Advances in HematologyPub Date : 2020-01-27eCollection Date: 2020-01-01DOI: 10.1155/2020/7636104
Mark Terence P Mujer, Manoj P Rai, Varunsiri Atti, Ian Limuel Dimaandal, Abigail S Chan, Shiva Shrotriya, Krishna Gundabolu, Prajwal Dhakal
{"title":"An Update on the Reversal of Non-Vitamin K Antagonist Oral Anticoagulants.","authors":"Mark Terence P Mujer, Manoj P Rai, Varunsiri Atti, Ian Limuel Dimaandal, Abigail S Chan, Shiva Shrotriya, Krishna Gundabolu, Prajwal Dhakal","doi":"10.1155/2020/7636104","DOIUrl":"https://doi.org/10.1155/2020/7636104","url":null,"abstract":"<p><p>Non-vitamin K antagonist oral anticoagulants (NOACs) include thrombin inhibitor dabigatran and coagulation factor Xa inhibitors rivaroxaban, apixaban, edoxaban, and betrixaban. NOACs have several benefits over warfarin, including faster time to the achieve effect, rapid onset of action, fewer documented food and drug interactions, lack of need for routine INR monitoring, and improved patient satisfaction. Local hemostatic measures, supportive care, and withholding the next NOAC dose are usually sufficient to achieve hemostasis among patients presenting with minor bleeding. The administration of reversal agents should be considered in patients on NOAC's with major bleeding manifestations (life-threatening bleeding, or major uncontrolled bleeding), or those who require rapid anticoagulant reversal for an emergent surgical procedure. The Food and Drug Administration (FDA) has approved two reversal agents for NOACs: idarucizumab for dabigatran and andexanet alfa for apixaban and rivaroxaban. The American College of Cardiology (ACC), American Heart Association (AHA), and Heart Rhythm Society (HRS) have released an updated guideline for the management of patients with atrial fibrillation that provides indications for the use of these reversal agents. In addition, the final results of the ANNEXA-4 study that evaluated the efficacy and safety of andexanet alfa were recently published. Several agents are in different phases of clinical trials, and among them, ciraparantag has shown promising results. However, their higher cost and limited availability remains a concern. Here, we provide a brief review of the available reversal agents for NOACs (nonspecific and specific), recent updates on reversal strategies, lab parameters (including point-of-care tests), NOAC resumption, and agents in development.</p>","PeriodicalId":7325,"journal":{"name":"Advances in Hematology","volume":"2020 ","pages":"7636104"},"PeriodicalIF":0.0,"publicationDate":"2020-01-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2020/7636104","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37787285","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Advances in HematologyPub Date : 2019-12-28eCollection Date: 2019-01-01DOI: 10.1155/2019/6045214
Suthan Pandarakutty, Kamala Murali, Judie Arulappan, Deepa Shaji Thomas
{"title":"Effectiveness of Nurse Led Intervention on Health Related Quality of Life among Children with Sickle Cell Disease in Oman: A Pilot Study.","authors":"Suthan Pandarakutty, Kamala Murali, Judie Arulappan, Deepa Shaji Thomas","doi":"10.1155/2019/6045214","DOIUrl":"https://doi.org/10.1155/2019/6045214","url":null,"abstract":"<p><p><i>Introduction.</i> The children with Sickle Cell Disease (SCD) generally have poor Health Related Quality of Life (HRQOL). The study aimed to evaluate the effectiveness of nurse led intervention on HRQOL among children with SCD.</p><p><strong>Methods: </strong>A total of 30 samples were selected using convenient sampling. Children with SCD and their caregivers completed Pediatric Quality of Life Inventory (PedsQL) SCD-Module version 3.0. The nurse led intervention was given to the study group for 10 consecutive weeks. The control group received the routine medical care. On completion of 10 weeks, the post-test was conducted.</p><p><strong>Results: </strong>The participants in study group had poor HRQOL scores (<i>P</i> > 0.05) in pre-test. After nurse led intervention, the HRQOL score in the study group significantly improved (<i>P</i> > 0.05) in pre-test. After nurse led intervention, the HRQOL score in the study group significantly improved (<i>Discussion.</i> Therefore nurse led intervention is effective in improving HRQOL among children with SCD.</p>","PeriodicalId":7325,"journal":{"name":"Advances in Hematology","volume":"2019 ","pages":"6045214"},"PeriodicalIF":0.0,"publicationDate":"2019-12-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2019/6045214","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37669089","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Advances in HematologyPub Date : 2019-12-18eCollection Date: 2019-01-01DOI: 10.1155/2019/2352919
M A ElBaiomy, S Aref, M El Zaafarany, Sara Atwa, Tamer Akl, Wafaa El-Beshbishi, Shaimaa El-Ashwah, L Ibrahim, M El-Ghonemy
{"title":"Prognostic Impact of Lymphoid Enhancer Factor 1 Expression and Serum Galectin.3 in Egyptian AML Patients.","authors":"M A ElBaiomy, S Aref, M El Zaafarany, Sara Atwa, Tamer Akl, Wafaa El-Beshbishi, Shaimaa El-Ashwah, L Ibrahim, M El-Ghonemy","doi":"10.1155/2019/2352919","DOIUrl":"https://doi.org/10.1155/2019/2352919","url":null,"abstract":"<p><strong>Background: </strong>Deregulation of the Wnt signaling pathway had a role in haematological malignancies. Previous studies reported that lymphoid enhancer factor 1 (LEF1) expression and serum Galectin-3 level could affect clinical parameters and outcome in acute myeloid leukemia patients, but as far as we know, no study has addressed their combined effect on AML patients.</p><p><strong>Aim: </strong>We studied the expression of LEF1 by real-time qPCR and measured serum level of Gal.3 by ELISA technique in peripheral blood of 69 AML patients and correlated it with different clinicopathological criteria of patients, response, PFS and OS.</p><p><strong>Results: </strong>We found high expression (LEF1<sup>high</sup>) was associated with better OS (<i>p</i> = 0.02) and EFS (<i>p</i> = 0.019) compared to LEF1<sup>low</sup>, low serum Gal.3 level had better OS (<i>p</i> = 0.014) and EFS (<i>p</i> = 0.02) compared to high serum Gal.3 level. LEF1<sup>high</sup> less likely to carry a FLT3-ITD (<i>p</i> = 0.047) compared to LEF1<sup>low</sup> patient, also LEF1<sup>high</sup> characterized by favorable risk (<i>p</i> = 0.02) than LEF1<sup>low</sup> patients. While patients with higher Gal-3 levels characterized by poor risk (<i>p</i> = 0.02) than lower Gal.3 lels, also more likely to carry a FLT3-ITD with borderline significance (<i>p</i> = 0.054). Combined LEF1<sup>high</sup>/Gal.3 low patients had lower baseline blast percentages (<i>p</i> = 0.02), favorable risk (<i>p</i> = 0.01), less likely to carry FLT3-ITD (<i>p</i> = 0.02), higher CR rate (<i>p</i> = 0.055), shorter time to CR (0.001) than other groups. Among high Gal.3 level group, LEF1<sup>high</sup>expression improved OS and EFS (20 and 15 months respectively) vs LEF1<sup>low</sup> expression (13 and 8 months respectively).</p><p><strong>Conclusion: </strong>We conclude that high LEF1 expression was a favorable prognostic marker which can define AML patient risk and outcome independent from assessing the serum galectin.3 level.</p>","PeriodicalId":7325,"journal":{"name":"Advances in Hematology","volume":"2019 ","pages":"2352919"},"PeriodicalIF":0.0,"publicationDate":"2019-12-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2019/2352919","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37535562","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
A. Abu Taha, Ahmad Yaseen, S. Suleiman, Omar Abu Zenah, Hammam Ali, R. Abu Seir, Khaled Younis
{"title":"Study of Frequency and Characteristics of Red Blood Cell Alloimmunization in Thalassemic Patients: Multicenter Study from Palestine","authors":"A. Abu Taha, Ahmad Yaseen, S. Suleiman, Omar Abu Zenah, Hammam Ali, R. Abu Seir, Khaled Younis","doi":"10.1155/2019/3295786","DOIUrl":"https://doi.org/10.1155/2019/3295786","url":null,"abstract":"Background. β-Thalassemia is a common inherited hemolytic disorder in Palestine. Red blood cell (RBC) transfusion is the principal treatment but it may cause RBC alloimmunization. This study was conducted to determine the prevalence and characteristics of RBC alloimmunization among thalassemic patients in northern governorates of Palestine. Methods. A prospective multicenter observational study was conducted in the thalassemia transfusion centers in the northern governorates of Palestine. The study included 215 thalassemia patients who received regular blood transfusions. Clinical and transfusion records of patients were examined. Antibody screening and identification was conducted using the microcolum gel technique. Results. Two hundred fifteen patients were included in the study. More than half (52.1%) of the patients were males. The median age of patients was 18 years (range: 12–24 years). The most frequent blood group was A (40.5%). Alloantibodies were detected in 12.6% of patients. Anti-D (33.3%), anti-K (25.9%) and anti-E (14.8%) were the most commonly isolated antibodies. There was no association between age, sex, starting age of transfusion, number of transfused units, history of splenectomy and alloimmunization. Conclusions. Anti-Rh and anti-K antibodies were common among this cohort of patients. Age, sex, starting age of transfusion, number of transfused units, and history of splenectomy could not predict the occurrence of alloimmunization.","PeriodicalId":7325,"journal":{"name":"Advances in Hematology","volume":"2019 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-11-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2019/3295786","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42005935","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
A. Bouchla, T. Thomopoulos, S. Papageorgiou, P. Tsirigotis, E. Bazani, K. Gkirkas, Diamantina Vasilatou, Eirini Glezou, Georgia Stavroulaki, Konstantinos Gkontopoulos, G. Dimitriadis, V. Pappa
{"title":"Coexistence of Myeloid and Lymphoid Neoplasms: A Single-Center Experience","authors":"A. Bouchla, T. Thomopoulos, S. Papageorgiou, P. Tsirigotis, E. Bazani, K. Gkirkas, Diamantina Vasilatou, Eirini Glezou, Georgia Stavroulaki, Konstantinos Gkontopoulos, G. Dimitriadis, V. Pappa","doi":"10.1155/2019/1486476","DOIUrl":"https://doi.org/10.1155/2019/1486476","url":null,"abstract":"The coexistence of a myeloid and a lymphoid neoplasm in the same patient is a rare finding. We retrospectively searched the records of the Hematology Division of the Second Department of Internal Medicine and Research Institute at Attikon University General Hospital of Athens from 2003 to 2018. Nine cases have been identified in a total of 244 BCR-/ABL1- negative MPN and 25 MDS/MPN patients and 1062 LPD patients referred to our institution between 2003 and 2018. Each case is distinct in the diversity of myeloid and lymphoid entities, the chronological occurrence of the two neoplasms, and the patient clinical course. All of them exhibit myeloproliferative (6 JAK2 V617F-positive cases) and lymphoproliferative features, with 1 monoclonal B-cell lymphocytosis (MBL), 3 B-chronic lymphocytic leukemias (B-CLL), 3 B-non-Hodgkin lymphomas (B-NHL), 1 multiple myeloma (MM), and 1 light and heavy deposition disease (LHCDD), while in three cases myelodysplasia is also present. The challenges in identifying and dealing with these rare situations in everyday clinical practice are depicted in this article.","PeriodicalId":7325,"journal":{"name":"Advances in Hematology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-11-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2019/1486476","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43302172","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Prevalence of Anemia and Associated Factors among Newly Diagnosed Patients with Solid Malignancy at Tikur Anbessa Specialized Hospital, Radiotherapy Center, Addis Ababa, Ethiopia","authors":"Edosa Kifle Tola, Mintewab Hussein, Jemal Alemu, Wondemagegnhu Tigeneh","doi":"10.1155/2019/8279789","DOIUrl":"https://doi.org/10.1155/2019/8279789","url":null,"abstract":"Background Anemia is a common finding in cancer, which is caused by many factors. It is a major cause of morbidity in cancer patients, worsens disease status and impairs treatment outcome; however, little is known about the prevalence of anemia and associated factors among cancer patients during diagnosis in developing countries like Ethiopia. In response to this, we have conducted research with the aim of assessing the prevalence of anemia and associated factors among newly diagnosed patients with solid malignancy at Tikur Anbessa Specialized Hospital (TASH), Radiotherapy center, Addis Ababa, Ethiopia. Methods Descriptive cross-sectional study was conducted from April to May 2014. A total of 422 newly diagnosed patients with solid malignancy attending Radiotherapy center, TASH were enrolled to assess anemia prevalence and associated factors. Data were coded, entered and analyzed using SPSS version16. Using logistic regression, chi squares, Odds ratio and 95% confidence intervals were computed to measure strength of association between variables. p-value < 0.05 was taken as statistically significant. Result Out of 422 respondents, 285 (68%) were females and 153 (36%) of respondents fell into 35–49 age group with age range between 18 and 80 years and the median age of 45. Magnitude of solid cancers was gynecologic (28.9%), breast (22.7%), nasopharyngeal carcinoma (NPC) (7.6%), colorectal (7.1%), sarcoma (6.9%), head and neck (4.5%), thyroid (3.3%), hepatoma (1.9%), and others (17.1%). The overall prevalence of anemia across different tumor was 23% and higher anemia prevalence was noted in gynecologic (37.7%) and colorectal carcinomas (26.7%). The majority of the anemic patients (68%) remained untreated for anemia. The mean trigger hemoglobin for transfusion was 7.7 g/dl. About 83.5% of anemia was mild to moderate type. Performance status (AOR = 3.344; 95% CI 1.410–7.927) and bleeding history (AOR = 3.628; 95% CI 1.800–7.314) showed statistically significant association with occurrence of anemia with p-value < 0.05. Conclusion Among solid cancers, gynecologic cancer remained the dominant one. Anemia prevalence was 23% in general, in which gynecologic and colorectal cancers were more prevalent. ECOG performance status and bleeding history showed a statistically significant association with the occurrence of anemia.","PeriodicalId":7325,"journal":{"name":"Advances in Hematology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-10-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2019/8279789","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47347897","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
M. Mingot-Castellano, M. A. Román, Luis Fernando Fernández Fuertes, T. González‐López, J. M. Guinea de Castro, I. Jarque, M. López-Fernández, M. Lozano, B. Sánchez González, David Valcárcel Ferreiras, J. R. González Porras
{"title":"Management of Adult Patients with Primary Immune Thrombocytopenia (ITP) in Clinical Practice: A Consensus Approach of the Spanish ITP Expert Group","authors":"M. Mingot-Castellano, M. A. Román, Luis Fernando Fernández Fuertes, T. González‐López, J. M. Guinea de Castro, I. Jarque, M. López-Fernández, M. Lozano, B. Sánchez González, David Valcárcel Ferreiras, J. R. González Porras","doi":"10.1155/2019/4621416","DOIUrl":"https://doi.org/10.1155/2019/4621416","url":null,"abstract":"Background and Objective Diagnosis and management of primary immune thrombocytopenia (ITP) have changed dramatically in the last decade. The aim of the study was to obtain information about the opinion of the Spanish ITP Group (GEPTI) members regarding the best clinical practices for diagnosis and management of adult patients with ITP. Materials and Methods A two-round Delphi method was carried out by sending to 129 experts a 90-item questionnaire developed by 11 specialists, with a 4-point Likert scale (“never,” “sometimes,” “frequently,” and “always”) for the assessment of responses. Results Forty out of the 129 experts participated in the survey (participation rate 30.2%) and 39 completed the questionnaire (response rate 97.5%). Salient consensus points included the following: the need to indicate workup studies from a sustained platelet count < 100 x 109/L in the absence of a clear etiology; bone marrow aspiration in elderly patients with suspected ITP; beginning treatment in asymptomatic patients with a platelet count < 20 x 109/L; not exceeding 6-7 weeks of corticosteroid therapy; switching from corticosteroids to one thrombopoietin receptor agonist (TRA); switching to other TRA or other options as combinations of them with immunosuppressive drugs in case of failure; how to reduce tapering TRA; treating patients with symptomatic persistent ITP and platelet count > 20 x 109/L; and considering mucosal or severe bleeding as a basic criterion for hospital admission. Conclusions The present consensus document provides a reference framework for the management of patients with ITP in clinical practice.","PeriodicalId":7325,"journal":{"name":"Advances in Hematology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-08-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2019/4621416","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47222195","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Paola Villafuerte-Gutiérrez, L. Villalón, J. Losa, C. Henriquez-Camacho
{"title":"Corrigendum to “Treatment of Febrile Neutropenia and Prophylaxis in Hematologic Malignancies: A Critical Review and Update”","authors":"Paola Villafuerte-Gutiérrez, L. Villalón, J. Losa, C. Henriquez-Camacho","doi":"10.1155/2019/4120631","DOIUrl":"https://doi.org/10.1155/2019/4120631","url":null,"abstract":"[This corrects the article DOI: 10.1155/2014/986938.].","PeriodicalId":7325,"journal":{"name":"Advances in Hematology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-07-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2019/4120631","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48399244","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Advances in HematologyPub Date : 2019-05-02eCollection Date: 2019-01-01DOI: 10.1155/2019/3961201
Heba M Al-Qattan, Dana F Amlih, Fatima S Sirajuddin, Dalal I Alhuzaimi, Mai S Alageel, Reema M Bin Tuwaim, Farjah H Al Qahtani
{"title":"Quantifying the Levels of Knowledge, Attitude, and Practice Associated with Sickle Cell Disease and Premarital Genetic Counseling in 350 Saudi Adults.","authors":"Heba M Al-Qattan, Dana F Amlih, Fatima S Sirajuddin, Dalal I Alhuzaimi, Mai S Alageel, Reema M Bin Tuwaim, Farjah H Al Qahtani","doi":"10.1155/2019/3961201","DOIUrl":"10.1155/2019/3961201","url":null,"abstract":"<p><p>Our study aims to observe the levels of knowledge, attitude, and practice (KAP) associated with sickle cell disease (SCD) and premarital genetic counseling (PMGC) in 351 Saudi adults. The relationships between KAP levels and sociodemographic characteristics (age, gender, marital status, and educational level) were observed. The study was conducted in King Khalid University Hospital between February 21, 2017, and March 7, 2018. A total of 351 Saudi participants attending the primary care clinic were selected using convenience sampling and were given a self-administered questionnaire. Overall, the 351 participants had the best attitude (41% scoring \"good\"), followed by knowledge (28.8%), and, lastly, practice (19.1%). Out of the sociodemographic characteristics, age group was the most statistically significant in all the three categories (knowledge, attitude, and practice). The > 50-year age group performed the worst in all the three categories. Despite the advancements in public healthcare measures in Saudi Arabia, our study revealed that there are still many gaps to be filled regarding the knowledge, attitude, and practice associated with SCD and PMGC.</p>","PeriodicalId":7325,"journal":{"name":"Advances in Hematology","volume":"2019 ","pages":"3961201"},"PeriodicalIF":0.0,"publicationDate":"2019-05-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6521475/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37321018","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Advances in HematologyPub Date : 2019-04-01eCollection Date: 2019-01-01DOI: 10.1155/2019/1783240
Yvonne A Dei-Adomakoh, Jane S Afriyie-Mensah, Audrey Forson, Martin Adadey, Thomas A Ndanu, Joseph K Acquaye
{"title":"Lung Function Abnormalities in Sickle Cell Anaemia.","authors":"Yvonne A Dei-Adomakoh, Jane S Afriyie-Mensah, Audrey Forson, Martin Adadey, Thomas A Ndanu, Joseph K Acquaye","doi":"10.1155/2019/1783240","DOIUrl":"https://doi.org/10.1155/2019/1783240","url":null,"abstract":"<p><strong>Background: </strong>Abnormalities in lung function tests have been shown to commonly occur in a majority of patients with sickle cell disease (SCD) even at steady state. The prevalence and pattern of these lung function abnormalities have been described in other populations but this is unknown among our sickle cell cohort. There is generally little information available on risk factors associated with the lung function abnormalities and its relevance in patient care.</p><p><strong>Method: </strong>This was an analytical cross-sectional study involving 76 clinically stable, hydroxyurea-naive adult Hb-SS participants and 76 nonsickle cell disease (non-SCD) controls. A structured questionnaire was used to obtain sociodemographic data and clinical history of the participants. Investigations performed included spirometry, pulse oximetry, tricuspid regurgitant jet velocity (TRV) measurements via echocardiogram, complete blood counts, free plasma haemoglobin, serum urea, and creatinine.</p><p><strong>Results: </strong>Weight, BMI, mean FVC, and FEV1% predicted values were comparatively lower among the Hb-SS patients (p < 0.001). Abnormal spirometry outcome occurred in 70.4% of Hb-SS patients, predominantly restrictive defects (p < 0.001), and showed no significant association with steady-state Hb, WBC count, free plasma haemoglobin, frequency of sickling crisis, chronic leg ulcers, and TRV measurements (p > 0.05). The mean oxygen saturation was comparatively lower among Hb-SS patients (p < 0.001).</p><p><strong>Conclusion: </strong>Measured lung volumes were significantly lower in Hb-SS patients when compared to non-SCD controls and this difference was not influenced by anthropometric variance. Lung function abnormalities, particularly restrictive defects, are prevalent in Hb-SS patients but showed no significant association with recognized markers of disease severity.</p>","PeriodicalId":7325,"journal":{"name":"Advances in Hematology","volume":"2019 ","pages":"1783240"},"PeriodicalIF":0.0,"publicationDate":"2019-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2019/1783240","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37377084","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}