<br />
HEMATOLOGY<br />
US Edition Vol 1.1 January 2020 emjreviews.com<br />
+ ASH CONGRESS 2019<br />
Reviewed<br />
+ EDITOR'S PICK<br />
Acute Chest Syndrome<br />
in Children with Sickle Cell<br />
Disease: Current Perspectives<br />
on Pathogenesis and<br />
Treatment<br />
+ CONGRESS INTERVIEWS<br />
Prof Theresa L. Coetzer and Dr Pierluigi<br />
Porcu speak about their current research<br />
and how ASH is supporting the<br />
ever-growing field of haematology<br />
+ ARTICLE<br />
Therapeutic Options in Myelodysplastic<br />
Syndromes Following Hypomethylating<br />
Agent Failure<br />
Creative Commons Attribution-Non Commercial 4.0 January 2020 • HEMATOLOGY<br />
1<br />
Contents<br />
+ EDITORIAL BOARD 4<br />
+ WELCOME <a title="EMJ Hematology US 1.1 2020 page 1" href="http://viewer.zmags.com/publication/6b16c32c?page=1"> HEMATOLOGY US Edition </a>
<a title="EMJ Hematology US 1.1 2020 page 2" href="http://viewer.zmags.com/publication/6b16c32c?page=2"> Contents + EDITORIAL BOARD </a>
<a title="EMJ Hematology US 1.1 2020 page 3" href="http://viewer.zmags.com/publication/6b16c32c?page=3"> “EMJ Hematology US Edition is </a>
<a title="EMJ Hematology US 1.1 2020 page 4" href="http://viewer.zmags.com/publication/6b16c32c?page=4"> Editorial Board Editor-in-Chief Dr Emanuele </a>
<a title="EMJ Hematology US 1.1 2020 page 5" href="http://viewer.zmags.com/publication/6b16c32c?page=5"> Aims and Scope EMJ is an online only, peer-review</a>
<a title="EMJ Hematology US 1.1 2020 page 6" href="http://viewer.zmags.com/publication/6b16c32c?page=6"> EMJ Hematol. US 1.12020 Chief Executive Officer</a>
<a title="EMJ Hematology US 1.1 2020 page 7" href="http://viewer.zmags.com/publication/6b16c32c?page=7"> Welcome A mere 6 weeks ago, the EMJ team were in </a>
<a title="EMJ Hematology US 1.1 2020 page 8" href="http://viewer.zmags.com/publication/6b16c32c?page=8"> Share your knowledge with the world. </a>
<a title="EMJ Hematology US 1.1 2020 page 9" href="http://viewer.zmags.com/publication/6b16c32c?page=9"> Foreword Dear Friends and Colleagues, I am very </a>
<a title="EMJ Hematology US 1.1 2020 page 10" href="http://viewer.zmags.com/publication/6b16c32c?page=10"> Available now.</a>
<a title="EMJ Hematology US 1.1 2020 page 11" href="http://viewer.zmags.com/publication/6b16c32c?page=11"> Read more. Congress Review + Review of the EHA</a>
<a title="EMJ Hematology US 1.1 2020 page 12" href="http://viewer.zmags.com/publication/6b16c32c?page=12"> </a>
<a title="EMJ Hematology US 1.1 2020 page 13" href="http://viewer.zmags.com/publication/6b16c32c?page=13"> Congress Review Review of the American Society</a>
<a title="EMJ Hematology US 1.1 2020 page 14" href="http://viewer.zmags.com/publication/6b16c32c?page=14"> acute myeloid leukaemia). Developed by leading cl</a>
<a title="EMJ Hematology US 1.1 2020 page 15" href="http://viewer.zmags.com/publication/6b16c32c?page=15"> ASH 2019 REVIEWED Creative Commons Attribution-</a>
<a title="EMJ Hematology US 1.1 2020 page 16" href="http://viewer.zmags.com/publication/6b16c32c?page=16"> New Gene Therapy Approach for Sickle Cell</a>
<a title="EMJ Hematology US 1.1 2020 page 17" href="http://viewer.zmags.com/publication/6b16c32c?page=17"> Maintenance of First Remission Leads to Improve</a>
<a title="EMJ Hematology US 1.1 2020 page 18" href="http://viewer.zmags.com/publication/6b16c32c?page=18"> Sutimlimab Offers Favourable Effects on Symptoms </a>
<a title="EMJ Hematology US 1.1 2020 page 19" href="http://viewer.zmags.com/publication/6b16c32c?page=19"> "Our study provides a much richer understanding o</a>
<a title="EMJ Hematology US 1.1 2020 page 20" href="http://viewer.zmags.com/publication/6b16c32c?page=20"> Triple Drug Combination Aids Survival Benefi</a>
<a title="EMJ Hematology US 1.1 2020 page 21" href="http://viewer.zmags.com/publication/6b16c32c?page=21"> Immunotherapy versus Chemotherapy for B-Acute Lym</a>
<a title="EMJ Hematology US 1.1 2020 page 22" href="http://viewer.zmags.com/publication/6b16c32c?page=22"> New 'Choosing Wisely' List Developed to Reduce </a>
<a title="EMJ Hematology US 1.1 2020 page 23" href="http://viewer.zmags.com/publication/6b16c32c?page=23"> A Bright Future for Sickle Cell Disease Patients </a>
<a title="EMJ Hematology US 1.1 2020 page 24" href="http://viewer.zmags.com/publication/6b16c32c?page=24"> Socioeconomic Factors at Play in Cancer Sur</a>
<a title="EMJ Hematology US 1.1 2020 page 25" href="http://viewer.zmags.com/publication/6b16c32c?page=25"> First Engineered Blood Vessel Model Sheds Light</a>
<a title="EMJ Hematology US 1.1 2020 page 26" href="http://viewer.zmags.com/publication/6b16c32c?page=26"> Congress Interviews We spoke to Prof Theresa L. </a>
<a title="EMJ Hematology US 1.1 2020 page 27" href="http://viewer.zmags.com/publication/6b16c32c?page=27"> Q5 including gene editing. I am currently involve</a>
<a title="EMJ Hematology US 1.1 2020 page 28" href="http://viewer.zmags.com/publication/6b16c32c?page=28"> Q8 upon return to their home country. The HVO- B</a>
<a title="EMJ Hematology US 1.1 2020 page 29" href="http://viewer.zmags.com/publication/6b16c32c?page=29"> Dr Pierluigi Porcu Chair of the ASH Foundation C</a>
<a title="EMJ Hematology US 1.1 2020 page 30" href="http://viewer.zmags.com/publication/6b16c32c?page=30"> additional Phase IIa patients, had no Gr</a>
<a title="EMJ Hematology US 1.1 2020 page 31" href="http://viewer.zmags.com/publication/6b16c32c?page=31"> that each patient has. For example, we have a</a>
<a title="EMJ Hematology US 1.1 2020 page 32" href="http://viewer.zmags.com/publication/6b16c32c?page=32"> Abstract Reviews The following pages showc</a>
<a title="EMJ Hematology US 1.1 2020 page 33" href="http://viewer.zmags.com/publication/6b16c32c?page=33"> the functional role Fli-1 plays in HSPC specificat</a>
<a title="EMJ Hematology US 1.1 2020 page 34" href="http://viewer.zmags.com/publication/6b16c32c?page=34"> Haemogenic endothelial cells isolated fro</a>
<a title="EMJ Hematology US 1.1 2020 page 35" href="http://viewer.zmags.com/publication/6b16c32c?page=35"> Novel CD33 Antibodies Reveal CD33 Splicing </a>
<a title="EMJ Hematology US 1.1 2020 page 36" href="http://viewer.zmags.com/publication/6b16c32c?page=36"> RESULTS Western blotting analysis using t</a>
<a title="EMJ Hematology US 1.1 2020 page 37" href="http://viewer.zmags.com/publication/6b16c32c?page=37"> METHODS AND RESULTS A retrospective chart revi</a>
<a title="EMJ Hematology US 1.1 2020 page 38" href="http://viewer.zmags.com/publication/6b16c32c?page=38"> Assessment of Mortality and its Associated Risk F</a>
<a title="EMJ Hematology US 1.1 2020 page 39" href="http://viewer.zmags.com/publication/6b16c32c?page=39"> importance to have some objective data guiding pa</a>
<a title="EMJ Hematology US 1.1 2020 page 40" href="http://viewer.zmags.com/publication/6b16c32c?page=40"> Lactate Release by Inflammatory Bone </a>
<a title="EMJ Hematology US 1.1 2020 page 41" href="http://viewer.zmags.com/publication/6b16c32c?page=41"> high generation of ROS, elevated HIF-1α levels, a</a>
<a title="EMJ Hematology US 1.1 2020 page 42" href="http://viewer.zmags.com/publication/6b16c32c?page=42"> Acute Chest Syndrome in Children with Sickle Cel</a>
<a title="EMJ Hematology US 1.1 2020 page 43" href="http://viewer.zmags.com/publication/6b16c32c?page=43"> INTRODUCTION Acute chest syndrome (ACS) is a</a>
<a title="EMJ Hematology US 1.1 2020 page 44" href="http://viewer.zmags.com/publication/6b16c32c?page=44"> leading to increased red blood cell sicklin</a>
<a title="EMJ Hematology US 1.1 2020 page 45" href="http://viewer.zmags.com/publication/6b16c32c?page=45"> mediators leukotrienes and prostaglandins; these </a>
<a title="EMJ Hematology US 1.1 2020 page 46" href="http://viewer.zmags.com/publication/6b16c32c?page=46"> CHEST SYNDROME TREATMENT MODALITIES FOR ACUTE AC</a>
<a title="EMJ Hematology US 1.1 2020 page 47" href="http://viewer.zmags.com/publication/6b16c32c?page=47"> The essential components of supportive care </a>
<a title="EMJ Hematology US 1.1 2020 page 48" href="http://viewer.zmags.com/publication/6b16c32c?page=48"> Nevertheless, automated red cell exchange</a>
<a title="EMJ Hematology US 1.1 2020 page 49" href="http://viewer.zmags.com/publication/6b16c32c?page=49"> remains controversial, reports show that when adm</a>
<a title="EMJ Hematology US 1.1 2020 page 50" href="http://viewer.zmags.com/publication/6b16c32c?page=50"> 17. Poulter EY et al. Acute chest </a>
<a title="EMJ Hematology US 1.1 2020 page 51" href="http://viewer.zmags.com/publication/6b16c32c?page=51"> expression and increased h2o2 61. </a>
<a title="EMJ Hematology US 1.1 2020 page 52" href="http://viewer.zmags.com/publication/6b16c32c?page=52"> Therapeutic Options in Myelodysplastic Syndromes </a>
<a title="EMJ Hematology US 1.1 2020 page 53" href="http://viewer.zmags.com/publication/6b16c32c?page=53"> and decitabine. HMA have been the standard of car</a>
<a title="EMJ Hematology US 1.1 2020 page 54" href="http://viewer.zmags.com/publication/6b16c32c?page=54"> Table 1: International Prognostic Scoring System </a>
<a title="EMJ Hematology US 1.1 2020 page 55" href="http://viewer.zmags.com/publication/6b16c32c?page=55"> Table 3: Agents under active investigation in pat</a>
<a title="EMJ Hematology US 1.1 2020 page 56" href="http://viewer.zmags.com/publication/6b16c32c?page=56"> Table 3 continued. Mechanism Agent NCT i</a>
<a title="EMJ Hematology US 1.1 2020 page 57" href="http://viewer.zmags.com/publication/6b16c32c?page=57"> (7+3, intermediate versus high dose cytosine</a>
<a title="EMJ Hematology US 1.1 2020 page 58" href="http://viewer.zmags.com/publication/6b16c32c?page=58"> dosing schedules in which azacitidine given as 30</a>
<a title="EMJ Hematology US 1.1 2020 page 59" href="http://viewer.zmags.com/publication/6b16c32c?page=59"> Lysine Demethylase 1 Inhibitors Lysine demethyl</a>
<a title="EMJ Hematology US 1.1 2020 page 60" href="http://viewer.zmags.com/publication/6b16c32c?page=60"> FLT3 Inhibitors The FLT3 gene encodes a tyrosine</a>
<a title="EMJ Hematology US 1.1 2020 page 61" href="http://viewer.zmags.com/publication/6b16c32c?page=61"> II study42 of venetoclax in combination wit</a>
<a title="EMJ Hematology US 1.1 2020 page 62" href="http://viewer.zmags.com/publication/6b16c32c?page=62"> (ACE-536) to treat anemia due 29. t</a>
<a title="EMJ Hematology US 1.1 2020 page 63" href="http://viewer.zmags.com/publication/6b16c32c?page=63"> NCT02610777. https:/clinicaltrials. 60. </a>
<a title="EMJ Hematology US 1.1 2020 page 64" href="http://viewer.zmags.com/publication/6b16c32c?page=64"> administration of azacitidine with or without ent</a>
<a title="EMJ Hematology US 1.1 2020 page 65" href="http://viewer.zmags.com/publication/6b16c32c?page=65"> Reed Sternberg-Like Cells in an Aggressive L</a>
<a title="EMJ Hematology US 1.1 2020 page 66" href="http://viewer.zmags.com/publication/6b16c32c?page=66"> Figure 1: Radiological findings. (A) Whole body CT</a>
<a title="EMJ Hematology US 1.1 2020 page 67" href="http://viewer.zmags.com/publication/6b16c32c?page=67"> Figure 2: Histomorphology and immunohistochemistr</a>
<a title="EMJ Hematology US 1.1 2020 page 68" href="http://viewer.zmags.com/publication/6b16c32c?page=68"> negative, and approximately 15% are CD4+/CD8-. Cy</a>
<a title="EMJ Hematology US 1.1 2020 page 69" href="http://viewer.zmags.com/publication/6b16c32c?page=69"> References 1. Pileri SA et al., “Peripheral </a>
<a title="EMJ Hematology US 1.1 2020 page 70" href="http://viewer.zmags.com/publication/6b16c32c?page=70"> We want you to write for the EMJ blog. C</a>
<a title="EMJ Hematology US 1.1 2020 page 71" href="http://viewer.zmags.com/publication/6b16c32c?page=71"> Pernicious Anaemia: Mechanisms, Diagnosis, a</a>
<a title="EMJ Hematology US 1.1 2020 page 72" href="http://viewer.zmags.com/publication/6b16c32c?page=72"> Whether the stomach pathogen Helicobacter pyl</a>
<a title="EMJ Hematology US 1.1 2020 page 73" href="http://viewer.zmags.com/publication/6b16c32c?page=73"> Physiopathology of Pernicious Anaemia PA is a </a>
<a title="EMJ Hematology US 1.1 2020 page 74" href="http://viewer.zmags.com/publication/6b16c32c?page=74"> The elevation in bilirubin levels, caused b</a>
<a title="EMJ Hematology US 1.1 2020 page 75" href="http://viewer.zmags.com/publication/6b16c32c?page=75"> WITH HYPERHOMOCYSTEINEMIA ASSOCIATED THROMBOEMBOL</a>
<a title="EMJ Hematology US 1.1 2020 page 76" href="http://viewer.zmags.com/publication/6b16c32c?page=76"> The anaemia is macrocytic and normochromic with </a>
<a title="EMJ Hematology US 1.1 2020 page 77" href="http://viewer.zmags.com/publication/6b16c32c?page=77"> Differentiating between vitamin B12 deficiency and</a>
<a title="EMJ Hematology US 1.1 2020 page 78" href="http://viewer.zmags.com/publication/6b16c32c?page=78"> Table 1: Other causes of macrocytic anaemia and c</a>
<a title="EMJ Hematology US 1.1 2020 page 79" href="http://viewer.zmags.com/publication/6b16c32c?page=79"> commencement of new symptoms. These may include e</a>
<a title="EMJ Hematology US 1.1 2020 page 80" href="http://viewer.zmags.com/publication/6b16c32c?page=80"> 36. Vannella L et al. Systematic review: </a>
<a title="EMJ Hematology US 1.1 2020 page 81" href="http://viewer.zmags.com/publication/6b16c32c?page=81"> ‘Girdle Syndrome’ Progressing to Ischaemic Coliti</a>
<a title="EMJ Hematology US 1.1 2020 page 82" href="http://viewer.zmags.com/publication/6b16c32c?page=82"> few weeks before; moreover, the patient noted dia</a>
<a title="EMJ Hematology US 1.1 2020 page 83" href="http://viewer.zmags.com/publication/6b16c32c?page=83"> U/L), alanine transaminase (52.0 U/L), and l</a>
<a title="EMJ Hematology US 1.1 2020 page 84" href="http://viewer.zmags.com/publication/6b16c32c?page=84"> DISCUSSION Acute abdominal pain is a common symp</a>
<a title="EMJ Hematology US 1.1 2020 page 85" href="http://viewer.zmags.com/publication/6b16c32c?page=85"> levels <28% with exchange transfusion therapy, on</a>
<a title="EMJ Hematology US 1.1 2020 page 86" href="http://viewer.zmags.com/publication/6b16c32c?page=86"> Never miss an update again. J</a>