Autophagy: A necessary event during erythropoiesis
Autophagy is a well-known cellular process involved in many physiological and pathological processes. During erythropoiesis, autophagy plays an important role participating in the clearance of unnecessary organelles such as ribosomes and mitochondria (mitophagy) allowing the correct formation of mature red blood cells. The dysfunction of autophagy proteins hamper the correct erythroid maturation, leading to anemia, the release of immature cells from the bone marrow and other hematological abnormalities. (Source: Blood Reviews)
Source: Blood Reviews - April 22, 2017 Category: Hematology Authors: Rub én Grosso, Claudio M. Fader, María I. Colombo Tags: Review Source Type: research
Should studies on Glanzmann thrombasthenia not be telling us more about cardiovascular disease and other major illnesses?
Glanzmann thrombasthenia (GT) is a rare inherited bleeding disorder caused by loss of αIIbβ3 integrin function in platelets. Most genetic variants of β3 also affect the widely expressed αvβ3 integrin. With brief mention of mouse models, I now look at the consequences of disease-causing ITGA2B and ITGB3 mutations on the non-hemostatic functions of platelets and other cells. Repor ts of arterial thrombosis in GT patients are rare, but other aspects of cardiovascular disease do occur including deep vein thrombosis and congenital heart defects. (Source: Blood Reviews)
Source: Blood Reviews - April 4, 2017 Category: Hematology Authors: Alan T. Nurden Tags: Review Source Type: research
The bone marrow microenvironment – Home of the leukemic blasts
Acute Myeloid Leukaemia (AML) is a genetically, biologically and clinically heterogeneous set of diseases, which are characterised by an increased growth of abnormal myeloid progenitor cells within the bone marrow (BM). Ex-vivo AML exhibits a high level of spontaneous apoptosis. Furthermore, relapse for patients achieving remission occurs from minimal residual disease harboured within the BM microenvironment. Taken together, these observations illustrate the importance of the BM microenvironment in sustaining AML. (Source: Blood Reviews)
Source: Blood Reviews - March 11, 2017 Category: Hematology Authors: Manar S Shafat, Bruno Gnaneswaran, Kristian M Bowles, Stuart A Rushworth Tags: Review Source Type: research
Light chain amyloidosis: Where are the light chains from and how they play their pathogenic role?
Amyloid light-chain (AL) amyloidosis is a plasma-cell dyscrasia, as well as the most common type of systematic amyloidosis. Pathogenic plasma cells that have distinct cytogenetic and molecular properties secrete an excess amount of amyloidogenic light chains. Assisted by post-translational modifications, matrix components, and other environmental factors, these light chains undergo a conformational change that triggers the formation of amyloid fibrils that overrides the extracellular protein quality control system. (Source: Blood Reviews)
Source: Blood Reviews - March 7, 2017 Category: Hematology Authors: Chunlan Zhang, Xufei Huang, Jian Li Tags: Review Source Type: research
Does percutaneous coronary stent implantation increase platelet reactivity?
High platelet reactivity (PR) values on treatment with clopidogrel are associated with an increased rate of thrombotic events after a percutaneous coronary intervention (PCI). However, we do not know the optimal timing of the performance of the PR measurements. Platelets might be activated during a PCI, which means that the timing of PR measurements, before or after PCI, could influence the outcome. In turn, this could lead to misinterpretation of the patient's response to antiplatelet therapy and a less accurate prediction of the patient's risk of thrombotic events during follow-up. (Source: Blood Reviews)
Source: Blood Reviews - March 7, 2017 Category: Hematology Authors: P.W.A. Janssen, E.A. Mol, S.M.C. Geene, E. Barbato, J.M. ten Berg Tags: Review Source Type: research
p53 dysregulation in B-cell malignancies: More than a single gene in the pathway to hell
We describe here the p53 landscape in B-cell malignancies, from B-Acute Lymphoblastic Leukemia to Plasma Cell Leukemia, by analyzing incidence of gain or loss of function of actors both upstream and within the p53 pathway, namely MYC, RAS, ARF, MDM2, ATM and TP53. Abnormalities are not equally distributed and their incidence is highly variable among malignancies. Deletion and mutation, usually associated, of ATM or TP53 are frequent in Diffuse Large B-Cell Lymphoma and Mantle Cell Lymphoma. (Source: Blood Reviews)
Source: Blood Reviews - March 3, 2017 Category: Hematology Authors: B Tessoulin, M Eveillard, A Lok, D Chiron, P Moreau, M Amiot, A Moreau-Aubry, S Le Gouill, C Pellat-Deceunynck Tags: Review Source Type: research
How gene polymorphisms can influence clinical response and toxicity following R-CHOP therapy in patients with Diffuse Large B Cell Lymphoma
The treatment of diffuse large B cell lymphoma (DLBCL) is generally based on multidrug chemotherapy, for instance the therapy with rituximab together with cyclophosphamide, vincristine, doxorubicin, and prednisone (R-CHOP). A significant proportion of DLBCL patients benefit from rituximab-based chemoimmunotherapy. However, among patients with DLBCL toxic effects due to therapy treatment are still very frequent, as well as inter-individual differences in the outcomes of patients even having similar stage, histological grade and histopathological type of the tumor. (Source: Blood Reviews)
Source: Blood Reviews - February 23, 2017 Category: Hematology Authors: Angela Falduto, Francesco Cimino, Antonio Speciale, Caterina Musolino, Sebastiano Gangemi, Antonella Saija, Alessandro Allegra Tags: Review Source Type: research
New insights into iron deficiency and iron deficiency anemia
Recent advances in iron metabolism have stimulated new interest in iron deficiency (ID) and its anemia (IDA), common conditions worldwide. Absolute ID/IDA, i.e. the decrease of total body iron, is easily diagnosed based on decreased levels of serum ferritin and transferrin saturation. Relative lack of iron in specific organs/tissues, and IDA in the context of inflammatory disorders, are diagnosed based on arbitrary cut offs of ferritin and transferrin saturation and/or marker combination (as the soluble transferrin receptor/ferritin index) in an appropriate clinical context. (Source: Blood Reviews)
Source: Blood Reviews - February 11, 2017 Category: Hematology Authors: Clara Camaschella Tags: Review Source Type: research
Complementopathies
The complement system is an essential part of the innate immune system that requires careful regulation to ensure responses are appropriately directed against harmful pathogens, while preventing collateral damage to normal host cells and tissues. While deficiency in some components of the complement pathway is associated with increased susceptibility to certain infections, it has also become clear that inappropriate activation of complement is an important contributor to human disease. A number of hematologic disorders are driven by complement, and these disorders may be termed “complementopathies”. (Source: Blood Reviews)
Source: Blood Reviews - February 4, 2017 Category: Hematology Authors: Andrea C. Baines, Robert A. Brodsky Tags: Review Source Type: research
Old and new oral anticoagulants: Food, herbal medicines and drug interactions
The most commonly prescribed oral anticoagulants worldwide are the vitamin K antagonists (VKAs) such as warfarin. Factors affecting the pharmacokinetics of VKAs are important because deviations from their narrow therapeutic window can result in bleedings due to over-anticoagulation or thrombosis because of under-anticoagulation. In addition to pharmacodynamic interactions (e.g., augmented bleeding risk for concomitant use of NSAIDs), interactions with drugs, foods, herbs, and over-the-counter medications may affect the risk/benefit ratio of VKAs. (Source: Blood Reviews)
Source: Blood Reviews - February 3, 2017 Category: Hematology Authors: Alessandro Di Minno, Beatrice Frigerio, Gaia Spadarella, Alessio Ravani, Daniela Sansaro, Mauro Amato, Joseph P. Kitzmiller, Mauro Pepi, Elena Tremoli, Damiano Baldassarre Tags: Review Source Type: research
Advances in oral anticoagulation therapy – What's in the pipeline?
Approximately 900,000 people are affected by some sort of venous thromboembolic (VTE) event every year in the United States. VTE diagnosis used to mean treatment with medications that required routine lab monitoring for safety and efficacy. Activated factor X (FXa) inhibition has emerged as a convenient pathway for management of VTE and currently three FXa inhibitors are available for anticoagulation management - rivaroxaban, apixaban, and edoxaban. Continued development of medications utilizing this pathway may offer advantages via novel pharmacokinetic or pharmacodynamic properties that may minimize the adverse effects a...
Source: Blood Reviews - February 2, 2017 Category: Hematology Authors: P.S.S. Rao, T. Burkart Tags: Review Source Type: research
The clinical impact of cytomegalovirus infection following allogeneic hematopoietic cell transplantation: Why the quest for meaningful prophylaxis still matters
Latent infection with human cytomegalovirus (CMV) is common. Functional immunity effectively contains such latent infections; however, CMV reactivation may cause significant complications in patients undergoing allogeneic hematopoietic cell transplantation (alloHCT). In spite of the universal implementation of post-transplant screening for CMV viremia and the institution of pre-emptive antiviral management, CMV disease still occurs in a small portion of patients. Moreover, interactions between CMV and the immune system have significant implications for the incidence of graft-versus-host disease, the recurrence of malignanc...
Source: Blood Reviews - February 1, 2017 Category: Hematology Authors: Shawna T. Chan, Aaron C. Logan Tags: Review Source Type: research
Bone marrow evaluation for diagnosis and monitoring of acute myeloid leukemia
The diagnosis of acute myeloid leukemia (AML) can be made based on peripheral blood or bone marrow blasts. In this review, we will discuss the role of bone marrow evaluation and peripheral blood monitoring in the diagnosis, management, and follow up of AML patients. For patients with circulating blasts, it is reasonable to perform the necessary studies needed for diagnosis and risk stratification, including multiparametric flow cytometry, cytogenetics, and molecular analysis, on a peripheral blood specimen. (Source: Blood Reviews)
Source: Blood Reviews - February 1, 2017 Category: Hematology Authors: Mary-Elizabeth Percival, Catherine Lai, Elihu Estey, Christopher S. Hourigan Tags: Review Source Type: research
The role of splenectomy in autoimmune hematological disorders: Outdated or still worth considering?
We discuss the role of splenectomy in the autoimmune hematological disorders immune thrombocytopenia (ITP), autoimmune hemolytic anemia (AIHA) and thrombotic thrombocytopenic purpura (TTP). Management of these disorders has dramatically changed the past decade as increasing knowledge of the immunopathogenesis has led to the introduction of new therapies. Until 10years ago, splenectomy was the established second-line treatment for ITP, considered when corticosteroids failed to induce a sustained response. (Source: Blood Reviews)
Source: Blood Reviews - January 3, 2017 Category: Hematology Authors: Judith Sys, Drew Provan, Alexander Schauwvlieghe, Steven Vanderschueren, Daan Dierickx Tags: Review Source Type: research
How do you decide on hormone replacement therapy in women with risk of venous thromboembolism?
Women are increasingly encouraged to participate in making decisions about hormone replacement therapy (HRT). In postmenopausal women with severe vasomotor symptoms, HRT can significantly improve the quality of life. However, the use of HRT may also increase the risk of venous thromboembolism (VTE), the risk which depends of both treatment-related and patient-related factors. This review summarizes some important points about the selection of the safest hormonal replacement modality in women with a history of VTE and management of VTE risks in postmenopausal women wishing to take HRT. (Source: Blood Reviews)
Source: Blood Reviews - December 13, 2016 Category: Hematology Authors: Danijela Lekovic, Predrag Miljic, Aleksandar Dmitrovic, Jecko Thachil Tags: Review Source Type: research
