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Showing papers in "Blood Reviews in 2010"


Journal ArticleDOI
TL;DR: This review will focus on the new evidence and concepts in HHT, placing these in context for both clinicians and scientists, with a particular emphasis on haematological settings.

414 citations


Journal ArticleDOI
TL;DR: The inherited marrow failure syndromes are a diverse set of genetic disorders characterized by hematopoietic aplasia and cancer predisposition and the clinical phenotypes are highly variable and much broader than previously recognized.

408 citations


Journal ArticleDOI
TL;DR: This review highlights trials investigating the role of As(2)O(3) in induction and consolidation for newly diagnosed APL, as well as its role in other hematologic malignancies.

245 citations


Journal ArticleDOI
TL;DR: The most common drugs to cause DIIHA are anti-microbials, which are associated with drug-dependent antibodies, and the most common drug to cause AIHA is fludarabine.

186 citations


Journal ArticleDOI
TL;DR: The genetic basis of each VWD type is explored, relating mutations identified to disease mechanism, and utility of genetic analysis within the different disease types is explored.

151 citations


Journal ArticleDOI
TL;DR: This review describes the current understanding regarding the biology of MM, and how this has led to a robust pipeline of novel therapeutic agents with the potential to overcome resistance to existing MM therapies and, therefore, further improve outcomes in patients with MM.

142 citations


Journal ArticleDOI
TL;DR: Most infants with birth weight <1.0 kg are given multiple red blood cell (RBC) transfusions within the first few weeks of life, and these are the mainstay of therapy with recombinant human erythropoietin largely unused because it fails to substantially diminish RBC transfusion needs.

124 citations


Journal ArticleDOI
TL;DR: It is demonstrated that a regimen of lenalidomide in combination with an optimal dose and schedule of dexamethasone may increase survival by allowing synergistic antiproliferative effects, without affecting immunomodulatory activity.

114 citations


Journal ArticleDOI
TL;DR: Correcting iron deficiency can improve anemia and reduce ESA requirements, and partial, but not complete, correction of anemia is associated with improved outcomes in patients with CKD.

107 citations


Journal ArticleDOI
TL;DR: The current understanding of developmental hemostasis is explained and the common pitfalls observed in clinical practice through failure to implement the principles into routine diagnostic work are described.

100 citations


Journal ArticleDOI
TL;DR: Increased survival in patients with β thalassaemia major (TM) allowed for several morbidities to manifest, and relative iron depletion may explain renal function changes attributed to chelation therapy; thus, sudden removal of iron or overchelation should be avoided.

Journal ArticleDOI
TL;DR: In this review, the in vitro differentiation potential of PSCs to the hematopoietic lineage is analysed by discussing blood cell types that can be currently obtained, limitations in derivation of adult-type HSCs and prospects for clinical application of P SCs-derived blood cells.

Journal ArticleDOI
TL;DR: A review of recent advances in understanding the epidemiology and pathogenetic mechanisms of thrombotic and bleeding complications in myeloproliferative disorders highlights recent advances.

Journal ArticleDOI
TL;DR: The survival prospects of critically ill patients with haematological malignancy are reviewed, as are the variables which might influence decisions about the limitation of life sustaining therapies (LLST).

Journal ArticleDOI
TL;DR: Invasive fungal infections are significant causes of morbidity and mortality in patients with hematological malignancies as discussed by the authors, patients with acute myeloid leukemia and those who have undergone allogeneic hematopoietic stem cell transplantation are at especially high risk.

Journal ArticleDOI
TL;DR: Recurrent VTE risk stratification where unprovoked VTE patients are categorised as low or high risk for recurrent VTE and clinical decision making is less ambiguous and ultimately will likely lead to better outcomes.

Journal ArticleDOI
TL;DR: A review of emerging new treatment modalities that are currently under investigation for patients with relapsed classical HL will focus on targeting oncogenic signaling pathways.

Journal ArticleDOI
TL;DR: It is found that defects in a limited number of pathways explain the majority of gene associations with IgE, and the mechanisms of control over IgE production and the manner in which they can be circumvented are demonstrated.

Journal ArticleDOI
TL;DR: There remains much research to be done in this field, in the meantime clinicians need to carefully consider the evidence available to them and manage each individual patient accordingly.

Journal ArticleDOI
TL;DR: The use of molecular profiling to suggest particular therapies is currently appropriate only in defining the treatment of the minority of patients with 17p deletions or p53 mutations and in all other circumstances remains a clinical trial question.

Journal ArticleDOI
TL;DR: Although these alternative therapies may overcome imatinib resistance, long-term remission or cure from CML is likely to require development of novel interventions that effectively eliminate CML stem cells (Ph+HSC).

Journal ArticleDOI
TL;DR: Early clinical results are promising, indicating that the novel lenalidomide-based drug combinations are effective and generally well tolerated in patients with MM; future research will continue to evaluate these novel combinations and help to identify the optimal setting in which they may provide the greatest impact on the disease course.

Journal ArticleDOI
TL;DR: The purpose of this review is to focus on these novel agents and the various treatment approaches that are currently being evaluated in non-Hodgkin lymphoma.

Journal ArticleDOI
TL;DR: Assays have confirmed that the majority of TTP cases are idiopathic and are associated with inhibitors and or IgG antibodies to ADAMTS 13, and further investigation into the antibodies produced in TTP and other aspects of immune dysfunction, such as T cells will further the understanding of this devastating disorder.

Journal ArticleDOI
TL;DR: The key observations from recent completed and ongoing studies that determined the effect of these novel therapies both in the setting of newly diagnosed myeloma and for relapsed disease are summarized.

Journal ArticleDOI
TL;DR: The identification of JAK2 and MPL mutations, and more recently TET2, CBL and ASXL-1 mutations in these disorders provide a basis for increased understanding of disease biology and mechanisms behind progression.

Journal ArticleDOI
TL;DR: Early results from phase III trials indicate that in patients with newly diagnosed MM, continuous lenalidomide therapy is well tolerated and associated with significant improvements in PFS, offering a new treatment option for patients with MM - although no OS benefit has yet been seen in this setting.


Journal ArticleDOI
TL;DR: The author received editorial support from Excerpta Medica in the preparation of this manuscript, and this work was funded by Celgene Corporation.

Journal Article
TL;DR: Menne TF, Goyenechea B, Sanchez-Puig N, Wong, C. M., Ancliff, P. J., Brost, R. L., Costanzo, M., Boone, C., Warren, A. as discussed by the authors corrected the omission of a reference in their discussion of the genetic interaction between Sdo1 and Tif6 in yeast.