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Hematología

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135 papers 25 to 100 followers
By Javier Pilcante Hematologist
https://www.readbyqxmd.com/read/30057832/a-case-of-primary-refractory-immune-thrombocytopenia-challenges-in-choice-of-therapies
#1
Hanyin Wang, Hande Tuncer
The value of combination therapy for refractory ITP is not well defined. We present the case of a 29-year-old male with severe ITP refractory to initial standard therapy including steroids, IVIG, and subsequent splenectomy, who was treated with the combination therapy of rituximab, romiplostim, and mycophenolate and eventually developed thrombocytosis requiring plateletpheresis. Our case highlights the importance of the need to understand predictors of response to standard upfront treatment of acute ITP.
2018: Case Reports in Hematology
https://www.readbyqxmd.com/read/29946191/diagnostic-algorithm-for-lower-risk-myelodysplastic-syndromes
#2
REVIEW
Ghulam J Mufti, Donal P McLornan, Arjan A van de Loosdrecht, Ulrich Germing, Robert P Hasserjian
Rapid advances over the past decade have uncovered the heterogeneous genomic and immunologic landscape of myelodysplastic syndromes (MDS). This has led to notable improvements in the accuracy and timing of diagnosis and prognostication of MDS, as well as the identification of possible novel targets for therapeutic intervention. For the practicing clinician, however, this increase in genomic, epigenomic, and immunologic knowledge needs consideration in a "real-world" context to aid diagnostic specificity...
June 26, 2018: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/29411417/chronic-myeloid-leukemia-2018-update-on-diagnosis-therapy-and-monitoring
#3
Elias Jabbour, Hagop Kantarjian
DISEASE OVERVIEW: Chronic myeloid leukemia (CML) is a myeloproliferative neoplasm with an incidence of 1-2 cases per 100 000 adults. It accounts for approximately 15% of newly diagnosed cases of leukemia in adults. DIAGNOSIS: CML is characterized by a balanced genetic translocation, t(9;22)(q34;q11.2), involving a fusion of the Abelson gene (ABL1) from chromosome 9q34 with the breakpoint cluster region (BCR) gene on chromosome 22q11.2. This rearrangement is known as the Philadelphia chromosome...
March 2018: American Journal of Hematology
https://www.readbyqxmd.com/read/30006329/the-role-of-adamts13-testing-in-the-diagnosis-and-management-of-thrombotic-microangiopathies-and-thrombosis
#4
Camila Masias, Spero R Cataland
ADAMTS13 (A Disintegrin And Metalloproteinase with Thrombospondin type 1 motif, 13) is a metalloprotease responsible for cleavage of ultra large Von Willebrand factor (ULVWF) multimers. Severely deficient activity of the protease can trigger an acute episode of thrombotic thrombocytopenic purpura (TTP). Our understanding of the pathophysiology of TTP, has allowed us to grasp the important role of ADAMTS13 in other thrombotic microangiopathies (TMA) and thrombotic disorders, such as ischemic stroke and coronary artery disease...
July 13, 2018: Blood
https://www.readbyqxmd.com/read/30012635/the-rise-of-apoptosis-targeting-apoptosis-in-hematologic-malignancies
#5
Rebecca Valentin, Stephanie Grabow, Matthew S Davids
Dysregulation of the B-cell leukemia/lymphoma-2 (BCL-2) family of proteins of the intrinsic apoptotic pathway is fundamental to the pathophysiology of many hematologic malignancies. The BCL-2 family consists of regulatory proteins that either induce apoptosis (pro-apoptotic) or inhibit it (pro-survival). BCL-2, MCL-1 (myeloid cell leukemia-1) and BCL-xL (B cell lymphoma-extra large) are pro-survival proteins that are prime targets for anti-cancer therapy, and molecules targeting each are in various stages of preclinical and clinical development...
July 16, 2018: Blood
https://www.readbyqxmd.com/read/29795386/myelodysplastic-syndromes-current-treatment-algorithm-2018
#6
David P Steensma
Myelodysplastic syndromes (MDS) include a group of clonal myeloid neoplasms characterized by cytopenias due to ineffective hematopoiesis, abnormal blood and marrow cell morphology, and a risk of clonal evolution and progression to acute myeloid leukemia (AML). Because outcomes for patients with MDS are heterogeneous, individual risk stratification using tools such as the revised International Prognostic Scoring System (IPSS-R) is important in managing patients-including selecting candidates for allogeneic hematopoietic stem cell transplantation (ASCT), the only potentially curative therapy for MDS...
May 24, 2018: Blood Cancer Journal
https://www.readbyqxmd.com/read/29808921/how-i-manage-patients-with-relapsed-refractory-diffuse-large-b-cell-lymphoma
#7
REVIEW
Christian Gisselbrecht, Eric Van Den Neste
Despite progress in the upfront treatment of diffuse large B cell lymphoma (DLBCL), patients still experience relapses. Salvage chemotherapy followed by autologous stem cell transplantation (ASCT) is the standard second-line treatment for relapsed and refractory (R/R) DLBCL. However, half of the patients will not be eligible for transplantation due to ineffective salvage treatment, and the other half will relapse after ASCT. In randomized studies, no salvage chemotherapy regimen is superior to another. The outcomes are affected by the secondary International Prognostic Index at relapse and various biological factors...
May 29, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29713445/the-evolution-of-stem-cell-transplantation-in-multiple-myeloma
#8
REVIEW
Sarakshi Mahajan, Nidhi Tandon, Shaji Kumar
Autologous stem-cell transplantation (ASCT) remains an integral part of treatment for previously untreated, and may have value in the treatment of relapsed patients with, multiple myeloma (MM). The addition of novel agents like immunomodulators and proteasome inhibitors as induction therapy before and as consolidation/maintenance therapy after ASCT has led to an improvement in complete response (CR) rates, progression-free survival (PFS) and overall survival (OS). With advances in supportive care, older patients and patients with renal insufficiency are now able to safely undergo the procedure...
May 2018: Therapeutic Advances in Hematology
https://www.readbyqxmd.com/read/29314206/follicular-lymphoma-2018-update-on-diagnosis-and-management
#9
Arnold Freedman
DISEASE OVERVIEW: Follicular lymphoma is generally an indolent B cell lymphoproliferative disorder of transformed follicular center B cells. Follicular lymphoma (FL) is characterized by diffuse lymphadenopathy, bone marrow involvement, splenomegaly and less commonly other extranodal sites of involvement. In general, cytopenias can occur but constitutional symptoms of fever, nightsweats, and weight loss are uncommon. DIAGNOSIS: Diagnosis is based on histology of preferably a biopsy of a lymph node...
February 2018: American Journal of Hematology
https://www.readbyqxmd.com/read/29891116/cancer-cachexia-diagnosis-assessment-and-treatment
#10
REVIEW
Mohammadamin Sadeghi, Mahsa Keshavarz-Fathi, Vickie Baracos, Jann Arends, Maryam Mahmoudi, Nima Rezaei
Cancer cachexia is a multi-factorial syndrome, which negatively affects quality of life, responsiveness to chemotherapy, and survival in advanced cancer patients. Our understanding of cachexia has grown greatly in recent years and the roles of many tumor-derived and host-derived compounds have been elucidated as mediators of cancer cachexia. However, cancer cachexia remains an unmet medical need and attempts towards a standard treatment guideline have been unsuccessful. This review covers the diagnosis, assessment, and treatment of cancer cachexia; the elements impeding the formulation of a standard management guideline; and future directions of research for the improvement and standardization of current treatment procedures...
July 2018: Critical Reviews in Oncology/hematology
https://www.readbyqxmd.com/read/29760702/emerging-concepts-in-immune-thrombocytopenia
#11
REVIEW
Maurice Swinkels, Maaike Rijkers, Jan Voorberg, Gestur Vidarsson, Frank W G Leebeek, A J Gerard Jansen
Immune thrombocytopenia (ITP) is an autoimmune disease defined by low platelet counts which presents with an increased bleeding risk. Several genetic risk factors (e.g., polymorphisms in immunity-related genes) predispose to ITP. Autoantibodies and cytotoxic CD8+ T cells (Tc) mediate the anti-platelet response leading to thrombocytopenia. Both effector arms enhance platelet clearance through phagocytosis by splenic macrophages or dendritic cells and by induction of apoptosis. Meanwhile, platelet production is inhibited by CD8+ Tc targeting megakaryocytes in the bone marrow...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29699840/iron-toxicity-its-effect-on-the-bone-marrow
#12
REVIEW
Alessandro Isidori, Lorenza Borin, Elena Elli, Roberto Latagliata, Bruno Martino, Giuseppe Palumbo, Federica Pilo, Federica Loscocco, Giuseppe Visani, Paolo Cianciulli
Excess iron can be extremely toxic for the body and may cause organ damage in the absence of iron chelation therapy. Preclinical studies on the role of free iron on bone marrow function have shown that iron toxicity leads to the accumulation of reactive oxygen species, affects the expression of genes coding for proteins that regulate hematopoiesis, and disrupts hematopoiesis. These effects could be partially attenuated by iron-chelation treatment with deferasirox, suggesting iron toxicity may have a negative impact on the hematopoietic microenvironment...
April 13, 2018: Blood Reviews
https://www.readbyqxmd.com/read/29724716/jak2-and-jmjd1c-activate-nfe2-in-mpns
#13
John D Crispino
No abstract text is available yet for this article.
May 3, 2018: Blood
https://www.readbyqxmd.com/read/29634090/hodgkin-lymphoma-2018-update-on-diagnosis-risk-stratification-and-management
#14
Stephen M Ansell
DISEASE OVERVIEW: Hodgkin lymphoma (HL) is an uncommon B-cell lymphoid malignancy affecting 8500 new patients annually and representing approximately 10.2% of all lymphomas in the United States. DIAGNOSIS: HL is composed of two distinct disease entities: classical HL and nodular lymphocyte predominant HL. Nodular sclerosis, mixed cellularity, lymphocyte depletion, and lymphocyte-rich HL are subgroups of classical HL. RISK STRATIFICATION: An accurate assessment of the stage of disease in patients with HL is critical for the selection of the appropriate therapy...
May 2018: American Journal of Hematology
https://www.readbyqxmd.com/read/27054026/the-evolving-role-of-response-adapted-pet-imaging-in-hodgkin-lymphoma
#15
REVIEW
Michael Coyle, Lale Kostakoglu, Andrew M Evens
(18)F-fluorodeoxyglucose positron emission tomography with (FDG-PET) has a well-established role in the pre- and post-treatment staging of Hodgkin lymphoma (HL), however its use as a predictive therapeutic tool via responded-adapted therapy continues to evolve. There have been a multitude of retrospective and noncontrolled clinical studies showing that early (or interim) FDG-PET is highly prognostic in HL, particularly in the advanced-stage setting. Response-adapted treatment approaches in HL are attempting to diminish toxicity for low-risk patients by minimizing therapy, and conversely, intensify treatment for high-risk patients...
April 2016: Therapeutic Advances in Hematology
https://www.readbyqxmd.com/read/28246555/management-of-thrombosis-in-paroxysmal-nocturnal-hemoglobinuria-a-clinician-s-guide
#16
REVIEW
Morag Griffin, Talha Munir
Paroxysmal nocturnal haemoglobinuria (PNH), an ultra-orphan disease with a prevalence of 15.9 per million in Europe, is a life-threatening disorder, characterized by haemolysis, bone marrow failure and thrombosis. Patients with PNH prior to the availability of eculizumab had a median survival of between 10 and 22 years, with thrombosis accounting for 22-67% of deaths. 29-44% of patients had at least one thrombosis. This paper provides a clinician's guide to the diagnosis, management and complications of PNH, with an emphasis on thrombosis...
March 2017: Therapeutic Advances in Hematology
https://www.readbyqxmd.com/read/29051800/current-strategies-for-salvage-treatment-for-relapsed-classical-hodgkin-lymphoma
#17
REVIEW
Liana Nikolaenko, Robert Chen, Alex F Herrera
Hodgkin lymphoma (HL) is curable in 70-80% of patients with first-line therapy. However, relapses occur in a minority of patients with favorable early stage disease and are more frequent in patients with advanced HL. Salvage chemotherapy followed by high-dose chemotherapy and autologous stem cell transplant (ASCT) for patients with chemotherapy-sensitive disease is a standard treatment sequence for relapsed or refractory (rel/ref) HL. Patients who achieve complete response prior to ASCT have better survival outcomes...
October 2017: Therapeutic Advances in Hematology
https://www.readbyqxmd.com/read/29387330/novel-therapies-and-current-clinical-progress-in-hemophilia-a
#18
REVIEW
Pauline Balkaransingh, Guy Young
The evolution of hemophilia treatment and care is a fascinating one but has been fraught with many challenges at every turn. Over the last 50 years or so patients with hemophilia and providers have witnessed great advances in the treatment of this disease. With these advances, there has been a dramatic decrease in the mortality and morbidity associated with hemophilia. Even with the remarkable advancements in treatment, however, new and old challenges continue to plague the hemophilia community. The cost of factor replacement and the frequency of infusions, especially in patients with severe hemophilia on prophylaxis, remains a significant challenge for this population...
February 2018: Therapeutic Advances in Hematology
https://www.readbyqxmd.com/read/29461260/hemolysis-and-immune-regulation
#19
Hui Zhong, Karina Yazdanbakhsh
PURPOSE OF REVIEW: Hemolytic anemias caused by premature destruction of red blood cells occur in many disorders including hemoglobinopathies, autoimmune conditions, during infection or following reaction to drugs or transfusions. Recent studies which will be reviewed here have uncovered several novel mechanisms by which hemolysis can alter immunological functions and increase the risk of severe complications in hemolytic disorders. RECENT FINDINGS: Plasma-free heme can induce the formation of neutrophil extracellular traps (NETs) through reactive oxygen species signaling...
May 2018: Current Opinion in Hematology
https://www.readbyqxmd.com/read/29402471/direct-oral-anticoagulant-use-in-patients-with-thrombophilia-antiphospholipid-syndrome-or-venous-thrombosis-of-unusual-sites-a-narrative-review
#20
REVIEW
Laurent Bertoletti, Ygal Benhamou, Yannick Béjot, Sylvestre Marechaux, Saida Cheggour, Boris Aleil, Nicolas Lellouche, Jean-Guillaume Dillinger, Aurélien Delluc
Direct oral anticoagulants (DOACs) are indicated in the treatment and prevention of venous thromboembolism (VTE). However, the use of DOACs in unusual VTE, including cerebral venous thrombosis (CVT) and splanchnic venous thrombosis (SVT), and in patients with biological thrombophilia including minor thrombophilia (Factor V Leiden and prothrombin G20210A), major innate thrombophilia (protein C and S deficiency, and antithrombin) and major acquired thrombophilia (antiphospholipid syndrome [APS]), remains controversial due to the paucity of available data...
July 2018: Blood Reviews
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