Mediterranean Journal of Hematology and Infectious Diseases <ul> <li class="show"><!--StartFragment --> <p><strong>The Mediterranean Journal of Hematology and Infectious Diseases has been selected for coverage in </strong><strong>Clarivate Analytics products and services. <br />Beginning with V. 7 (1) 2015, this publication is indexed and abstracted in:</strong></p> <p><strong>♦ Science Citation Index Expanded</strong><br /><strong>♦ Journal Citation Reports/InCites</strong> <br /><br /><strong>♦ First 2017 official Impact Factor: 1.183</strong> <br /><strong>♦ Official Impact Factor 2018. 1.586</strong> <br /><strong>♦ Official Impact Factor 2019. 1.600</strong></p> <p><strong><span class="info_label">ISI Journal Citation Reports @ Ranking: 2017</span></strong></p> <p><strong>List of contents:</strong></p> <p> </p> <p><a title="Volume 13, Year 2021" href=""><strong>13:(1) (2021):</strong></a> <strong><a title="Volume 13, Year 2021" href=""><img src="" alt="" /></a> ORIGINAL ARTICLES, REVIEWS:</strong></p> <p><strong>Reviews </strong><strong>Topics</strong><strong>:</strong></p> <p><strong><a title="Volume 12, Year 2020" href="">12:(1) (2020):</a> </strong><strong><a title="Volume 12, Year 2020" href=""><img src="" alt="" /></a> ORIGINAL ARTICLES, REVIEWS:</strong></p> <p><strong>COVID-19 and </strong><strong>Reviews</strong><strong> </strong><strong>Topics</strong><strong>:</strong></p> <ul> <li class="show">ANEMIAS DUE TO DEFICIENCY OR DEFECTIVE UTILIZATION OF IRON AND/OR VITAMINS. Guest Editor: Domenico Girelli. <a href="">More...</a></li> <li class="show">News on COVID-19, Review, Original Articles, and Letters to the Editor of the Mediterranean Journal of Hematology and Infectious Diseases. <a href="">More...</a></li> </ul> <p><a title="Volume 11, Year 2019" href=""><strong>11:(1) (2019):</strong></a> <strong><a title="Volume 11, Year 2019" href=""><img src="" alt="" /></a> ORIGINAL ARTICLES, REVIEWS:</strong></p> <p><strong>Reviews </strong><strong>Topics</strong><strong>:</strong></p> <ul> <li class="show">UPDATE ON THALASSEMIA AND HEMOGLOBINOPATHIES. Guest Editor: Raffaella Origa<strong>. </strong><a href="">More...</a></li> <li class="show">UPDATE ON HEMOPHILIA. Guest Editor: Giancarlo Castaman<strong>. </strong><a href="">More...</a></li> </ul> <p><strong><a href="">10:(1) (2018):</a> <a title="Volume 10, Year 2018" href=""><img src="" alt="" /></a> ORIGINAL ARTICLES, REVIEWS:</strong></p> <p><strong>Review Topics:</strong></p> <ul> <li class="show">UPDATE ON VIRAL INFECTIONS AND LYMPHOPROLIFERATIVE DISEASES. Guest Editors: M. Luppi and L. Arcaini <a href="">More...</a></li> </ul> <p><a href=""><strong>9:(1) (2017): </strong></a><strong><a title="Volume 9, Year 2017" href=""><img src="" alt="" /></a>ORIGINAL ARTICLES, REVIEWS:</strong></p> <p><strong>Review Topics:</strong></p> <ul> <li class="show">UPDATE ON SECONDARY LEUKEMIAS. Guest Editor: Richard Larson <a href="">More...</a></li> <li class="show">UPDATE on “Rare Plasma Cell Dyscrasias” Guest Editor: M.T. Petrucci <a href="">More...</a></li> </ul> <p><strong><a href="">8:(1) (2016):</a> <a title="Volume 8, Year 2016" href=""><img src="" alt="" /></a> ORIGINAL ARTICLES, REVIEWS:</strong></p> <p><strong>Review Topics:</strong></p> <ul> <li class="show">HEMATOPOIETIC STEM CELL TRANSPLANTATION AND INFECTIONS. Guest Editor: Miguel Sanz <a href="">More...</a></li> <li class="show">REVIEW SERIES: UPDATE ON FOLLICULAR LYMPHOMA. Guest Editor: Corrado Tarella <a href="">More...</a></li> </ul> <p><strong>8:(Supplementary Issue) (2016): <a href=""><img src="" alt="" /></a> Fifth International Symposium on Secondary Leukemia and Leukemogenesis</strong></p> <p><strong><a href="">7:(1) (2015):</a> <a title="Volume 7, Year 2015" href=""><img src="" alt="" /></a> ORIGINAL ARTICLES, REVIEWS:</strong></p> <p>Review Topics:</p> <ul> <li class="show">MYELODYSPLASTIC SYNDROMES. AN UPDATE. SINCE 2015. Guest Editors: C. Mecucci and M.T. Voso. <a href="">More...</a></li> <li class="show">BACTERIAL INFECTIONS IN HEMATOLOGIC PATIENTS: AN UPDATE. SINCE 2015.Guest Editors F. Aversa and M. Tumbarello <a href="">More...</a></li> </ul> <p><strong><a href="">6:(1) (2014):</a> <a title="Volume 6, Year 2014" href=""><img src="" alt="" /></a> ORIGINAL ARTICLES, REVIEWS:</strong></p> <p>Review Topics:</p> <ul> <li class="show">CHRONIC MYELOID LEUKEMIAS: AN UPDATE. Guest Editors: M. Baccarani and F. Pane. <a href="">More...</a></li> <li class="show">UPDATE IN HODGKIN LYMPHOMA. Guest Editor: A. Gallamini <a href="">More...</a></li> <li class="show">ACUTE LYMPHOID LEUKEMIA IN ADULTS: AN UPDATE. Guest Editors: R. Bassan and A.Rambaldi <a href="">More...</a></li> </ul> <p><strong><a href="">5:(1) (2013):</a> <a title="Volume 5, Year 2013" href=""><img src="" alt="" /></a> ORIGINAL ARTICLES, REVIEWS:</strong></p> <p>Review Topics:</p> <ul> <li class="show">ACUTE MYELOID LEUKEMIA IN THE ELDERLY. Guest Editors: S. Amadori and A. Venditti. <a href="">More...</a></li> <li class="show">VON WILLEBRAND FACTOR UPDATE. Guest Editors: A. Federici and F. Rodeghiero. <a href="">More...</a></li> <li class="show">TUBERCULOSIS UPDATE. Guest Editors: R. Cauda and A. Matteelli. <a href="">More...</a></li> </ul> <p><strong><a href="">4:(1) (2012):</a> <a title="Malaria In The World, Chronic Lymphoid Leukemia, Autologous Hemopoietic Stem Cell Transplantation In Leukemia And Lymphoma" href=""><img src="" alt="" /></a>ORIGINAL ARTICLES, REVIEWS:</strong></p> <p>Review Topics:</p> <ul> <li class="show">MALARIA IN THE WORLD: 2012 Update. Guest Editors: F. Castelli and E. Pizzigallo <a href="">More...</a></li> <li class="show">CHRONIC LYMPHOID LEUKEMIA: Update on Immunological Dysfunctions and Infections. Guest Editors: D. Efremov and L. Laurenti <a href="">More...</a></li> <li class="show">AUTOLOGOUS HEMOPOIETIC STEM CELL TRANSPLANTATION IN LEUKEMIA AND LYMPHOMA: 2012 UPDATE. Guest Editors: G. Meloni and G. Visani <a href="">More...</a></li> </ul> <p><strong><a href="">3:(1) (2011):</a> <a title="Fungal Infections, Thrombosis In The Mediterranean Area, Acute Promyelocytic Leukemia In The Mediterranean Area And In The Developing Countries, Therapy-Related Myeloid Neoplasms." href=""><img src="" alt="" /></a> ORIGINAL ARTICLES, REVIEWS:</strong></p> <p>Review Topics:</p> <ul> <li class="show">FUNGAL INFECTIONS. Guest Editor: L. Pagano <a href="">More...</a></li> <li class="show">THROMBOSIS IN THE MEDITERRANEAN AREA. Guest Editor: V. De Stefano <a href="">More...</a></li> <li class="show">ACUTE PROMYELOCYTIC LEUKEMIA IN THE MEDITERRANEAN AREA AND I THE DEVELOPING COUNTRIES: Guest Editors: F. Lo-Coco and G. Avvisati <a href="">More...</a></li> <li class="show">THERAPY-RELATED MYELOID NEOPLASMS: Guest Editor: R. Larson <a href="">More...</a></li> </ul> </li> </ul> Università Cattolica Sacro Cuore, Rome, Italy en-US Mediterranean Journal of Hematology and Infectious Diseases 2035-3006 <p>This is an Open Access article distributed under the terms of the Creative Commons Attribution License (<a href="" target="_blank" rel="noopener"></a>) which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.</p> <p><strong>Transfer of Copyright and Permission to Reproduce Parts of Published Papers.</strong>&nbsp;Authors will grant copyright of their articles to the Institute of Hematology, Catholic University, Rome . No formal permission will be required to reproduce parts (tables or illustrations) of published papers, provided the source is quoted appropriately and reproduction has no commercial intent.&nbsp;<strong>Reproductions with commercial intent will require written permission and payment of royalties.</strong></p> <div class="grammarly-disable-indicator">&nbsp;</div> Cover <p>Printable Cover Vol 13, Year 2021</p> Francesco Guidi Copyright (c) 2021 Francesco Guidi 2021-01-18 2021-01-18 13 1 SOCIOECONOMIC STATUS IS GLOBALLY A PROGNOSTIC FACTOR FOR OVERALL SURVIVAL OF MULTIPLE MYELOMA PATIENTS; SYNTHESIS OF STUDIES AND REVIEW OF THE LITERATURE <p>Socioeconomic status (SES) is reflecting differences in sociodemographic factors affecting cancer survivorship. Deprived, low SES populations has a higher prevalence of multiple myeloma and worst survival, a gap that widens over time.</p> <p><strong>Methods</strong>: We performed a meta-analysis of 16 studies (registries and cohorts) reporting survival data of myeloma patients according to SES. Ten studies reported Hazzard Ratio (HR) (95 % CI) and 16 studies reported p values. We combined the HR from 10 studies and by using the Mosteller-Bush formula we performed the synthesis of p values according to the area of the globe.</p> <p><strong>Results: A combination</strong> of HR from 10 studies including 85198 myeloma patients weighted to sample size of each study and adopting the hypothesis of random effect returned a combined HR: 1,26 (1,13-1,31) in favor of high SES patients.</p> <p>USA: Synthesis of p values coming from 6 studies (n=89807 pts) by using the Mosteller and Bush formula extracted a p-value of &lt;0.0001 favoring high SES patients</p> <p>Oceania: Synthesis of p values in two cohorts from Australia and New Zealand (n= 10196 pts) returned a p-value of 0,022 favoring high SES patients</p> <p>Europe: The synthesis of p values from UK and Greece studies (n=18533 pts) returned a p-value of &lt;0,0001 favoring high SES patients</p> <p>Asia: Synthesis of 2 studies from Asia (n=915 pts) returned a p-value of &lt;0,0001 favoring high SES patients</p> <p><strong>Conclusions:</strong> Across the globe and widening over decades socioeconomic status remains a gap for equality in myeloma care</p> Stergios Intzes Marianthi Symeonidou Konstantinos Zagoridis Aikaterini Pentidou Spanoudakis Emmanouil Copyright (c) 2020 Stergios Intzes, Marianthi Symeonidou, Konstantinos Zagoridis, Aikaterini Pentidou, Spanoudakis Emmanouil 2021-01-01 2021-01-01 13 1 e2021006 e2021006 10.4084/mjhid.2021.006 Thalassemia carrier detection among pregnant women <p>Thalassemia major becomes the fifth catastrophic disease in Indonesia, however, national wide screening program is not mandatory. This study aimed to re-assess the validity of the various erythrocyte indices in determining ?-thalalssemia carrier among pregnant women in a low resource setting area. An analytic study was performed, comparing conventional cut-off various erythrocyte indices with new modified cut-offs by Kumar <em>et al</em>. The concordance analysis was calculated with Mentzer Index (MI) as reference. The validity of erythrocyte indices against hemoglobin (Hb) A2 were analysed, confirmed by molecular examination for ?- and common ?-Globin South East Asia population. Of 102 pregnant women, 34% was still anemic after completion of 90 iron pills. The concordance of conventional cut-off Shine &amp; Lal index (&lt;1530) was none to slight in agreement (kappa 0.097) to conventional cut-off MI (&lt;13). The concordance of SLI increased significantly to substantial agreement when both used modified cut-offs (kappa 0.729). However, both SLI and MI had missed most of HbE carriers and ?-thalassemia carriers which seemed to be prevalent in this population as shown by DNA examination. In contrary, simple MCV&lt;80fl and MCH&lt;27pg had covered those carriers. This is the first study from Nusa Tenggara Timur, a low resource area in Indonesia in attempt to mass screen thalassemia carriers in this area where a simple MCV&lt;80 fL and MCH&lt;27pg have been used for preliminary screening rather than other indices. Since the population in eastern part of Indonesia has different genetic background compared to the west, DNA analysis is of great interest to map the spectrum of globin mutations.</p> Edhyana Sahiratmadja Merry M.V. Seu Ita M. Nainggolan Johanes C. Mose Ramdan Panigoro Copyright (c) 2020 Edhyana Sahiratmadja, Merry M.V. Seu, Ita M. Nainggolan, Johanes C. Mose, Ramdan Panigoro 2021-01-01 2021-01-01 13 1 e2021003 e2021003 10.4084/mjhid.2021.003 Coronavirus disease 2019 (COVID-19) severity in patients with thalassemias: A Nationwide Iranian Experience <p>None</p> Mehran Karimi Sezaneh Haghpanah Azita Azarkeivan Sara Matin Arash Safaei Vincenzo De Sanctis Copyright (c) 2020 Mehran Karimi, Sezaneh Haghpanah , Azita Azarkeivan , Sara Matin, Arash Safaei, Vincenzo De Sanctis 2021-01-01 2021-01-01 13 1 e2021008 e2021008 10.4084/mjhid.2021.008 Clinical and laboratory features of Hemoglobin La Desirade variant in association with sickle cell and alpha thalassemia genes <p><strong>Abstract </strong></p> <p>Hemoglobin La Desirade (Hb La Desirade) is an unstable hemoglobin variant characterized by amino acid Alanine (Ala) replacing Valine (Val) at position 129 (H7) in the beta chain. Hb La Desirade exhibits a decreased oxygen affinity and normal heme-heme interaction. Interestingly, on analysis by standard electrophoresis, it migrates in the same region as normal HbA, and HbA actually represents a combination of HbA and Hb La Desirade together. This variant was reported as compound heterozygous with other Hemoglobin variants such as HbS, HbC or beta thalassemia, and more recently with Southeast Asian ovalocytosis and Hb Louisville with varying clinical manifestations.&nbsp; Herein, we describe the clinical and laboratory findings in a number of Omani Arab families who presented to our service for various reasons, presenting with Hemoglobin La Desirade with sickle gene and alpha thalassemia. Our patients with Hb La Desirade trait, were clinically asymptomatic with no evidence of anemia. However when it is associated with other abnormal hemoglobin variants such as HbS, leading to sickle/La Desirade compound heterozygosity, there was mild anemia with significant degree of hypochromia and microcytosis. The most striking feature was that the levels of HbS and HbA were almost equal on HPLC, and these cases could be misdiagnosed as sickle cell trait (SCT). However, the levels of Hb S in these compound heterozygotes (40.4-45.9) were higher than normally seen for the diagnosis of SCT in this population.</p> <p>&nbsp;</p> Salam Alkindi Shoaib Al Zadjali Mohamed Al Rawahi Hamoud Al Haddabi Shahina Daar Refaat Abdullah Elsadek Bahaa Eldeen Diab Sherkawy Anil Pathare Copyright (c) 2020 Salam Alkindi, Dr. , Mr. , Mr. , prof. , Dr. , Dr. , Dr. 2021-01-01 2021-01-01 13 1 e2021010 e2021010 10.4084/mjhid.2021.010 THE HEMATOLOGICAL PARAMETERS AND BIOCHEMICAL MARKERS OF IRON STATUS IN ALFA-THALASSEMIA 3.7KB DELETION FROM METROPOLITAN REGION OF MANAUS, AMAZONAS, BRAZIL. <p><strong>Background: </strong>Alpha Thalassemia (a-thal) is a heterogeneous group of hereditary alterations caused by deletions that affect alpha regulatory genes and the 3.7Kb deletion is the most frequent worldwide. In Brazil, the prevalence ranges from 20% and 35%, depending mainly on race, being more predominant in Afro-descendants. <strong>Purpose</strong>: The aim was to determine ?-thal -<sup>?3.7Kb</sup> and -<sup>?4.2Kb</sup> deletions, estimating their frequency in individuals from five regions of Amazon. <strong>Methods: </strong>Volunteers (individuals &gt;18 years old, of both genders) blood samples (n=1809) were collected from march 2014 to september 2017, at hospitals and/or health centers of each participant city. Alpha Thalassemia 3.7<sup>Kb</sup> genotyping was performed by GAP-PCR, while 4.2Kb deletion by Multiplex-PCR. The studied population included: Manaus (capital), 356 (19.7%) samples; Iranduba 232 (12.8%); Manacapuru, 287 (15.9%); Presidente Figueiredo, 370 (20.5%); Itacoatiara, 301 (16.6%); and Coari, 263 (14.5%). <strong>Results: </strong>The average age among males was 35.3±14.8, while for females it was 36.7±14.9 years old. Alpha Thalassemia was diagnosed in 143 individuals (7.9%) and all of these individuals carried the 3.7<sup>Kb</sup> deletion, 5.95% in heterozygosis and 1.95% in homozygosis. The associations analyses to the <strong>a</strong>-thal genotypes were statistically significant for all hematological parameters (p&lt;.001), except serum iron and serum ferritin analyses among carriers ?-thal. Microcytosis (MCV &lt;80fL) was found in 158 individuals (8,46%). <strong>Conclusion: </strong>The alfa-thal prevalence corroborates with other Brazilians studies. Molecular diagnosis is important to prevent the most severe forms of the disease, thus epidemiological studies using molecular tools become extremely important in regions where the disease is underestimated. In this context, this is the first study that reports ?-thalassemia deletion in the population from State of Amazonas. Our findings are relevant because they have impact in the clinical therapeutic choice and also demonstrate the importance of differential diagnosis between genetic anemia and iron deficiency anemia.</p> Fernanda Cozendey Anselmo Abdou Gafar Soumanou Cleidiane de Aguiar Ferreira Flora Maia Viga Sobrinha Ana Caroline Santos Castro Rafael Oliveira Brito Adolfo José Mota Marilda de Souza Gonçalves Jose Pereira Moura Neto Copyright (c) 2020 Fernanda Cozendey Anselmo, Abdou Gafar Soumanou , Cleidiane de Aguiar Ferreira , Flora Maia Viga Sobrinha, Ana Caroline Santos Castro, Rafael Oliveira Brito, Adolfo José Mota, Marilda de Souza Gonçalves, Jose Pereira Moura Neto 2021-01-01 2021-01-01 13 1 e2021001 e2021001 10.4084/MJHID.2021.001 PREVALENCE OF SERIOUS BACTERIAL INFECTIONS IN CHILDREN WITH SICKLE CELL DISEASE AT KING ABDULAZIZ HOSPITAL, AL AHSA <p>Aim: The main aim was to report the prevalence and severity of serious bacterial infections (SBI) in children with sickle cell disease at King Abdulaziz Hospital, Al Ahsa, Saudi Arabia to aid in determining whether outpatient management of such cases is appropriate.</p> <p>Methods: We conducted a retrospective chart review of febrile children less than 14 years of age admitted with sickle cell disease 2005 through 2015.</p> <p>Results: During 320 admissions, 25 children had SBIs (8%) including pneumonia (n=11), osteomyelitis (n=8), bacteremia (n=3, all with <em>Salmonella</em> species) and UTI (n=3). All recovered uneventfully.</p> <p>Conclusion: It appears that in the current era, less than 10% of febrile children with sickle cell disease in our center are diagnosed with a SBI. Over an 11-year period, there were no sequelae or deaths from SBI. Given these excellent outcomes, outpatient ceftriaxone should be considered for febrile well appearing children with sickle cell disease if they have no apparent source and parents are judged to be reliable.</p> Manal Alsaif Joan Robinson Moshtag Abdulbaqi Mustafa Aghbari Khalid Al Noaim Muneera Alabdulqader Copyright (c) 2020 Manal Alsaif, Joan Robinson, Moshtag Abdulbaqi, Mustafa Aghbari, Khalid Al Noaim, Muneera Alabdulqader 2021-01-01 2021-01-01 13 1 e2021002 e2021002 10.4084/mjhid.2021.002 SOCIAL AND FINANCIAL BARRIERS TO OPTIMUM TKI TREATMENT IN PATIENTS WITH CHRONIC MYELOID LEUKEMIA- A KNOWLEDGE-ATTITUDES-PRACTICES STUDY FROM INDIA <p>Introduction: Outcomes in chronic myeloid leukemia (CML) have vastly improved after the introduction of tyrosine kinase inhibitors. However, patients in low and middle income countries face many challenges due to social and financial barriers.</p> <p>Objective: This study was conducted with the aim of understanding socio-economic hindrances and knowledge-attitudes-practices of patients of chronic phase CML who are taking imatinib.</p> <p>Materials and Methods: Patients of chronic phase CML, aged 15 and above, taking imatinib for 6 months or more were included in the study. A questionnaire (in Hindi language) was administered, enquiring about perceptions of nature of disease and its treatment, how imatinib was obtained, drug-taking behaviour, economic and social burden of the treatment.</p> <p>Results: Four hundred patients were recruited (median age 37 years, median duration on imatinib 63 months). Patients hailed from 16 different Indian states and 29.75% patients had to travel more than 500 kilometres for their hospital visit. Scheduled visits were missed by 14.75%. One third of the patients were unaware of the lifelong duration of treatment and 41.75% were unaware of the risks of discontinuing treatment. Treatment was financed by three different means- 61.75% received imatinib free of cost via the Glivec International Patient Assistance Program (GIPAP), 14.25% had treatment costs reimbursed by their employer, and 24% bore the cost of treatment themselves. Patients felt financially burdened due to cost of drugs (self-paying patients), cost of investigations, expenditure of the commute and stay for hospital visit, and loss of working days due to hospital visits.</p> <p>Conclusion: Cost of treatment, lack of widespread availability of hematology services and poor patient awareness are significant barriers to optimum treatment of CML in low and middle income countries.</p> Naveen Gupta Manoranjan Mahapatra Tulika Seth Seema Tyagi Sudha Sazawal Renu Saxena Copyright (c) 2020 Naveen Gupta, Manoranjan Mahapatra, Tulika Seth, Seema Tyagi, Sudha Sazawal, Renu Saxena 2021-01-01 2021-01-01 13 1 e2021004 e2021004 10.4084/mjhid.2021.004 DONOR KIR3DL1/RECEPTOR HLA-BW4-80I COMBINATION REDUCES ACUTE LEUKEMIA RELAPSE AFTER UMBILICAL CORD BLOOD TRANSPLANTATION WITHOUT IN VITRO T-CELL DEPLETION <p><em>Background:</em> Donor natural killer (NK) cell alloreactivity in umbilical cord bone marrow transplantation (UCBT) can lead to leukemic relapse. However, NK cell function is calibrated by interaction with human leukocyte antigens (HLAs). This study aimed to investigate graft-resistant leukemia after transplantation and compared specific genotypes of killer immunoglobulin-like receptors (KIRs) in donors and human leukocyte antigen ligands in patients.</p> <p><em>Methods:</em> We retrospectively analyzed 232 patients with acute leukemia from a single center. Patients had undergone UCBT with myeloablative conditioning and without anti-thymocyte globulin. We identified the KIR genotypes of cord blood donors using polymerase chain reaction with sequence-specific primers. All of the donors contained KIR3DL1.</p> <p><em>Results: </em>The patients were divided into three groups according to the HLA-B locus. The donor KIR3DL1 and recipient HLA-Bw4-80I combination was predictive of being highly educated, and was associated with a lower relapse (<em>P</em> = 0.006) and better overall survival (probability of relapse = 0.13, <em>P</em> &lt; 0.001) than the uneducated group. We found no significant increase in the incidence of acute or chronic graft-versus-host disease.</p> <p><em>Conclusions:</em> Our data suggest that the donor KIR3DL1/receptor and HLA-Bw4-80I combination in UCBT results in stronger graft-versus-leukemia effects and improved outcomes in patients with acute leukemia.</p> xinchen fang Zimin Sun Huilan Liu Xiang Wan Wen Yao Kaidi Song Kaidi Song Baolin Tang Xiaoyu zhu Jun Peng Copyright (c) 2020 xinchen fang, Xiaoyu zhu, Baolin Tang, Kaidi Song, Kaidi Song, Wen Yao, Xiang Wan, Huilan Liu, Zimin Sun, Jun Peng 2021-01-01 2021-01-01 13 1 e2021005 e2021005 10.4084/mjhid.2021.005 The INFLUENCE OF NUTRITION ON DISEASE SEVERITY AND HEALTH-RELATED QUALITY OF LIFE IN ADULTS WITH SICKLE CELL DISEASE <h3><strong><em>Background &amp; Aims:</em></strong> Sickle cell disease (SCD has a worldwide distribution and causes significant morbidity and mortality in children and adults. Few studies addressed the determinants of SCD severity in adults; therefore, we investigated the impact of nutrition on the outcome of SCD and health-related quality of life (HRQoL) in adult patients,</h3> <p><strong><em>Methods:</em></strong> In this longitudinal study, we recruited and prospectively followed 62 adults with SCD&nbsp; (aged ?18 years) for a &nbsp;median of 93 months. At entry and follow-up, patients provided <em>medical and dietary history</em>, &nbsp;had a physical examination and anthropometric measurements, assessment of protein-energy intake, measurement of micronutrient levels, estimation of&nbsp; SCD severity score, and determination of the HRQoL &nbsp;(SF-26v2). The study outcome was a composite of hospitalization due to SCD crises or death.</p> <p><strong><em>Results:</em></strong> At baseline, 42 (67.74%) patients had macro and, or micro-undernutrition (Group A), and 20 (32.26%) were well nourished. (Group B). &nbsp;The&nbsp;&nbsp; BMI and most anthropometric measurements were significantly lower in SCD patients compared to control subjects. &nbsp;In several SCD patients, undernutrition resulted from reduced food intake and reduced absorption due to concomitant gastrointestinal disorders. Seventy percent of&nbsp; SCD patients had vitamin D,&nbsp; vitamin B12, and zinc deficiencies. Under-nourished patients had significantly higher SCD severity scores, frequent SCD related hospitalizations, higher mortality rates, and reduced HRQoL compared to well-nourished patients.&nbsp; Protein-energy and micronutrient deficiencies were independent predictors of severe SCD and mortality. Correction of undernutrition and hydroxyurea therapy improved SCD severity scores and HRQoL.</p> <p><strong><em>Conclusion:</em></strong> Patients with sickle cell disease have various degrees of macro and micro deficiencies, which increase SCD severity and hospitalizations and reduce the health-related quality of life. Early diagnosis and prompt correction of macro and micronutrient deficiencies need to be incorporated in the standard of care of SCD patients to improve the disease outcomes.&nbsp;</p> Sanaa Kamal Moheyeldeen Mohamed Naghib Jamaan Al Zahrani Huda Hassan Karim Abdel Aziz Moawado Omar Arrahman Copyright (c) 2020 Sanaa Kamal, Moheyeldeen Mohamed Naghib, Jamaan Al Zahrani, Huda Hassan, Karim Abdel Aziz Moawado, Omar Arrahman 2021-01-01 2021-01-01 13 1 e2021007 e2021007 10.4084/mjhid.2021.007 SIMPLE PERIPHERAL BLOOD SMEAR FINDINGS OF COVID-19 PATIENTS PROVIDE INFORMATION ABOUT THE SEVERITY OF THE DISEASE AND THE DURATION OF HOSPITAL STAY. <p><strong>Backround</strong></p> <p>Data about the morphological changes of Covid-19 infection in peripheral blood smear are limited and association with clinical severity of the disease are not known yet. We aimed to examine the characteristics of the cells detected in the pathological rate and / or appearance and whether these findings are related to the clinical course by evaluating the peripheral blood smear at the time of diagnosis in Covid-19 patients.</p> <p><strong>Methods</strong></p> <p>Clinical features, laboratory data, peripheral blood smear of fifty patients diagnosed with Covid-19 by PCR was evaluated at diagnosis. Peripheral smear samples of the patients were compared with the age and sex matched 30 healthy controls. Pictures were taken from the paitients’peripheral blood smear. Patients were divided into two groups. &nbsp;Early and advanced stage patient groups were compared in terms of laboratory data and peripheral smear findings. The relationship between the laboratory values of all patients and the duration of hospitalization was analyzed<strong>.</strong></p> <p><strong>Results</strong></p> <p>Pseudo pelger-huet, atypical lymphocytes, vacuole monocytes and pycnotic neutrophils rates were high in the patient group. Increased pseudo pelger-huet anomaly, psodo-pelger huet/mature lymphocyte ratio, decreased number of mature lymphocytes, and eosinophils in peripheral blood smear were observed in the advanced stage patients (p &lt;0.05). A negative correlation was observed between the duration of hospitalization and mature lymphocyte, and monocytes with vacuoles rates (p &lt;0.05).</p> <p><strong>Conclusion</strong></p> <p>Peripheral smear is a cheap, easily performed, and rapid test. Increased pseudo-pelger huet anomaly/mature lymphocytes rate is suggesting advanced stage disease, while high initial monocytes with vacuoles and mature lymphocyte rates at the time of diagnosis may be an indicator of shortened duration of hospitalization.</p> Ilhami Berber Ozlem Cagasar Ahmet Sarıcı Nurcan Kirici Berber Ismet Aydogdu Asli Yildirim Harika Gozde Gocukara Bag Leman Acun Delen Ozkan Ulutas Copyright (c) 2020 Ilhami Berber, Ozlem Cagasar, Ahmet Sar?c?, Nurcan Kirici Berber, Ismet Aydogdu, Asli Yildirim, Harika Gozde Gocukara Bag, Leman Acun Delen, Ozkan Ulutas 2021-01-01 2021-01-01 13 1 e2021009 e2021009 10.4084/mjhid.2021.009 HEMATOLOGY PATIENT PROTECTION DURING THE COVID-19 PANDEMIC IN ITALY: A NATIONWIDE NURSING SURVEY <p><strong>Background:</strong> Italy has been one of the first European countries hit by the COVID-19 pandemic, with many patients dying from severe respiratory issues, especially frail subjects. Hematology patients are generally thought to be at high risk of developing severe COVID-19-associated complications. The aim of this work was to describe the infection control measures adopted in Italian hematology settings to protect patients and healthcare professionals.</p> <p><strong>Materials and Methods:</strong> On behalf of the Nursing Campus in Hematology Group, a nationwide nursing survey was conducted. Questionnaire items included general information, infection control measures, patient and healthcare professional protection, information management, and participants opinion on key issues. Data have been analyzed by center location (Northern, Central or Southern Italy) and by patient age (adult vs pediatric).</p> <p><strong>Results:</strong> Forty-four Italian hematology centers participated, representing 52.4% of those invited. Patients underwent nasopharyngeal swabs (93.2%) generally the day before admission (43.2%), though less frequently in southern centers (p = 0.0377). Visitor restrictions were implemented in all centers: 65.9% barred all visitors, while 25.0% allowed visitors only for patients with specific conditions, especially in central Italy. Deficiency of personal protective equipment, including masks (45.5%) and gloves (22.7%), was reported, although the nurses’ opinion was that the emergency was nevertheless well managed in terms of protecting patients and professionals. Almost all healthcare institutions (97.7%) provided recommendations on emergency management. No significant differences were found between adult and pediatric centers in terms of infection prevention and control.</p> <p><strong>Discussion:</strong>. Low variability in patient protection strategies was observed, meaning that national recommendations were effective. However, some critical issues emerged regarding the management of infected healthcare professionals and their contacts.</p> Stefano Botti Nicola Serra Fausto Castagnetti Sabina Chiaretti Nicola Mordini Gianpaolo Gargiulo Laura Orlando Copyright (c) 2020 Stefano Botti, Nicola Serra, Fausto Castagnetti, Sabina Chiaretti, Nicola Mordini, Gianpaolo Gargiulo, Laura Orlando 2021-01-01 2021-01-01 13 1 e2021011 e2021011 10.4084/mjhid.2021.011 REAL-WORLD OUTCOME IN THE PRE-CAR-T ERA OF MYELOMA PATIENTS QUALIFYING FOR CAR-T CELL THERAPY <p><strong>Introduction: </strong>CAR-T cell therapy is likely to be introduced starting from 2021 in patients with relapsed/refractory myeloma (r/r MM) in Europe. In order to qualify for commercial CAR-T treatment, it is assumed that r/r MM patients will have to be exposed to at least three lines of previous treatments including lenalidomide, bortezomib and anti-CD38 treatment. However, the outcome of this particular subgroup of r/r MM patients is largely unknown whereas this knowledge is crucial to estimate the possible benefit of eventual CAR-T treatment.</p> <p><strong>Methods:</strong> In this non-interventional, retrospective single-center study, we analyzed all subsequent r/r MM patients treated between 01/2016 (when anti-CD38 treatment was commercially introduced in Switzerland) and 04/2020 at the University Hospital of Bern. Patients were eligible for the study if they had received at least three lines of treatment including one proteasome inhibitor (PI), one immunomodulatory drug (IMID) and one anti-CD38 antibody, and if they were in need of subsequent treatment and effectively received further lines of treatment.</p> <p><strong>Results:</strong> Among 56 patients fulfilling the criteria of at least three lines of treatment including PI, IMID and anti-CD38 treatment, only 34 (60%) effectively received subsequent further therapy. This suggests that 40% of r/r MM patients never receive additional treatment after at least three lines of treatment including PI, IMID and anti-CD38 treatment. For patients receiving further treatment, the median number of previous lines of treatment was 4.5 (range 2-12), including autologous stem cell transplantation in 31 (91%) patients. 13 (37%) patients were penta-refractory. The most frequently used treatment options were IMID/dexamethasone treatment in 11 (32%) patients, followed by PI/dexamethasone in 10 (29%) patients. 21 (62%) patients received two or more additional lines of therapy. The median PFS was 6.6 months (range 0–36.6 months), the median TTNT was 7.5 months (range 1.4-24.5 months) and the median OS was 13.5 months, (range 0.1-38 months) for the first subsequent treatment. The overall response rate (ORR) to the first subsequent treatment was 41%, with a median duration of the response of 5 months (range 1-37 months). 12% of the patients achieved VGPR or better, with a median duration of response of 8 months (range 3-37 months).</p> <p><strong>Conclusion: </strong>Myeloma patients refractory after at least three lines of anti-CD38/PI/IMID treatment have a poor prognosis with a PFS of 6.6 months and OS of 13.5 months. These data may serve as reference to compare the potential benefit of CAR-T treatment in this group of myeloma patients when available in the near future.</p> Ulrike Bacher Simon Brechbühl Barbara Jeker Thomas Pabst Copyright (c) 2020 Ulrike Bacher, Simon Brechbühl, Barbara Jeker, Thomas Pabst 2021-01-01 2021-01-01 13 1 e2021012 e2021012 10.4084/mjhid.2021.012 THE IMPORTANCE OF TARGETED NEXT-GENERATION SEQUENCING USAGE IN CYTOGENETICALLY NORMAL MYELOID MALIGNANCIES <p>Advanced diagnostic methods give an advantage for the identification of the abnormalities in myeloid malignancies. Various researchers have shown the potential importance of genetic tests both before the onset of the disease and during the remission. Large testing panels prevents false negative results in myeloid malignancies. But the important question is how the results of conventional cytogenetic and molecular cytogenetic techniques can be merged together with NGS technologies. In this paper, we drew an algorithm for evaluation of the myeloid malignancies. In order to evaluate genetic abnormalities, we performed cytogenetics, molecular cytogenetics and NGS testing in hematologic malignancies. In this study, we analyzed 100 patients who admitted to Medical Genetics Laboratory within different type of myeloid malignancies. We highlighted the possible diagnostic algorithm for cytogenetically normal cases. We applied NGS 141 gene panel for cytogenetically normal patients and we detected two or more pathogenic variations in 61 out of 100 patients (61%). The pathogenic variation detection rate of NGS varies in disease groups: AML were 85% and MDS were 23%. Here, we identified 24 novel variation out of total pathogenic variations in myeloid malignancies. A total 18 novel variation were identified in AML and 6 novel variation were identified in MDS. Despite of long turnaround time, conventional techniques are still golden standard for myeloid malignancies but sometimes cryptic gene fusions or complex abnormalities cannot be identified easily by conventional techniques. In these conditions, advanced technologies like NGS are highly recommended.</p> Emine Atli Rasime Kalkan Cisem Mail Damla Eker Ufuk Demirci Selma Demir Sinem Yalcintepe Hakki Onur Kirkizlar Engin Atli Hakan Gurkan Ahmet Muzaffer Demir Copyright (c) 2020 Emine Atli, Hakan Gurkan, Engin Atli, Hakki Onur Kirkizlar, Sinem Yalcintepe, Selma Demir, Ufuk Demirci, Damla Eker, Cisem Mail, Rasime Kalkan, Ahmet Muzaffer Demir 2021-01-01 2021-01-01 13 1 e2021013 e2021013 10.4084/mjhid.2021.013 ELEVATED SERUM PENTRAXIN-3 LEVELS IS POSITIVELY CORRELATED TO DISEASE SEVERITY AND COAGULOPATHY IN COVID-19 PATIENTS <p><strong>Abstract</strong></p> <p><strong>BACKGROUND</strong> Coronavirus disease 2019 (COVID-19) is highly contagious and deadly and is associated with coagulopathy. Pentraxin-3(PTX3) participates in innate resistance to infections and plays a role in thrombogenesis.</p> <p><strong>PURPOSE</strong> The present study aimed to investigate the role of PTX3 in coagulopathy in patients with COVID-19.</p> <p><strong>METHODS </strong>A retrospective study including thirty-nine COVID-19 patients enrolled in Hunan, China were performed. The patients were classified into the D-dimer_L (D-dimer?1mg/L) and D-dimer_H (D-dimer?1mg/L) groups basing on the plasma D-dimer levels on admission. Serum PTX3 levels were detected by enzyme-linked immunosorbent assays and compared between those two groups, and then linear regression models were applied to analyze the association between PTX3 and D-dimer.</p> <p><strong>RESULTS</strong> Our results showed that serum PTX3 levels (median values, 10.21 vs 3.36, <em>P</em> &lt; 0.001), chest computerized tomography scores (median values, 10.0 vs 9.0, <em>P</em> &lt; 0.05), and length of stay (16.0±4.2 vs 10.7±3.6, <em>P </em>= 0.001) in the D-dimer_H group were significantly higher than that in D-dimer_L group. The coefficient of determination for PTX3 was 0.651 (<em>P</em> &lt; 0.001) in the D-dimer_H group.</p> <p><strong>CONCLUSION</strong> Serum level of PTX3 was positively correlated with disease severity and coagulopathy. Detection of serum PTX3 level could assist to identify severer patients on admission and may be a potential therapeutic target for coagulopathy in patients with COVID-19.</p> Ming Tong Fang Chen Xiaolin Xiao Lianhong Zou Yu Jiang Changping Hu Qing Zheng Hong Xu Chen Zhu Ying Xiong Yimin Zhu Copyright (c) 2020 Ming Tong, Ying Xiong, Chen Zhu, Hong Xu, Qing Zheng, Changping Hu, Yu Jiang, Lianhong Zou, Xiaolin Xiao, Fang Chen, Yimin Zhu 2021-01-01 2021-01-01 13 1 e2021015 e2021015 10.4084/mjhid.2021.015 Worldwide distribution of prekallikrein (PK) deficiency: the defect seems mainly concentrated in West African countries and in the United States Antonio Girolami Silvia Ferrari Copyright (c) 2020 Antonio Girolami, Silvia Ferrari 2021-01-01 2021-01-01 13 1 e2021014 e2021014 10.4084/mjhid.2021.014