Higher-risk myelodysplastic syndromes with del(5q): does the del(5q) matter?


:Introduction: Myelodysplastic Syndrome (MDS) represents a group of cancers characterized by abnormal blood cell formation and maturation, leading to various degrees of cytopenias and potential transformation to acute myeloid leukemia. Deletion of the long arm of chromosome 5 (del(5q)) is the most common clonal chromosomal anomaly in MDS, yet the population in this disease subtype is quite heterogeneous. This manuscript analyzes literature on high-risk MDS with del(5q) abnormalities.Areas covered: The paper will review outcomes with lenalidomide among high-risk MDS patients with del(5q). It will discuss the implications of harboring TP53 gene mutations, and share the data for allogeneic hematopoietic stem cell transplantations in this setting. Finally, the report evaluates the risk of disease progression in these patients.Expert commentary: Improved characterization of MDS has enhanced our understanding of patients with anomalies involving del(5q). Emerging literature is exploring combination therapy beyond lenalidomide, and next-generation sequencing may identify secondary mutations that could be an additional avenue for treatment.


Expert Rev Hematol


Gorshein E,Weber UM,Gore S




Has Abstract


2020-03-01 00:00:00












  • Akt signaling in platelets and thrombosis.

    abstract::Akt is a Ser-Thr kinase with pleiotropic effects on cell survival, growth and metabolism. Recent evidence from gene-deletion studies in mice, and analysis of human platelets treated with Akt inhibitors, suggest that Akt regulates platelet activation, with potential consequences for thrombosis. Akt activation is regula...

    journal_title:Expert review of hematology

    pub_type: 杂志文章


    authors: Woulfe DS

    更新日期:2010-02-01 00:00:00

  • Primary mediastinal lymphoma: diagnosis and treatment options.

    abstract::Primary mediastinal large B-cell lymphoma (PMBCL) is a unique B-cell lymphoma variant that arises from a putative thymic medulla B cell. It constitutes 2-4% of non-Hodgkin lymphomas and occurs most frequently in young females. PMBCL is characterized by a diffuse proliferation of medium-to-large B cells associated with...

    journal_title:Expert review of hematology

    pub_type: 杂志文章,评审


    authors: Martelli M,Di Rocco A,Russo E,Perrone S,Foà R

    更新日期:2015-04-01 00:00:00

  • Small molecule inhibitors in acute myeloid leukemia: from the bench to the clinic.

    abstract::Many patients with acute myeloid leukemia will eventually develop refractory or relapsed disease. In the absence of standard therapy for this population, there is currently an urgent unmet need for novel therapeutic agents. Targeted therapy with small molecule inhibitors represents a new therapeutic intervention that ...

    journal_title:Expert review of hematology

    pub_type: 杂志文章,评审


    authors: Al-Hussaini M,DiPersio JF

    更新日期:2014-08-01 00:00:00

  • Immunomodulatory drug- and proteasome inhibitor-backbone regimens in the treatment of relapsed multiple myeloma: an evidence-based review.

    abstract:INTRODUCTION:Recent advances and drug approvals in the last decade have substantially changed the landscape of relapsed and refractory multiple myeloma (RRMM), which not only improved outcomes for patients but also increased complexity of treatment decisions. The approvals are based on randomized studies of novel agent...

    journal_title:Expert review of hematology

    pub_type: 杂志文章


    authors: Sanchez L,Barley K,Richter J,Franz J,Cho HJ,Jagannath S,Madduri D,Parekh S,Richard S,Chari A

    更新日期:2020-09-01 00:00:00

  • Current and future prospects for hemophilia gene therapy.

    abstract::Here we review the recent literature on Hemophilia gene transfer/therapy. Gene therapy is one of several new technologies being developed as a treatment for bleeding disorders. We will discuss current and pending clinical efforts and attempt to relate how the field is trending. In doing so, we will focus on the use of...

    journal_title:Expert review of hematology

    pub_type: 杂志文章,评审


    authors: Ward P,Walsh CE

    更新日期:2016-07-01 00:00:00

  • The management and treatment of acute leukemias in the elderly population.

    abstract:INTRODUCTION:Treatment of elderly patients with acute leukemia is challenging. Older age is associated with increased risk of treatment-related toxicity. Currently, no consensus exists regarding optimal therapy in this patient population. Areas covered: The following review is a comprehensive summary of various therape...

    journal_title:Expert review of hematology

    pub_type: 杂志文章,评审


    authors: Thomas X

    更新日期:2017-11-01 00:00:00

  • Position paper on management of iron deficiency in adult cancer patients.

    abstract:INTRODUCTION:Disorders of iron metabolism are commonly seen in onco-hematological clinical practice. Iron-deficiency anemia and cancer-associated anemia are usually treated with supportive therapies. Optimal management of these conditions are discussed in this perspective paper. Areas covered: A position paper discussi...

    journal_title:Expert review of hematology

    pub_type: 杂志文章


    authors: Barni S,Gascòn P,Petrelli F,García-Erce JA,Pedrazzoli P,Rosti G,Giordano G,Mafodda A,Múñoz M

    更新日期:2017-08-01 00:00:00

  • Mitochondria and Iron: current questions.

    abstract:INTRODUCTION:Mitochondria are cellular organelles that perform numerous bioenergetic, biosynthetic, and regulatory functions and play a central role in iron metabolism. Extracellular iron is taken up by cells and transported to the mitochondria, where it is utilized for synthesis of cofactors essential to the function ...

    journal_title:Expert review of hematology

    pub_type: 杂志文章,评审


    authors: Paul BT,Manz DH,Torti FM,Torti SV

    更新日期:2017-01-01 00:00:00

  • Analysis of Hb levels and degree of anemia in relation to genotype in 615 patients with hemoglobin H disease.

    abstract:OBJECTIVES:We analyzed hemoglobin (Hb) levels and degree of anemia in relation to genotype in patients with hemoglobin H (Hb H) disease, thereby providing a scientific basis for the prevention and treatment of Hb H disease in the Guangxi region of China. METHODS:Hb analysis was conducted in 615 patients using high per...

    journal_title:Expert review of hematology

    pub_type: 杂志文章


    authors: Luo S,Chen X,Chen L,Zhong Q,Wang Q,Xu Z,Huang J,Yan T,Tang N

    更新日期:2020-09-01 00:00:00

  • Diagnosis and management of catastrophic antiphospholipid syndrome.

    abstract:INTRODUCTION:Catastrophic antiphospholipid syndrome (CAPS) is a rare, life-threatening disease. In 1992, Asherson defined it as a widespread coagulopathy related to the antiphospholipid antibodies (aPL). CAPS requires rapid diagnosis and prompt initiation of treatment. Areas covered: This paper discusses all aspects of...

    journal_title:Expert review of hematology

    pub_type: 杂志文章,评审


    authors: Carmi O,Berla M,Shoenfeld Y,Levy Y

    更新日期:2017-04-01 00:00:00

  • Immunotherapy for the treatment of Hodgkin lymphoma.

    abstract:INTRODUCTION:Most patients with Hodgkin lymphoma (HL) enjoy durable remissions following front-line treatment but 30% of patients are refractory or relapse after first line therapy. Salvage chemotherapy followed by autologous stem cell transplantation (ASCT) can cure an additional 50-55% of relapsing patients but new t...

    journal_title:Expert review of hematology

    pub_type: 杂志文章,评审


    authors: Donato EM,Fernández-Zarzoso M,De La Rubia J

    更新日期:2017-05-01 00:00:00

  • Lestaurtinib: a multi-targeted FLT3 inhibitor.

    abstract::Internal tandem duplication mutations of FMS-like tyrosine kinase-3 (FLT3) have been associated with poor outcomes in acute myelogenous leukemia. Over the course of the last several years, multiple agents have been developed and studied as potential inhibitors of FLT3 with the hope of providing clinical benefit for th...

    journal_title:Expert review of hematology

    pub_type: 杂志文章,评审


    authors: Fathi AT,Levis M

    更新日期:2009-02-01 00:00:00

  • Advances in fertility preservation of female patients with hematological malignancies.

    abstract:INTRODUCTION:The most common forms of hematological malignancies that occur in female reproductive years are lymphoma and leukemia. Areas covered: Several aggressive gonadotoxic regimens such as alkylating chemotherapy and total body irradiation are used frequently in treatment of lymphoma and leukemia leading to subse...

    journal_title:Expert review of hematology

    pub_type: 杂志文章


    authors: Salama M,Isachenko V,Isachenko E,Rahimi G,Mallmann P

    更新日期:2017-11-01 00:00:00

  • Combining gene therapy and fetal hemoglobin induction for treatment of β-thalassemia.

    abstract::β-thalassemias are caused by nearly 300 mutations of the β-globin gene, leading to a low or absent production of adult hemoglobin (HbA). Two major therapeutic approaches have recently been proposed: gene therapy and induction of fetal hemoglobin (HbF) with the objective of achieving clinically relevant levels of Hbs. ...

    journal_title:Expert review of hematology

    pub_type: 杂志文章


    authors: Breda L,Rivella S,Zuccato C,Gambari R

    更新日期:2013-06-01 00:00:00

  • Large granular lymphocytic leukemia. Current diagnostic and therapeutic approaches and novel treatment options.

    abstract:INTRODUCTION:Large granular lymphocytic leukemia (LGLL) is a low grade lymphoproliferative disorder characterized by the clonal proliferation of large granular lymphocytes (LGL) and recognised by the WHO. The diagnosis and management of these patients is challenging due to the limited information from prospective studi...

    journal_title:Expert review of hematology

    pub_type: 杂志文章,评审


    authors: Matutes E

    更新日期:2017-03-01 00:00:00

  • A spotlight on the management of complications associated with myeloproliferative neoplasms: a clinician's perspective.

    abstract:INTRODUCTION:Myeloproliferative neoplasms (MPNs) are associated with a variety of symptoms and signs which cause major morbidity for the patients. The disorders are associated with increased incidence of thromboembolic and hemorrhagic events which can lead to complications and shortened life expectancy. Areas covered: ...

    journal_title:Expert review of hematology

    pub_type: 杂志文章,评审


    authors: Greenfield G,McMullin MF

    更新日期:2018-01-01 00:00:00

  • Prognostic tools in follicular lymphomas.

    abstract::Despite significant improvements in treatment modalities over the 10 years, the clinical course of patients with follicular lymphoma (FL) remains heterogeneous. Thus, prognostic indexes are still required to direct treatment choices and for the design of clinical trials. Investigators have conducted a variety of studi...

    journal_title:Expert review of hematology

    pub_type: 杂志文章,评审


    authors: Luminari S,Cox MC,Montanini A,Federico M

    更新日期:2009-10-01 00:00:00

  • Emerging therapeutic targets for the treatment of human acute myeloid leukemia (part 1) - gene transcription, cell cycle regulation, metabolism and intercellular communication.

    abstract::Human acute myeloid leukemia is a heterogeneous disease and the effect of therapeutic targeting of specific molecular mechanisms will probably vary between patient subsets. Cell cycle regulators are among the emerging targets (e.g., aurora and polo-like kinases, cyclin-dependent kinases). Inhibition of communication b...

    journal_title:Expert review of hematology

    pub_type: 杂志文章,评审


    authors: Reikvam H,Hauge M,Brenner AK,Hatfield KJ,Bruserud Ø

    更新日期:2015-06-01 00:00:00

  • The multiple myeloma treatment landscape: international guideline recommendations and clinical practice in Europe.

    abstract:INTRODUCTION:Guidelines provide recommendations on the management of multiple myeloma (MM), but there are no standard algorithms for the choice and sequencing of treatments. As a result, there is widespread variation in the interpretation and implementation of these guidelines. Areas covered: This review will cover: th...

    journal_title:Expert review of hematology

    pub_type: 杂志文章,评审


    authors: Cavo M,Terpos E,Bargay J,Einsele H,Cavet J,Greil R,de Wit E

    更新日期:2018-03-01 00:00:00

  • Polyphosphates: a link between platelet activation, intrinsic coagulation and inflammation?

    abstract::Early evidence has already shown that activated platelets play a role in the intrinsic pathway of coagulation, but the mechanism remained unclear. The finding that the dense granules of platelets contain polyphosphate (polyP), which has procoagulant effects, resolved this missing link between activated platelets and c...

    journal_title:Expert review of hematology

    pub_type: 评论,杂志文章


    authors: van der Meijden PE,Heemskerk JW

    更新日期:2010-06-01 00:00:00

  • Bronchial myeloid sarcoma with concurrent Aspergillus fumigatus infection in a patient presenting with hemoptysis.

    abstract::Myeloid sarcoma (MS) is an extramedullary myeloid neoplasm characterized by proliferation of myeloblasts which can occur in any organ or site. Bronchial and pulmonary involvement, however, is uncommon. We describe a case of bronchial MS in an 81-year-old female with a history of high-grade myelodysplastic syndrome; sh...

    journal_title:Expert review of hematology

    pub_type: 杂志文章


    authors: Vennepureddy A,Valecha G,Murukutla S,Hussein S,Terjanian T

    更新日期:2015-08-01 00:00:00

  • How ruxolitinib modified the outcome in myelofibrosis: focus on overall survival, allele burden reduction and fibrosis changes.

    abstract:INTRODUCTION:Ruxolitinib is a potent and selective JAK1/JAK2 inhibitor that has shown superiority as compared to available conventional chemotherapies, in terms of reduction in splenomegaly and improvement of symptoms and quality of life. Areas covered: Data published about overall survival in the major randomized spon...

    journal_title:Expert review of hematology

    pub_type: 杂志文章,评审


    authors: Massaro F,Molica M,Breccia M

    更新日期:2017-02-01 00:00:00

  • An update on pathogenesis and diagnosis of thrombotic thrombocytopenic purpura.

    abstract::Introduction: Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, consumption thrombocytopenia, and organ injury. TTP pathophysiology is based on a severe ADAMTS13 deficiency, the specific von Willebrand factor (VWF)-cl...

    journal_title:Expert review of hematology

    pub_type: 杂志文章,评审


    authors: Joly BS,Coppo P,Veyradier A

    更新日期:2019-06-01 00:00:00

  • Prolonged and increased postoperative risk of venous thromboembolism: rationale for even more 'extended' prophylaxis?

    abstract::It is well known that the risk of venous thromboembolism is increased after surgery; however, specific data on how long this risk lasts or how the risk varies by type of surgery is limited. The Million Women Study is a population-based prospective study that recruited 1.3 million women through the National Health Serv...

    journal_title:Expert review of hematology

    pub_type: 评论,杂志文章


    authors: Faltas B

    更新日期:2010-04-01 00:00:00

  • The gut microbiota - a modulator of endothelial cell function and a contributing environmental factor to arterial thrombosis.

    abstract::Introduction: There is emerging evidence linking the commensal gut microbiota with the development of cardiovascular disease and arterial thrombosis. In immunothrombosis, the host clotting system protects against the dissemination of invading microbes, not considering the huge number of microbes that interact with hos...

    journal_title:Expert review of hematology

    pub_type: 杂志文章,评审


    authors: Formes H,Reinhardt C

    更新日期:2019-07-01 00:00:00

  • Novel targeted therapeutics for mantle cell lymphoma--what's on the horizon?

    abstract::Major advances have significantly improved the outcome of mantle cell lymphoma (MCL). Incorporation of rituximab to CHOP regimen, the adoption of high dose cytarabine with frontline autologous stem cell transplantation in young patients, maintenance rituximab or bortezomib based chemotherapy in elderly patients, impro...

    journal_title:Expert review of hematology

    pub_type: 杂志文章,评审


    authors: El Halabi L,Ghez D,Ribrag V

    更新日期:2016-03-01 00:00:00

  • Point mutations which should not be overlooked in Hb H disease.

    abstract:BACKGROUND:Hb H disease is an alpha-thalassemia (α-thal) syndrome characterized by chronic hemolytic anemia that occurs when three of total four α-globin genes lost their function due to completely deletions or different kind of mutations. OBJECTIVE:We here described 66 patients who have been diagnosed for Hb H diseas...

    journal_title:Expert review of hematology

    pub_type: 杂志文章


    authors: Farashi S,Bayat N,Vakili S,Faramarzi Garous N,Ashki M,Imanian H,Najmabadi H,Azarkeivan A

    更新日期:2016-01-01 00:00:00

  • Risk and prognosis of adult primary immune thrombocytopenia.

    abstract::Adult immune thrombocytopenia was previously considered a benign disease affecting young people and with a low risk of severe bleeding. This view was challenged by studies published during the past decade, as the median age of adult immune thrombocytopenia patients has been found to be 55-60 years and the incidence in...

    journal_title:Expert review of hematology

    pub_type: 杂志文章,评审


    authors: Frederiksen H,Christiansen CF,Nørgaard M

    更新日期:2012-04-01 00:00:00

  • Current options to manage Waldenström's macroglobulinemia.

    abstract:INTRODUCTION:Waldenström's macroglobulinemia (WM) is a rare, incurable B-cell lymphoma, with a median survival of 5-10 years in symptomatic patients. There is no consensus on the standard of care and several agents are currently used in these patients. Areas covered: In this article, we will review the use of standard ...

    journal_title:Expert review of hematology

    pub_type: 杂志文章,评审


    authors: Benevolo G,Nicolosi M,Santambrogio E,Vitolo U

    更新日期:2017-07-01 00:00:00

  • Novel approaches to the treatment of sickle cell disease: the potential of histone deacetylase inhibitors.

    abstract::Sickle cell disease (SCD) is a severe genetic disorder of hemoglobin causing vaso-occlusion. Patients suffer severe anemia, strokes, renal failure, pulmonary compromise and shortened life expectancy. Over 90,000 people in the USA have SCD, and the options for therapy are limited and only partially effective. With the ...

    journal_title:Expert review of hematology

    pub_type: 杂志文章,评审


    authors: Okam MM,Ebert BL

    更新日期:2012-06-01 00:00:00