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Current Opinion in Hematology

Priya Sakthivel, Dunja Bruder
PURPOSE OF REVIEW: The formation of noncaseating granuloma is a hallmark of pulmonary sarcoidosis. This review summarizes recent progress made to explain the cellular dynamics within the granuloma structure that may considerably differ between the two clinically distinct variants, that is, acute and chronic sarcoidosis. RECENT FINDINGS: Compelling evidence exists that in acute but not chronic sarcoidosis CD4 T lymphocytes specifically recognizing the auto-antigen vimentin on human leukocyte antigen-DR3 molecules accumulate in sarcoid granuloma...
October 15, 2016: Current Opinion in Hematology
Cédric Seignez, Mia Phillipson
PURPOSE OF REVIEW: This review describes the mechanisms by which neutrophils contribute to angiogenesis in hypoxic tissues during different conditions and diseases (e.g., menstrual cycle, wound healing, ischemic diseases, cancers), with particular focus on the recently described proangiogenic neutrophil subpopulation. RECENT FINDINGS: The importance of neutrophils in initiation of angiogenesis has been described during the past decade, and is believed to occur through release of the well-known proangiogenic factors Bv8, vascular endothelial growth factor A, and matrix metalloproteinase 9...
October 15, 2016: Current Opinion in Hematology
Yini Wang, Zhao Wang
PURPOSE OF REVIEW: Hemophagocytic lymphohistiocytosis (HLH) is a condition of uncontrolled immune activation with a high mortality rate. The recommended therapeutic guideline for HLH was published by the Histiocyte Society in 1994 and revised in 2004, which greatly improved the survival in patients with HLH. However, HLH is still a refractory disease for which the search for novel treatments continues. This article overviewed recent advances in treatment of HLH. RECENT FINDINGS: Current practices in treatment extend from chemo-immunotherapy to some new cytokine-targeting biologicals, which are more effective to eliminate pathologically activated T cells and resist exaggerated cytokine storm...
October 15, 2016: Current Opinion in Hematology
Siobhan O Burns, Alex Zarafov, Adrian J Thrasher
PURPOSE OF REVIEW: Primary immunodeficiencies (PIDs) are inherited conditions where components of the immune system are missing or dysfunctional. Over 300 genes have been causally linked to monogenic forms of PID, including a number that regulate the actin cytoskeleton. The majority of cytoskeletal defects disrupt assembly and disassembly of filamentous actin in multiple immune cell lineages impacting functions such as cell migration and adhesion, pathogen uptake, intercellular communication, intracellular signalling, and cell division...
October 5, 2016: Current Opinion in Hematology
Anika Stadtmann, Alexander Zarbock
PURPOSE OF REVIEW: Since the discovery of the lack of kindlin-3 expression as the reason for the immunopathology leukocyte adhesion deficiency III syndrome, the role of kindlin-3 in inflammatory processes was investigated in a numerous studies. This review gives an overview about recent findings regarding the role of kindlin-3 in neutrophil activation and recruitment. RECENT FINDINGS: Kindlin-3, together with talin-1, contributes essentially to the activation of β2-integrins in neutrophils...
October 5, 2016: Current Opinion in Hematology
Ralf Willebrand, David Voehringer
PURPOSE OF REVIEW: Eosinophils are a subset of granulocytes generally associated with type 2 immune responses. They can contribute to protection against helminths but also mediate pro-inflammatory functions during allergic immune responses. Only recently, eosinophils were also found to exert many other functions such as regulation of glucose and fat metabolism, thermogenesis, survival of plasma cells, and antitumor activity. The mechanisms that control eosinophil development and survival are only partially understood...
September 26, 2016: Current Opinion in Hematology
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No abstract text is available yet for this article.
November 2016: Current Opinion in Hematology
Andrea Bacigalupo
No abstract text is available yet for this article.
November 2016: Current Opinion in Hematology
Robert Chiesa, Robert F Wynn, Paul Veys
PURPOSE OF REVIEW: This review summarizes the main results of haematopoietic stem cell transplantation (HSCT) in selected inborn errors of metabolism (IEMs). RECENT FINDINGS: Early diagnosis and immediate referral to an IEM specialist is of paramount importance to improve clinical outcome: patients who are transplanted early or in their presymptomatic phase generally achieve better correction of their somatic symptoms and neurocognitive development. Long-term outcome in children with Hurler syndrome is influenced by age at HSCT, baseline clinical status and post-HSCT enzyme levels...
November 2016: Current Opinion in Hematology
Shaimaa Elmahadi, Hideki Muramatsu, Seiji Kojima
PURPOSE OF REVIEW: Dyskeratosis congenita is an inherited bone marrow failure syndrome caused by defects in telomere maintenance. Hematopoietic stem cell transplantation (HSCT) is the only curative treatment for bone marrow failure because of dyskeratosis congenita. The present review summarizes the literature with respect to the diagnosis and treatment of patients with dyskeratosis congenita who received HSCT, and discusses the recent progress in the management of dyskeratosis congenita...
November 2016: Current Opinion in Hematology
George E Georges, Rainer Storb
PURPOSE OF REVIEW: There has been a steady improvement in outcomes with allogeneic bone marrow transplantation (BMT) for severe aplastic anemia (SAA), because of progress in optimization of the conditioning regimens, donor hematopoietic cell source, and supportive care. Here, we review recently published data that highlight the improvements and current issues in the treatment of SAA. RECENT FINDINGS: Approximately one-third of aplastic anemia patients treated with immune suppressive therapy (IST) have acquired mutations in myeloid cancer candidate genes...
November 2016: Current Opinion in Hematology
Philip C Spinella, Andrew P Cap
PURPOSE OF REVIEW: We present data comparing whole blood with blood components and summarize the data that support increased availability of whole blood for patients with life-threatening bleeding. RECENT FINDINGS: Recent data indicate that whole-blood transfusion is associated with improved or comparable survival compared with resuscitation with blood components. These data complement randomized controlled trials indicating that platelet-containing blood products stored at 4 °C have superior hemostatic function, compared with platelet-containing blood products at 22 °C...
November 2016: Current Opinion in Hematology
Andrew E Levin, Peter J Krause
PURPOSE OF REVIEW: This review summarizes the current status of blood screening to prevent transfusion-transmitted babesiosis (TTB). RECENT FINDINGS: Babesia microti has recently been determined to be the most common transfusion-transmitted pathogen in the United States. Patients who acquire TTB often experience severe illness with an associated mortality rate of about 20%. Recent studies have demonstrated that laboratory screening using B. microti antibody and/or PCR assays can effectively identify infectious blood donors and that this approach may offer a cost- effective means of intervention...
November 2016: Current Opinion in Hematology
Franco Locatelli, Pietro Merli, Luisa Strocchio
PURPOSE OF REVIEW: Lack of a human leukocyte antigen (HLA)-matched family donor is a major obstacle limiting the use of allogeneic hematopoietic stem cell transplantation (HSCT) to cure thalassemia major. Use of alternative donors, if found well tolerated enough, may be a viable option for transplantation in this setting, allowing to expand the number of patients treatable by HSCT. This review will provide an overview of alternative HSCT approaches in thalassemia major. RECENT FINDINGS: With the introduction of high-resolution molecular HLA-typing techniques, the outcome of matched unrelated donor HSCT recipients has become comparable with that of patients given matched family donor HSCT...
November 2016: Current Opinion in Hematology
Richard O Francis
PURPOSE OF REVIEW: Increased use of platelets and the brief shelf life allowed by storage at 22 °C are promoting exploration of new methods to store platelets. As storage may affect the hemostatic efficacy of platelets, it is very important to assess their in-vivo hemostatic efficacy. The potential usefulness of preclinical models in evaluating methods for storing platelets will be discussed. RECENT FINDINGS: The use of new technologies to examine the in-vitro characteristics of stored platelet products continues to evolve...
November 2016: Current Opinion in Hematology
Surapol Issaragrisil, Chutima Kunacheewa
PURPOSE OF REVIEW: Bone marrow transplantation is the only curative treatment for severe thalassemia. Since its successful first report in 1981, more than 4000 patients with this disease worldwide underwent bone marrow transplantation. The purpose of this review is to update the most recent reports of matched sibling donor hematopoietic stem cell transplantation in thalassemia. RECENT FINDINGS: Advanced and improved transplant techniques result in the improved outcomes in those transplants from a matched sibling donor with transplant-related mortality less than 5%...
November 2016: Current Opinion in Hematology
Huy P Pham, Joseph Schwartz
PURPOSE OF REVIEW: Therapeutic apheresis can be used to treat many diseases. The American Society for Apheresis (ASFA) publishes Guidelines on the use of therapeutic apheresis every 3 years with the goal of providing the best available evidence for apheresis practice as well as clinical expertise. The 2016 (7th ed.) ASFA Guidelines contain 87 diseases (up from 78 in the 6th ed.) and 179 indications. This review outlines three new therapeutic apheresis indications for hematological disorders...
November 2016: Current Opinion in Hematology
Akshay Shah, Stuart McKechnie, Susan J Brunskill, Simon J Stanworth
PURPOSE OF REVIEW: Red blood cell transfusion is a common treatment for anaemia worldwide, but concerns continue to be raised about adverse effects of cellular blood components, which are biological products. One hypothesis for the adverse effects associated with blood transfusion is the harmful effects of storage on red cells that have been demonstrated in laboratory and animal studies. Over the past few years, a number of more significant randomized controlled trials comparing 'fresh' versus 'older' blood have been published in an attempt to address the clinical consequences of storage age, with two further large trials ongoing...
November 2016: Current Opinion in Hematology
Tara M Robinson, Ephraim J Fuchs
PURPOSE OF REVIEW: As the safety and availability of allogeneic hematopoietic stem cell transplantation (HSCT) have improved, this procedure is becoming a viable option for nonmalignant conditions such as sickle cell disease (SCD). There are very few treatment options available for SCD, and even with optimal care SCD patients still suffer from a myriad of comorbidities to multiple organ systems and have a decreased life span. In this review, we will summarize results from trials of HSCT for children or adults with SCD using a variety of graft sources as well as conditioning and graft-versus-host disease prophylaxis regimens, and discuss the unique challenges that arise in these patients...
November 2016: Current Opinion in Hematology
Jeanne E Hendrickson, Stephanie C Eisenbarth, Christopher A Tormey
PURPOSE OF REVIEW: To summarize recent discoveries from clinical studies and animal models that contribute to understanding the alloimmune response to non-ABO blood group antigens. RECENT FINDINGS: Several studies have confirmed high rates of alloimmunization among patients requiring chronic red blood cell (RBC) transfusion. Moreover, 'triggers' for alloantibody development in the transfusion setting have been identified, with a number of investigations linking recipient inflammation to a higher likelihood of alloimmunization...
November 2016: Current Opinion in Hematology
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