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Hematología

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124 papers 25 to 100 followers
By Javier Pilcante Hematologist
https://www.readbyqxmd.com/read/29699840/iron-toxicity-its-effect-on-the-bone-marrow
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
https://www.readbyqxmd.com/read/28321258/the-evolution-of-prognostic-factors-in-multiple-myeloma
#10
REVIEW
Amr Hanbali, Mona Hassanein, Walid Rasheed, Mahmoud Aljurf, Fahad Alsharif
Multiple myeloma (MM) is a heterogeneous hematologic malignancy involving the proliferation of plasma cells derived by different genetic events contributing to the development, progression, and prognosis of this disease. Despite improvement in treatment strategies of MM over the last decade, the disease remains incurable. All efforts are currently focused on understanding the prognostic markers of the disease hoping to incorporate the new therapeutic modalities to convert the disease into curable one. We present this comprehensive review to summarize the current standard prognostic markers used in MM along with novel techniques that are still in development and highlight their implications in current clinical practice...
2017: Advances in Hematology
https://www.readbyqxmd.com/read/29512199/chronic-neutrophilic-leukemia-2018-update-on-diagnosis-molecular-genetics-and-management
#11
Michelle A Elliott, Ayalew Tefferi
DISEASE OVERVIEW AND DIAGNOSIS: Chronic neutrophilic leukemia (CNL) is a potentially aggressive myeloproliferative neoplasm, for which current WHO diagnostic criteria include leukocytosis of ≥ 25 x 109 /L of which ≥ 80% are neutrophils, with < 10% circulating neutrophil precursors with blasts rarely observed. In addition, there is no dysplasia, nor clinical or molecular criteria for other myeloproliferative neoplasms. UPDATE ON DIAGNOSIS: Previously the diagnosis of CNL was often as one of exclusion based on no identifiable cause for physiologic neutrophilia in patients fulfilling the aforementioned criteria...
August 2018: American Journal of Hematology
https://www.readbyqxmd.com/read/29574922/transitioning-patients-with-immune-thrombocytopenia-to-second-line-therapy-challenges-and-best-practices
#12
REVIEW
Adam Cuker
In patients with immune thrombocytopenia who do not adequately respond to first-line therapy, there is no clear consensus on which second-line therapy to initiate and when. This situation leads to suboptimal approaches, including prolonged exposure to treatments that are not intended for long-term use (eg, corticosteroids) and overuse of off-label therapies (eg, rituximab) while approved, more efficacious options exist. These approaches may not only fail to address symptoms and burden of disease, but may also worsen health-related quality of life...
March 25, 2018: American Journal of Hematology
https://www.readbyqxmd.com/read/28655976/iron-deficiency-anemia-in-chronic-liver-disease-etiopathogenesis-diagnosis-and-treatment
#13
REVIEW
Eleana Gkamprela, Melanie Deutsch, Dimitrios Pectasides
Chronic liver disease is accompanied by multiple hematological abnormalities. Iron deficiency anemia is a frequent complication of advanced liver disease. The etiology is multifactorial, mostly due to chronic hemorrhage into the gastrointestinal tract. The diagnosis of iron deficiency anemia is very challenging, as simple laboratory methods, including serum iron, ferritin, transferrin saturation (Tsat), and mean corpuscular volume are affected by the liver disease itself or the cause of the disease, resulting in difficulty in the interpretation of the results...
2017: Annals of Gastroenterology: Quarterly Publication of the Hellenic Society of Gastroenterology
https://www.readbyqxmd.com/read/28662310/thrombotic-thrombocytopenic-purpura-pathogenesis-diagnosis-and-potential-novel-therapeutics
#14
REVIEW
M Saha, J K McDaniel, X L Zheng
Thrombotic thrombocytopenic purpura (TTP), a potentially fatal clinical syndrome, is primarily caused by autoantibodies against the von Willebrand factor (VWF)-cleaving metalloprotease ADAMTS-13. In general, severe deficiency of plasma ADAMTS-13 activity (< 10 IU dL-1 ) with or without detectable inhibitory autoantibodies against ADAMTS-13 supports the diagnosis of TTP. A patient usually presents with thrombocytopenia and microangiopathic hemolytic anemia (i.e. schistocytes, elevated serum lactate dehydrogenase, decreased hemoglobin and haptoglobin) without other known etiologies that cause thrombotic microangiopathy (TMA)...
October 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28028030/myeloid-neoplasms-with-eosinophilia
#15
REVIEW
Andreas Reiter, Jason Gotlib
Molecular diagnostics has generated substantial dividends in dissecting the genetic basis of myeloid neoplasms with eosinophilia. The family of diseases generated by dysregulated fusion tyrosine kinase (TK) genes is recognized by the World Health Organization (WHO) category, "Myeloid/lymphoid neoplasms with eosinophilia and rearrangement of PDGFRA , PDGFRB , or FGFR1 , or with PCM1-JAK2 " In addition to myeloproliferative neoplasms (MPN), these patients can present with myelodysplastic syndrome/MPN, as well as de novo or secondary mixed-phenotype leukemias or lymphomas...
February 9, 2017: Blood
https://www.readbyqxmd.com/read/26209894/eosinophilia-in-hematologic-disorders
#16
REVIEW
Lorenzo Falchi, Srdan Verstovsek
Eosinophilia in the peripheral blood can be the manifestation various medical conditions, including benign or malignant disorders. There are 3 main types of eosinophilia-associated myeloid neoplasms (MN-eos): myeloid and lymphoid neoplasms, chronic eosinophilic leukemia not otherwise specified, and idiopathic hypereosinophilic syndrome (HES). Imatinib mesylate has revolutionized the treatment of molecularly defined MN-eos, and novel agents have been successfully used to treat HES. The discovery of new, recurrent molecular alterations in patients with MN-eos may improve their diagnosis and therapy...
August 2015: Immunology and Allergy Clinics of North America
https://www.readbyqxmd.com/read/27913542/new-therapies-for-hemophilia
#17
REVIEW
Steven W Pipe
Individuals with severe hemophilia have benefitted from 5 decades of advances that have led to widespread availability of safe and efficacious factors VIII and IX, a multidisciplinary integrated care model through a network of specialized hemophilia treatment centers, and aggressive introduction of prophylactic replacement therapy to prevent bleeding and preserve joint health. Yet, there are remaining challenges and treatment gaps which have prevented complete abrogation of all joint bleeding, and progressive joint deterioration may continue in some affected individuals over the course of a lifetime...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913546/diagnosing-von-willebrand-disease-genetic-analysis
#18
REVIEW
Anne Goodeve
Investigation of a patient with possible von Willebrand disease (VWD) includes a range of phenotypic analyses. Often, this is sufficient to discern disease type, and this will suggest relevant treatment. However, for some patients, phenotypic analysis does not sufficiently explain the patient's disorder, and for this group, genetic analysis can aid diagnosis of disease type. Polymerase chain reaction and Sanger sequencing have been mainstays of genetic analysis for several years. More recently, next-generation sequencing has become available, with the advantage that several genes can be simultaneously analyzed where necessary, eg, for discrimination of possible type 2N VWD or mild hemophilia A...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913547/new-treatment-approaches-to-von-willebrand-disease
#19
REVIEW
Michelle Lavin, James S O'Donnell
von Willebrand disease (VWD) is the commonest inherited bleeding disorder and results from either a quantitative or qualitative deficiency in the plasma glycoprotein von Willebrand factor (VWF). Recent large cohort studies have significantly enhanced our understanding of the molecular mechanisms involved in the pathogenesis of VWD. In contrast, however, there have been relatively few advances in the therapeutic options available for the treatment of bleeding in patients with VWD. Established treatment options include tranexamic acid, 1-deamino-8-d-arginine vasopressin (DDAVP), and plasma-derived VWF concentrates...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913548/warm-antibody-autoimmune-hemolytic-anemia
#20
REVIEW
Theodosia A Kalfa
Autoimmune hemolytic anemia (AIHA) is a rare and heterogeneous disease that affects 1 to 3/100 000 patients per year. AIHA caused by warm autoantibodies (w-AIHA), ie, antibodies that react with their antigens on the red blood cell optimally at 37°C, is the most common type, comprising ∼70% to 80% of all adult cases and ∼50% of pediatric cases. About half of the w-AIHA cases are called primary because no specific etiology can be found, whereas the rest are secondary to other recognizable underlying disorders...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
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