Turkish Journal of Hematology Volume: 37 Issue: 1 / 2020

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LETTERS TO THE EDITOR Turk J Hematol 2020;37:57-76Table 2. Bleeding scores positivity regarding each symptom invon Willebrand type 1 and type 3 patients.Bleeding symptomVWDtype 1§VWDtype 3*Epistaxis 22% 100% 0.000Cutaneous bleeding 3.7% 62.5% 0.000Minor bleeding 5.6% 75% 0.000Oral cavity bleeding 5.6% 75% 0.000Bleeding after tooth extraction 22.2% 37.5% 0.295Intramuscular bleeding 3.7% 37.5% 0.013Hemarthrosis 3.7% 25% 0.077Menorrhagia** 41.3% 50% 0.506Central nervous system bleeding 1.9% 12.5% 0.243Umbilical bleeding 1.9% 25% 0.041Postsurgical bleeding 1.7% 0% 0.757Cephalhematoma 0% 12.5% 0.129Macroscopic hematuria 3.7% 12.5% 0.344§ von Willebrand type 1 group (n=54). * von Willebrand type 3 group (n=8).Epistaxis, superficial bleedings, bleeding from minor wounds, oral bleeding, umbilicalbleeding, muscle hematoma, and hemarthrosis were found to be statisticallysignificant in showing dependence between diagnosis status (VWD types 1-3) andbleeding symptoms (p<0.05).**Menorrhagia symptom was compared among female patients in both groups.VWD: von Willebrand diseasebe lower compared to those in the previous literature [4,5,8].This may be explained by the inclusion of “low VWF levels”(intermediate levels of VWF:Ag, 30-50 IU/dL) in the group withtype 1 VWD. People with “low VWF levels” falsely labeled as“VWD type 1 patients” may have lower reported bleeding scorescompared to true VWD patients, leading to low positive scores.Our study shows that the ISTH/BAT and PBQ can be useful inthe evaluation of the bleeding symptoms of patients. Furtherstudies with larger patient and control groups are warrantedto show the usage of bleeding scores in daily outpatientpractice. We, as the mentees of the Hemophilia Masterclass, feelmuch appreciation to our mentors and the Turkish Society ofHematology for their contributions to our progress in the fieldof hemophilia.Keywords: Von Willebrand Disease, Pediatric BleedingQuestionnaire, ISTH/BAT scoreAnahtar Sözcükler: Von Willebrand Hastalığı, Pediatrik KanamaSkoru, ISTH/BAT skorupInformed Consent: Informed consent was obtained from allpatients or their legal guardians.Authorship ContributionsSurgical and Medical Practices: F.B.B.A., E.G.Ü., Y.Z., M.S.E., E.Ü.,H.M.Ö., C.A.; Concept: F.B.B.A.; Design: F.B.B.A., E.G.Ü; DataCollection or Processing: F.B.B.A., Y.Z.; Analysis or Interpretation:Y.Z., F.B.B.A., E.G.Ü; Literature Search: F.B.B.A., E.G.Ü; Writing:F.B.B.A.Conflict of Interest: No conflict of interest was declared by theauthors.Financial Disclosure: The authors declared that this studyreceived no financial support.References1. Tosetto A, Castaman G, Rodeghiero F. Bleeding scores in inherited bleedingdisorders: clinical or research tools? Haemophilia 2008:14:415-422.2. Rodeghiero F, Castaman G, Tosetto A, Batlle J, Baudo F, Cappeletti A, CasanaP, De Bosch N, Eikenboom JC, Federici AB, Lethagen S, Linari S, Srivastava A.The discriminant power of bleeding history for the diagnosis of type 1 vonWillebrand disease: results from a multicenter study. J Thromb Haemoast2005;3:2619-2626.3. Tosetto A, Rodeghiero F, Castaman G, Goodeve A, Federici AB, Batlle J, MeyerD, Fressinaud E, Mazurier C, Goudemand J, Eikenboom J, Schneppenheim R,Budde U, Ingerslev J, Vorlova Z, Habart D, Holmberg L, Lethagen S, Pasi J,Hill F, Peake I. A quantitative analysis of bleeding symptoms in type 1 vonWillebrand disease: results from a multicenter European study (MCMDM-1VWD). J Thromb Haemost 2006;4:766-773.4. Bowman M, Riddel J, Rand ML, Tosetto A, Silva M, James PD. Evaluationof the diagnostic utility for von Willebrand disease of a pediatric bleedingquestionnaire. J Thromb Haemost 2009;7:1418-1421.5. Belen B, Kocak U, Isik M, Keskin EY, Oner N, Sal E, Kaya Z, Yenicesu I, GurselT. Evaluation of Pediatric Bleeding Questionnaire in Turkish children withvon Willebrand disease and Platelet function disorders. Clin Appl ThrombHemost 2015;21:565-569.6. Rodeghiero F, Tosetto A, Abshire T, Arnold DM, Coller B, James P, NeunertC, Lillicrap D; ISTH/SSC Joint VWF and Perinatal/Pediatric HemostasisSubcommittees Working Group. ISTH/SSC bleeding assessment tool: astandardized questionnaire and a proposal for a new bleeding score forinherited bleeding disorders. J Thromb Haemost 2010;8:2063-2065.7. Bidlingmaier C, Grote V, Budde U, Olivieri M, Kurnik K. Prospective evaluationof pediatric bleeding questionnaire and the ISTH bleeding assessment toolin children and parents in clinical routine. J Thromb Haemost 2012;10:1335-1341.8. Pathare A, Omrani SA, Al Hajri F, Al Obaidani N, Al Balushi B, Al Falahi K.Bleeding score in type 1 von Willebrand disease patients using the ISTH-BATquestionnaire. Int J Lab Hematol 2018;40:175-180.©Copyright 2020 by Turkish Society of HematologyTurkish Journal of Hematology, Published by Galenos Publishing HouseAddress for Correspondence/Yazışma Adresi: Fatma Burcu Belen Apak, M.D., Başkent University Faculty ofMedicine, Department of Pediatric Hematology Oncology, Ankara, TurkeyPhone : +90 532 581 45 51E-mail : draidabb@gmail.com ORCID: orcid.org/0000-0002-9278-6703Received/Geliş tarihi: July 25, 2019Accepted/Kabul tarihi: November 12, 2019DOI: 10.4274/tjh.galenos.2019.2019.044658
Turk J Hematol 2020;37:57-76LETTERS TO THE EDITORChildren with Iron Deficiency Anemia Have a Tendency toHypercoagulation: An Evaluation by ThromboelastographyDemir Eksikliği Anemisi Olan Çocukların Hiperkoagülasyona Eğilimleri Vardır:Tromboelastograf ile Bir DeğerlendirmeCeren Kılcı 1 , Lale Olcay 2 , Beril Özdemir 3 , Ali Fettah 4 , Meriç Yavuz Çolak 51Başkent University Faculty of Medicine, Department of Pediatrics, Ankara, Turkey2Başkent University Faculty of Medicine, Department of Pediatrics, Department of Pediatric Hematology-Oncology, Ankara, Turkey3Başkent University Faculty of Medicine, Department of Pediatrics, Ankara, Turkey4Sami Ulus Pediatrics Training and Research Hospital, Department of Pediatric Hematology-Oncology Ankara, Turkey5Başkent University, Faculty of Health Sciences, Ankara, TurkeyTo the Editor,In the literature, there are many reports about patients whodeveloped thrombosis with coexistent iron deficiency (ID) or IDanemia (IDA) [1,2,3,4,5,6,7,8].Moreover, the frequency of severe anemia [1] and IDA [2] inpatients who developed cerebral venous thrombosis (CVT) ordeep venous thrombosis (including pulmonary embolism),respectively, was shown to be higher than in controls. Theoccluded vessels were cerebral vessels in 96.2% and 46.4% ofthe affected children and adults, respectively [8].The predilection to hypercoagulation in ID/IDA was predicted tobe due to reactive thrombocytosis, microcytosis, dehydration,infections, alterations in laminar flow, formation of turbulence,corruption of the oxidant/antioxidant balance, increases inplatelet aggregation, increased procoagulants, and hypoxia[3,4,5,6,7,8]. However, laboratory investigations of this topic arestill rare.Herein we aim to provide laboratory evidence of the propensityto thrombosis in IDA using thromboelastography, which canqualitatively determine the status of coagulation as hyper- orhypocoagulation, and to state whether the abnormality stemsfrom any pathology in primary hemostasis, secondary hemostasis,or the fibrinolytic system or any effects of anticoagulantsor inhibitors within 30 min. With thromboelastography, theformation, strength, elasticity, and firmness of a clot can beshown using parameters such as reaction (R) time, clot formation(K) time, alpha (α) angle, maximum amplitude (MA), maximumlysis (LY30), and coagulation index (CI). Their functions andimplications are presented in Supplemental Table 1.Blood samples from 34 IDA patients between the ages of 3.5 and191 months and from 39 healthy children of 12 to 191 months ofage were studied using the flat cup test in thromboelastography(TEG ® 5000 Thromboelastograph® Hemostasis Analyzer).Patients with chronic (including thalassemia) or infectious/inflammatory diseases, high c-reactive protein (CRP) levels,obesity, hypertension, smoking habit, hyperuricemia, liveror renal function abnormalities, vitamin B12 or folic aciddeficiencies, and self or family history of thrombosis or bleedingwere excluded from the study, as were those on any drugtherapy.The thromboelastographic measurements observed in the IDAand control groups were as follows: K, 1.4±0.6 vs. 1.8±1.1 min(p=0.03); MA, 70.6±4.9 mm vs. 66.9±8.3 mm (p=0.05); LY30,3.8±4.4 vs. 2.0±3.2 (p=0.12); R, 3.9±1.4 vs. 4.0±1.4 min (p=0.78);α, 53.0±8.9° vs. 53.0±9.6° (p=0.91); and CI, 1.0±1.4 vs. 0.3±2.1(p=0.19) (Supplemental Table 2; Figures 1A and 1B).Significant decrease in K and increase in MA with borderlinesignificance compared to the controls implied hypercoagulability,which was possibly due to increased fibrinogen levels and/or toa lesser extent increased thrombocyte functions (SupplementalTable 1). Inflammation-related hyperfibrinogenemia was aremote possibility since patients with infection/inflammationand high CRP levels were excluded; however, we could notestablish fibrinogen levels and thrombocyte functions. Otherstudies showed normal levels of fibrinogen [9] and increased[10] or decreased thrombocyte aggregation [11] in IDA.Our findings revealed a positive linear relationship betweenserum iron levels and α (p=0.034; r=0.339) and between redblood cell distribution width (RDW) and α (p=0.004; r=0.448),and an inverse linear relationship between RDW and K (p=0.048;r=-0.319) in the control group.In the IDA group, there was a positive and weak linear relationshipbetween ferritin and α (p=0.049; r=0.341), a positive linearrelationship between mean corpuscular volume (MCV) and MA(p=0.04; r=0.353), and an inverse linear relationship betweenthrombocyte count and K (p=0.041; r=-0.353).59
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