Di Nisio, M; Rutjes, AWS; Ferrante, N; Tiboni, GM; Cuccurullo, F; Porreca, E,期刊:Blood, 页码:2670-2678 , 文章类型: Review,,卷期:2011年118-10]
- Thrombophilia has been associated with pregnancy complications and recurrent miscarriage. The aim of this systematic review was to evaluate the controversial association between thrombophilia and failures of assisted rep...
- Myeloid dysplasia and bone marrow hypocellularity in adenosine deaminase-deficient severe combined immune deficiency
[作者:Sokolic, R; Maric, I; Kesserwan, C; Garabedian, E; Hanson, IC; Dodds, M; Buckley, R; Issekutz, AC; Kamani, N; Shaw, K; Tan, B; Bali, P; Hershfield, MS; Kohn, DB; Wayne, AS; Candotti, F,期刊:Blood, 页码:2688-2694 , 文章类型: Article,,卷期:2011年118-10]
- Genetic deficiency of adenosine deaminase (ADA) can cause profound lymphopenia and result in the clinical presentation of severe combined immune deficiency (SCID). However, because of the ubiquitous expression of ADA, AD...
- Enhanced pharmacokinetic properties of a glycoPEGylated recombinant factor IX: a first human dose trial in patients with hemophilia B
[作者:Negrier, C; Knobe, K; Tiede, A; Giangrande, P; Moss, J,期刊:Blood, 页码:2695-2701 , 文章类型: Article,,卷期:2011年118-10]
- Replacement therapy with factor IX (FIX) concentrates is the recommended treatment for patients with hemophilia B, an X-linked bleeding disorder occurring in 1: 25 000 male births. N9-GP is a recombinant FIX molecule wit...
- BCR-ABL suppresses autophagy through ATF5-mediated regulation of mTOR transcription
[作者:Sheng, Z; Ma, LY; Sun, JYE; Zhu, LHJ; Green, MR,期刊:Blood, 页码:2840-2848 , 文章类型: Article,,卷期:2011年118-10]
- The oncoprotein BCR-ABL transforms myeloid progenitor cells and is responsible for the development of chronic myeloid leukemia (CML). In transformed cells, BCR-ABL suppresses apoptosis as well as autophagy, a catabolic p...
- Differential niche and Wnt requirements during acute myeloid leukemia progression
[作者:Lane, SW; Wang, YZJ; Lo Celso, C; Ragu, C; Bullinger, L; Sykes, SM; Ferraro, F; Shterental, S; Lin, CP; Gilliland, DG; Scadden, DT; Armstrong, SA; Williams, DA,期刊:Blood, 页码:2849-2856 , 文章类型: Article,,卷期:2011年118-10]
- Hematopoietic stem cells (HSCs) engage in complex bidirectional signals with the hematopoietic microenvironment (HM), and there is emerging evidence that leukemia stem cells (LSCs) may use similar interactions. Using a s...
- MSI2 protein expression predicts unfavorable outcome in acute myeloid leukemia
[作者:Byers, RJ; Currie, T; Tholouli, E; Rodig, SJ; Kutok, JL,期刊:Blood, 页码:2857-2867 , 文章类型: Article,,卷期:2011年118-10]
- MSI2 is highly expressed in human myeloid leukemia (AML) cell lines, and high expression of MSI2 mRNA is associated with decreased survival in AML, suggesting its use as a new prognostic marker. To test this, we measured...
- A pilot study of subcutaneous decitabine in beta-thalassemia intermedia
[作者:Olivieri, NF; Saunthararajah, Y; Thayalasuthan, V; Kwiatkowski, J; Ware, RE; Kuypers, FA; Kim, HY; Trachtenberg, FL; Vichinsky, EP,期刊:Blood, 页码:2708-2711 , 文章类型: Article,,卷期:2011年118-10]
- Ineffective erythropoiesis, the hallmark of beta-thalassemia, is a result of alpha/non-alpha globin chain imbalance.(1) One strategy to redress globin-chain imbalance is to induce gamma-globin gene (HBG) expression. Repr...
- Stromal pleiotrophin regulates repopulation behavior of hematopoietic stem cells
[作者:Istvanffy, R; Kroger, M; Eckl, C; Gitzelmann, S; Vilne, B; Bock, F; Graf, S; Schiemann, M; Keller, UB; Peschel, C; Oostendorp, RAJ,期刊:Blood, 页码:2712-2722 , 文章类型: Article,,卷期:2011年118-10]
- Pleiotrophin (Ptn) is strongly expressed by stromal cells which maintain HSCs. However, in vivo, Ptn deficiency does not alter steady-state hematopoiesis. However, knockdown of Ptn (Ptn(KD)) in stromal cells increases pr...
- Identification of Flt3(+)CD150(-) myeloid progenitors in adult mouse bone marrow that harbor T lymphoid developmental potential
[作者:Chi, AWS; Chavez, A; Xu, LW; Weber, BN; Shestova, O; Schaffer, A; Wertheim, G; Pear, WS; Izon, D; Bhandoola, A,期刊:Blood, 页码:2723-2732 , 文章类型: Article,,卷期:2011年118-10]
- Common myeloid progenitors (CMPs) were first identified as progenitors that were restricted to myeloid and erythroid lineages. However, it was recently demonstrated that expression of both lymphoidand myeloid-related gen...
- The critical role of agrin in the hematopoietic stem cell niche
[作者:Mazzon, C; Anselmo, A; Cibella, J; Soldani, C; Destro, A; Kim, N; Roncalli, M; Burden, SJ; Dustin, ML; Sarukhan, A; Viola, A,期刊:Blood, 页码:2733-2742 , 文章类型: Article,,卷期:2011年118-10]
- Hematopoiesis is the process leading to the sustained production of blood cells by hematopoietic stem cells (HSCs). Growth, survival, and differentiation of HSCs occur in specialized microenvironments called "hematopoiet...
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