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https://www.readbyqxmd.com/read/30515771/how-i-manage-children-with-diamond-blackfan-anaemia
#1
REVIEW
Marije Bartels, Marc Bierings
Diamond-Blackfan anaemia (DBA) is a rare inherited marrow failure disorder, characterized by hypoplastic anaemia, congenital anomalies and a predisposition to cancer as a result of ribosomal dysfunction. Historically, treatment is based on glucocorticoids and/or blood transfusions, which is accompanied by significant toxicity and long-term sequelae. Currently, stem cell transplantation is the only curative option for the haematological DBA phenotype. Whereas this procedure has been quite successful in the last decade in selected patients, novel therapies and biological insights are still warranted to improve clinical care for all DBA patients...
December 4, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/30335252/-how-i-manage-drug-therapy-failure
#2
A J Scheen
Drug therapy failure may occur with any medical intervention. It is badly accepted by both the physician and the patient. Drug failure may be attributed to the physician, the patient, the medication or the severity of the disease itself. Once drug failure is confirmed, causes should rapidly be identified in order to find a solution because several ones may often be offered.
October 2018: Revue Médicale de Liège
https://www.readbyqxmd.com/read/30032558/-how-i-manage-minimal-residual-disease-positive-patients-with-acute-leukemia-who-underwent-allogeneic-stem-cell-transplantation
#3
Y J Chang, X J Huang
No abstract text is available yet for this article.
June 14, 2018: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/29808921/how-i-manage-patients-with-relapsed-refractory-diffuse-large-b-cell-lymphoma
#4
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...
September 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29538795/how-i-manage-patients-with-anticoagulation-associated-bleeding-or-urgent-surgery
#5
REVIEW
Thomas C Sauter, Balthasar Eberle, Walter A Wuillemin, Thomas Thiele, Anne Angelillo-Scherrer, Aristomenis K Exadaktylos, Gabor Erdoes, Adam Cuker, Michael Nagler
Antithrombotic treatment puts patients at risk of major bleeding. Fast and adequate response to anticoagulant-associated bleeding may not only stop the bleeding but prevent severe complications. However, practical treatment algorithms to guide physicians in emergency situations are lacking. Important principles that arise from management of bleeding in general are (a) implementation of an in-house algorithm, (b) rapid identification and treatment of the bleeding source, (c) adequate fluid resuscitation, (d) consideration of the application of tranexamic acid and (e) appropriate coagulation testing...
2018: Swiss Medical Weekly
https://www.readbyqxmd.com/read/29377071/how-i-manage-red-cell-transfusions-in-patients-with-sickle-cell-disease
#6
REVIEW
David C Rees, Susan Robinson, Jo Howard
Sickle cell disease is one of the commonest serious inherited diseases in the world, and red cell transfusion is still one of the few effective treatments for acute and chronic complications. Transfusion corrects anaemia and dilutes out the number of red cells able to cause vaso-occlusion and vascular damage. Urgent red cell transfusions are used to correct acute anaemia, treat acute chest syndrome and patients with acute neurological symptoms. We use elective transfusions preoperatively for moderate risk surgery, and in some pregnant women...
February 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29363757/how-i-manage-patients-with-cold-agglutinin-disease
#7
Sigbjørn Berentsen
Cold agglutinin disease (CAD) is an uncommon autoimmune haemolytic anaemia in which a well-defined, clonal low-grade lymphoproliferative disorder of the bone marrow results in erythrocyte destruction mediated by the classical complement pathway. The pathogenesis, clinical features and diagnostic criteria are reviewed. Although anaemia is mild in some patients, approximately one-third of untreated patients have a haemoglobin level of ≤80 g/l, and about 50% have been considered transfusion dependent for shorter or longer periods...
May 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29224310/-how-i-manage-cytomegalovirus-infection-after-hematopoietic-stem-cell-transplantation
#8
Q F Liu
No abstract text is available yet for this article.
November 14, 2017: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/29183887/how-i-manage-monoclonal-gammopathy-of-undetermined-significance
#9
REVIEW
Ronald S Go, S Vincent Rajkumar
Monoclonal gammopathy of undetermined significance (MGUS) is, in many ways, a unique hematologic entity. Unlike most hematologic conditions in which the diagnosis is intentional and credited to hematologists, the discovery of MGUS is most often incidental and made by nonhematologists. MGUS is considered an obligate precursor to several lymphoplasmacytic malignancies, including immunoglobulin light-chain amyloidosis, multiple myeloma, and Waldenström macroglobulinemia. Therefore, long-term follow-up is generally recommended...
January 11, 2018: Blood
https://www.readbyqxmd.com/read/29169653/-how-to-manage-ebv-reactivation-and-ebv-ptld-cmv-and-human-herpesvirus-6-reactivation-and-infection-after-allogeneic-stem-cell-transplantation-a-report-of-the-sfgm-tc-update
#10
REVIEW
Eolia Brissot, Tamim Alsuliman, Bérengère Gruson, Eric Hermet, Yordanka Tirefort, Ibrahim Yakoub-Agha, Sophie Alain
The French society of bone marrow transplantation and cell therapy (SFGM-TC) organizes annually workshops in the attempt to harmonize clinical practices between different francophone transplantation center. Here, we report our recommendations regarding the management of Epstein Barr virus reactivation and lymphoproliferative disorders, cytomegalovirus (CMV) and human herpes virus 6 (HHV6) after allogeneic stem cell transplantation.
December 2017: Bulletin du Cancer
https://www.readbyqxmd.com/read/29048128/how-i-manage-relapse-of-chronic-myeloid-leukaemia-after-stopping-tyrosine-kinase-inhibitor-therapy
#11
REVIEW
Delphine Rea, François-Xavier Mahon
During the last 10 years, clinical trials formally demonstrated that about 50% of patients with chronic phase (CP) chronic myeloid leukaemia (CML) who achieve and maintain deep molecular responses for a prolonged period of time during treatment with imatinib or new generation tyrosine kinase inhibitors (TKIs) may successfully stop their anti-leukaemic therapy. Based on the accumulated knowledge from abundant clinical trial experience, TKI discontinuation is becoming an important goal to achieve and is about to enter clinical practice...
January 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/28877978/how-i-manage-pulmonary-langerhans-cell-histiocytosis
#12
REVIEW
Gwenaël Lorillon, Abdellatif Tazi
Pulmonary Langerhans cell histiocytosis (PLCH) is a rare sporadic cystic lung disease of unknown aetiology that is characterised by the infiltration and destruction of the wall of distal bronchioles by CD1a+ Langerhans-like cells. In adults, PLCH is frequently isolated and affects young smokers of both sexes. Recent multicentre studies have led to the more standardised management of patients in clinical practice. Smoking cessation is essential and is occasionally the only suitable intervention. Serial lung function testing is important because a significant proportion of patients may experience an early decline in forced expiratory volume in 1 s and develop airflow obstruction...
September 30, 2017: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/28771666/how-i-manage-sickle-cell-patients-with-high-transcranial-doppler-results
#13
REVIEW
John Brewin, Banu Kaya, Subarna Chakravorty
Stroke is one of the most severe complications to affect children with sickle cell anaemia (SCA). Transcranial doppler (TCD) is an accurate and non-invasive method to determine stroke risk. Randomised controlled trials have demonstrated the efficacy of chronic transfusion therapy in stroke prevention based on risk stratification determined by TCD velocities. This has led to the regular use of TCD monitoring for children with SCA in order to determine stroke risk. Significant resource allocation is necessary to facilitate training, quality assurance and failsafe arrangements for non-attenders...
November 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28474441/how-i-manage-patients-with-fanconi-anaemia
#14
REVIEW
Carlo Dufour
Fanconi Anaemia is a rare, genetic heterogeneous multisystem disease that is the most common congenital syndrome of marrow failure. Twenty genes have been reported to cause the disease. Remarkable progress has been made over the last 20 years in the understanding of the genetic and pathophysiological mechanisms. Unfortunately, these advances have not been completely paralleled by advances in medical treatment, where the most important component remains stem cell transplantation. This therapy, although contributing to long-term negative effects, such as increased occurrence of late malignancies, is the only current option capable of prolonging the survival of patients...
July 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28419427/how-i-manage-children-with-neutropenia
#15
REVIEW
David C Dale
Neutropenia, usually defined as a blood neutrophil count <1·5 × 109 /l, is a common medical problem for children and adults. There are many causes for neutropenia, and at each stage in life the clinical pattern of causes and consequences differs significantly. I recommend utilizing the age of the child and clinical observations for the preliminary diagnosis and primary management. In premature infants, neutropenia is quite common and contributes to the risk of sepsis with necrotizing enterocolitis. At birth and for the first few months of life, neutropenia is often attributable to isoimmune or alloimmune mechanisms and predisposes to the risk of severe bacterial infections...
August 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28295192/state-of-the-art-how-i-manage-immune-thrombocytopenia
#16
REVIEW
Nichola Cooper
The management of patients with immune thrombocytopenia (ITP) is rapidly evolving. Over the last 15 years, a number of novel treatments have improved practice, with many steroid-sparing agents and a reduction in the progression to splenectomy. Although this has improved clinical care, many therapeutic challenges remain. There is no diagnostic test, no biomarkers to direct treatment and few comparative studies to help management decisions. Development of up to date guidelines is difficult with little high-grade evidence...
April 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28275090/how-i-manage-the-toxicities-of-myeloma-drugs
#17
REVIEW
Michel Delforge, Heinz Ludwig
The treatment of multiple myeloma is considered a continuously evolving paradigm as a result of the growing availability of new and highly effective drugs, including first- and second-generation proteasome inhibitors, immunomodulatory agents, and monoclonal antibodies. Clinical trials advocate long-term rather than short-term treatment schedules with combinations of these new anti-myeloma drug classes. Although the overall toxicity profile of the recommended regimens can be considered favorable, their increasing complexity and prolonged use warrant a heightened vigilance for early and late side effects, a priori because real-life patients can be more frail or present with 1 or more comorbidities...
April 27, 2017: Blood
https://www.readbyqxmd.com/read/28146266/how-i-manage-patients-with-hairy-cell-leukaemia
#18
REVIEW
Philip A Thompson, Farhad Ravandi
Patients with hairy cell leukaemia (HCL) have highly favourable outcomes after purine analogue therapy. However, most patients subsequently relapse and require re-treatment. A minority of patients develop purine analogue-refractory disease. Targeted therapies have improved outcomes for such patients. Recently, the BRAF V600E mutation was identified in most patients with classical HCL, resulting in constitutive mitogen-activated protein kinase pathway activation; impressive responses are achieved in heavily pre-treated patients with BRAF inhibition...
May 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28096090/how-i-manage-ibrutinib-refractory-chronic-lymphocytic-leukemia
#19
REVIEW
Jennifer A Woyach
The introduction of the Bruton tyrosine kinase (BTK) inhibitor ibrutinib has dramatically changed the management of chronic lymphocytic leukemia (CLL). Although responses have been durable in the majority of patients, relapses do occur, especially in the high-risk patient population. Most relapses occur as the result of acquired mutations in BTK and PLCG2, which may facilitate success with alternative targeted therapies. As outcomes after ibrutinib relapse have been reported to be poor, specific strategies are needed for this patient population...
March 9, 2017: Blood
https://www.readbyqxmd.com/read/27982416/how-i-manage-peripheral-t-cell-lymphoma-not-otherwise-specified-and-angioimmunoblastic-t-cell-lymphoma-current-practice-and-a-glimpse-into-the-future
#20
REVIEW
Norbert Schmitz, Laurence de Leval
Peripheral T-cell lymphoma (PTCL), not otherwise specified (NOS) and angioimmunoblastic T-cell lymphoma (AITL) are the most frequent of more than 20 mature PTCL entities featuring a broad spectrum of morphological, immunophenotypic, molecular and clinical characteristics. Unfortunately, recent progress in understanding the (epi)genetic background of PTCL has not been met with similar advances in treatment. Thus, CHO(E)P [cyclophosphamide, doxorubicin, vincristine, and prednisone (plus etoposide)] remains standard first-line therapy...
March 2017: British Journal of Haematology
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