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Homozygous Delta-Beta Thalassemia in a Child: a Rare Cause of Elevated Fetal Hemoglobin
Case Report Homozygous delta-beta Thalassemia in a Child: a Rare Cause of Elevated Fetal Hemoglobin Verma S MD1, Bhargava M MD2, Mittal SK MD 3, Gupta R MD4 1. Senior Resident, Department of Pathology, Chacha Nehru Bal Chikitsalaya, Delhi,India. 2. Consultant Pathologist, Department of Pathology, Pushpanjali Crosslay Hospital,India. 3. Director & Senior Consultant, Department of Pediatrics, Pushpanjali Crosslay Hospital,India. 4. Assistant Professor and Head, Departments of Pathology, Chacha Nehru Bal Chikitsalaya, Delhi,India. Received: 15 November 2012 Accepted: 26 January 2013 Abstract Background complete absence of HbA and HbA2 with HbF Delta beta (δβ) thalassemia is an unusual variant of constituting 100% of the hemoglobin. Hemoglobin thalassemia with elevated level of fetal hemoglobin analysis of both parents showed elevated level of (HbF). Homozygous patients of this disorder, unlike HbF with normal HbA2. A final diagnosis of δβ- β-thalassemia, show mild anemia. Only few cases of thalassemia in the child with both parents being δβ-thalassemia have been reported from India in the carriers was rendered. available indexed English literature. Conclusion Case presentation Delta beta-thalassemia is an uncommon cause of A four-year old male child was evaluated for recent- markedly elevated fetal hemoglobin beyond fetal onset jaundice. Hematological investigations showed period. Clinical and haematological parameters mild anemia with microcytic hypochromic red cells. should be evaluated to render an accurate diagnosis. A comprehensive analysis of hemoglobin by high- Key Words performance liquid chromatography (HPLC) showed Delta-Beta Thalassemia ;Homozygote; Chromatography, High Pressure Liquid Corresponding Author Ruchika Gupta,Department of Pathology,Chacha Nehru Bal Chikitsalaya (Associate Hospital of Maulana Azad Medical College),Geeta Colony,Delhi (India), E-mail: [email protected] Introduction confirmatory test for diagnosis of this rare disorder Delta beta (δβ) thalassemia is an infrequent cause of (4). -
Serious Hazards of Transfusion (SHOT) Annual Report 2017
ANNUAL SHOT REPORT 2017 working with Affiliated to the Royal College of Pathologists DS OF ZAR TR A AN H S S F U U O S I I R O E N S A Y N R N A U S A R L E S YEARS IV H N O N T A RE T PORT 21S ANNUAL SHOT REPORT 2017 Serious Hazards of Transfusion (SHOT) Steering Group Chair Professor Mark Bellamy SHOT Medical Director Dr Paula Bolton-Maggs Operations Manager Ms Alison Watt Research Analyst Ms Debbi Poles Patient Blood Management Practitioner Mrs Jayne Addison Clinical Incidents Specialist Mr Simon Carter-Graham Mrs Ann Fogg Laboratory Incidents Specialist Mrs Hema Mistry National Coordinator for Mrs Rachael Morrison (Ms Dory Kovacs in 2018) Transfusion-Transmitted Infections (Public Health England) Working Expert Group (WEG) & Writing Group, on behalf of the SHOT Steering Group Chair: Dr Paula Bolton-Maggs Professor Mark Bellamy, Ms Alison Watt, Ms Debbi Poles, Mrs Hema Mistry, Mr Simon Carter-Graham, Mrs Ann Fogg, Mrs Jayne Addison, Mrs Rachael Morrison, Dr Tom Latham, Mrs Diane Sydney, Dr Helen New, Dr Megan Rowley, Dr Fiona Regan, Mr Chris Robbie, Dr Peter Baker, Dr Janet Birchall, Dr Jane Keidan, Mrs Terrie Perry, Mrs Katy Cowan, Dr Sharran Grey, Mrs Clare Denison, Dr Catherine Ralph, Dr Sarah Haynes, Mrs Pamela Diamond, Dr Anicee Danaee, Ms Tracey Tomlinson, Dr Shruthi Narayan, Mrs Heather Clarke. Steering Group (SG) during 2017 Chair: Professor Mark Bellamy Dr Shubha Allard British Society for Haematology Guidelines National Blood Transfusion Committee Dr Ganesh Suntharalingam Intensive Care Society, Faculty of Intensive Care Medicine -
Sehgal Index and Its Comparison with Mentzer's Index and Green and King Index in Assessment of Peripheral Blood Smear with Marked Anisopoikilocytosis
International Journal of Research in Medical Sciences Rastogi N et al. Int J Res Med Sci. 2020 Aug;8(8):2972-2977 www.msjonline.org pISSN 2320-6071 | eISSN 2320-6012 DOI: http://dx.doi.org/10.18203/2320-6012.ijrms20203449 Original Research Article Sehgal index and its comparison with Mentzer's index and Green and King index in assessment of peripheral blood smear with marked anisopoikilocytosis Naincy Rastogi*, Arvind S. Bhake Department of Pathology, Jawaharlal Nehru Medical College, Sawangi, Wardha, Maharashtra, India Received: 20 February 2019 Revised: 29 May 2020 Accepted: 06 June 2020 *Correspondence: Dr. Naincy Rastogi, E-mail: [email protected] Copyright: © the author(s), publisher and licensee Medip Academy. This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. ABSTRACT Background: Mild microcytic hypochromic anaemias due to iron deficiency (IDA) and beta thalassemia trait(β-TT) continue to be a cause of significant burden to the society, particularly in the poorer developing countries. The objective of the present study was to study the RBC based indices in patients of marked anisopoikilocytosis in determining the etiology of it, to standardize few automated red cell parameters, and also objective grading of RBC morphology on peripheral smear and interpreting its utility in indicating a diagnosis. Also, to establish a relation between value of RBC indices with that of degree of anisocytosis. Methods: A total of 500 patients diagnosed with mild microcytic hypochromic anaemia on complete blood count and peripheral blood film were included in the study. -
Filterability of Erythrocytes and Whole Blood in Preterm and Full-Term Neonates and Adults
003 1-3998/86/2012-1269$02.00/0 PEDIATRIC RESEARCH Vol. 20, No. 12, 1986 Copyright O 1986 International Pediatric Research Foundation, Inc. Printed in U.S. A. Filterability of Erythrocytes and Whole Blood in Preterm and Full-Term Neonates and Adults OTWIN LINDERKAMP, BERNHARD J. HAMMER, AND ROLF MILLER Department of Pediatrics, University of Heidelberg, Heidelberg, Federal Republic of Germany ABSTRACT. Filtration techniques are widely used to as- Hyperviscosity in neonates is a frequent and potentially serious sess red blood cell (RBC) deformability and flow behavior condition, which may cause circulatory failure and impaired of RBC in microcirculation. In this study filtration rates of microcirculation in various vital organs (1). Blood viscosity is RBC from 10 very low birth weight infants (24-30 wk mainly determined by hematocrit. However, decreased RBC gestation), 10 more mature preterm infants (31-36 wk deformability can also contribute to an increase in blood viscos- gestation), 10 full-term neonates, and 10 adults were meas- ity. At high hematocrit, the deformability of RBC is particularly ured by using Nucleopore filters with pore diameters of 5 crucial because blood viscosity exhibits a larger increase with pm and filtration pressures of 1, 2,5, and 10 cm H20.The increasing hematocrit when RBC deformability is impaired (2). major results follow: 1) At each of four filtration pressures, Moreover, decreased RBC deformability may hinder the entry filtration rates of washed RBC were significantly (p < of 8-pm diameter RBC into capillaries with diameters of 3-4 pm 0.05) lower in the preterm infants than in the term neonates (3), slow down oxygen uptake and release in capillaries (4), and who in turn showed lower values than adults. -
Etiological Study of Microcytic Hypochromic Anemia Kafle S1, Lakhey M2
Journal of Pathology of Nepal (2016) Vol. 6, 994 -997 cal Patholo Journal of lini gis f C t o o f N n e io p t a a l i - c 2 o 0 s 1 s 0 PATHOLOGY A N u e d p a n of Nepal l a M m e h d t i a c K al , A ad ss o oc n R www.acpnepal.com iatio bitio n Building Exhi Original Article Etiological study of microcytic hypochromic anemia Kafle S1, Lakhey M2 1Department of Pathology, Nepal Medical College Teaching Hospital, Kathmandu, Nepal. 2Department of Pathology, Kathmandu Medical College Teaching Hospital, Kathmandu, Nepal. ABSTRACT Keywords: Background: Microcytic hypochromic anemia is a distinct morphologic subtype of anemia with well- Microcytic; defined etiology and treatment. The objective of this study was to determine the etiology and frequency Hypochromic; of microcytic hypochromic anemia. Anemia; Iron; Materials and Methods: This cross-sectional observational study was conducted at Kathmandu Thalassemia; Medical College Teaching Hospital. One hundred cases of microcytic hypochromic anemia were Iron profile; included. Relevant clinical history, hemogram, reticulocyte count, iron profiles were documented in a Electrophoresis proforma. Bone marrow aspiration and hemoglobin electrophoresis was conducted when required. Data was analysed by Microsoft SPSS 16 windows. Result: Iron deficiency was the commonest etiology (49%). Dysfunctional uterine bleeding (20.8%) was the commonest cause of iron deficiency, malignancy (24.3%) was the commonest cause of anemia of chronic disease. Mean value of Mean Corpuscular Volume was lowest in hemolytic anemia (71.0fl). Mean Red cell Distribution Width was normal (14.0%) in hemolytic anemia but was raised in other types. -
C:\Documents and Settings\Babu\
MME -303 Child Health Indira Gandhi National Open University School of Health Sciences Block 6 CHILDHOOD MORBIDITY UNIT 28 Common Paediatric Problems 5 UNIT 29 Cardiovascular, Haematological and Renal Disorders 24 UNIT 30 Gastrointestinal, Parasitic and Neurological Disorders 61 UNIT 31 Tuberculosis 90 UNIT 32 HIV/AIDS 106 UNIT 33 Common Paediatric Emergencies 119 UNIT 34 Common Paediatric Surgical Problems 130 PROGRAMMECORE TEAM Dr. I.C. Tiwari Dr. (Mrs.) Kamala Ganesh Dr. L.N. Balaji Dr. S.B. Arora Dr. Ruchika Kuba Sr. Consultant, UNICEF Director Professor and Head of Chief, Planning Division Course Coordinator Course Coordinator New Delhi O&G Deptt., MAMC, New Delhi UNICEF, New Delhi SOHS, IGNOU, New Delhi SOHS, IGNOU, New Delhi Dr. S.C. Chawla Dr. Sidharth Ramji Prof. (Col.) P.K. Dutta Dr. T.K. Jena Director Professor and Head of Professor, Deptt. of Paediatrics Programme Coordinator Course Coordinator PSM Deptt., LHMC, New Delhi MAMC, New Delhi SOHS, IGNOU, New Delhi SOHS, IGNOU, New Delhi COURSE REVISION TEAM (1st Revision) Dr. Neena Raina Dr. Harish Kumar Dr. M.S. Prasad Prof. A.K. Agarwal Dr. Ruchika Kuba Technical Officer, WHO Consultant Paediatrics, Head, Deptt. of Paediatrics Director, SOHS Sr. Lecturer and Course SEARO Office, New Delhi D.D.U. Hospital, New Delhi Safdarjang Hospital, IGNOU, New Delhi Coordinator New Delhi SOHS, IGNOU, New Delhi Dr. Siddharth Ramji Dr. A.K. Patwari Dr. T.K. Jena Professor, Deptt. of Peadiatrics National Professional Officer, Dr. Piyush Gupta Reader and Programme LNJP and MAMC, Delhi WHO Nirman Bhawan, Reader, Deptt. of Paediatrics Coordinator New Delhi GTB Hospital and UCMS SOHS, IGNOU, New Delhi New Delhi COURSE REVISION TEAM (2nd Revision) Dr. -
Copyrighted Material
Index Page numbers in italic refer to fi gures. Page numbers in bold refer to tables. -
Hereditary Persistenceof Fetal Hemoglobin, /8
Am J Hum Genet 27:140-154, 1975 Hereditary Persistence of Fetal Hemoglobin, /8 Thalassemia, and the Hemoglobin d-fl Locus: Further Family Data and Genetic Interpretations NICHOLAS C. BETHLENFALVAY,' ARNO G. MOTULSKY,2 BELA RINGELHANN,3 HERMANN LEHMANN,4 JAMES R. HUMBERT,5 AND F. I. D. KONOTEY-AHULU6 Hereditary persistence of fetal hemoglobin (HPFH) was first documented in 1955 in Ghana [1] and has also been described in non-African populations [2-7]. The association of HPFH with /8 thalassemia [8-12] and with Hb S and C [9, 13-151 has been amply documented, but heterozygosity for both HPFH and the 8 chain variant Hb B2 has been reported only once [9, 16]. Weatherall and Clegg [17] summarize all these cases. The y-,8 fusion gene Hb Kenya was first reported in a family where both Hb B2 and Hb Kenya were segregating [18]. In this paper one black kindred from the United States and two from Ghana are described which add further to linkage information on HPFH and the linked structural loci for Hb 8 and Hb 8. MATERIALS AND METHODS Standard hematologic methods were used for red cell count, hemoglobin, hematocrit, and osmotic fragility determinations. To demonstrate fetal hemoglobin in red cells, the method of Kleihauer et al. [19] was followed using brilliant cresyl blue-treated blood to eliminate false positive staining of young red cells due to diffuse ribonucleoprotein [20]. The Hb A2 and B2 levels were determined on starch block according to Gerald and Diamond [21]. For best separation of these fractions, electrophoresis was carried Received July 15, 1971; revised October 31, 1974. -
UKOSS Vasa Praevia Form
ID Number: UK Obstetric Surveillance System Vasa Praevia in Pregnancy Study 02/14 Data Collection Form - CASE Please report any woman delivering on or after 1st December 2014 and before 1st December 2015 Case Definition: A case should meet at least one of the criteria below: 1. Suspected VP on antenatal U/S ≥18 weeks gestation, and confirmed on antenatal U/S ≥31 weeks gestation (if not delivered prior to 31 weeks) 2. Palpation or visualisation of the fetal vessels during labour 3. Rupture of membranes with bleeding associated with fetal death/exsanguination or severe neonatal anaemia 4. Antenatal or intrapartum bleeding of fetal origin with pathologic CTG and/or positive Apt6* test 5. VP documented in medical records as reason for admission and caesarean section And At least one of the following: • Clinical examination of the placenta confirming intact or ruptured velamentous vessels.These may be a velamentous insertion of the umbilical cord or exposed fetal vessels between placental lobes • Confirmation of VP on pathologcial examination of the placenta • Torn umbilical cord or placenta (not able to provide placental examination) Please return the completed form to: UKOSS National Perinatal Epidemiology Unit University of Oxford Old Road Campus Oxford OX3 7LF Fax: 01865 617775 CASEPhone: 01865 289714 Case reported in: *For guidance please see back cover Instructions 1. Please do not enter any personally identifiable information (e.g. name, address or hospital number) on this form. 2. Please record the ID number from the front of this form against the woman’s name on the Clinician’s Section of the blue card retained in the UKOSS folder. -
Hydroxyurea Enhances Fetal Hemoglobin Production in Sickle Cell Anemia
Hydroxyurea enhances fetal hemoglobin production in sickle cell anemia. O S Platt, … , B Miller, D G Nathan J Clin Invest. 1984;74(2):652-656. https://doi.org/10.1172/JCI111464. Research Article Hydroxyurea, a widely used cytotoxic/cytostatic agent that does not influence methylation of DNA bases, increases fetal hemoglobin production in anemic monkeys. To determine its effect in sickle cell anemia, we treated two patients with a total of four, 5-d courses (50 mg/kg per d, divided into three oral doses). With each course, fetal reticulocytes increased within 48-72 h, peaked in 7-11 d, and fell by 18-21 d. In patient I, fetal reticulocytes increased from 16.0 +/- 2.0% to peaks of 37.7 +/- 1.2, 40.0 +/- 2.0, and 32.0 +/- 1.4% in three successive courses. In patient II the increase was from 8.7 +/- 1.2 to 50.0 +/- 2.0%. Fetal hemoglobin increased from 7.9 to 12.3% in patient I and from 5.3 to 7.4% in patient II. Hemoglobin of patient I increased from 9.0 to 10.5 g/dl and in patient II from 6.7 to 9.9 g/dl. Additional single-day courses of hydroxyurea every 7-20 d maintained the fetal hemoglobin of patient I t 10.8-14.4%, and the total hemoglobin at 8.7- 10.8 g/dl for an additional 60 d. The lowest absolute granulocyte count was 1,600/mm3; the lowest platelet count was 390,000/mm3. The amount of fetal hemoglobin per erythroid burst colony-forming unit (BFU-E)-derived colony cell was unchanged, but the number of cells per BFU-E-derived colony increased. -
Glucose Phosphate Isomerase Deficiency: Unusual Acute Hemolytic Crisis in a Middle-Aged Woman
Henry Ford Hospital Medical Journal Volume 28 Number 4 Article 3 12-1980 Glucose Phosphate Isomerase Deficiency: Unusual acute hemolytic crisis in a middle-aged woman Koichi Maeda Sheikh M. Saeed Raymond W. Monto Ernest Beutler Follow this and additional works at: https://scholarlycommons.henryford.com/hfhmedjournal Part of the Life Sciences Commons, Medical Specialties Commons, and the Public Health Commons Recommended Citation Maeda, Koichi; Saeed, Sheikh M.; Monto, Raymond W.; and Beutler, Ernest (1980) "Glucose Phosphate Isomerase Deficiency: Unusual acute hemolytic crisis in a middle-aged woman," Henry Ford Hospital Medical Journal : Vol. 28 : No. 4 , 193-196. Available at: https://scholarlycommons.henryford.com/hfhmedjournal/vol28/iss4/3 This Article is brought to you for free and open access by Henry Ford Health System Scholarly Commons. It has been accepted for inclusion in Henry Ford Hospital Medical Journal by an authorized editor of Henry Ford Health System Scholarly Commons. Henry Ford Hosp Med Vol 28, No 4, 1980 Glucose Phosphate Isomerase Deficiency Unusual acute hemolytic crisis in a middle-aged woman Koichi Maeda, MD,* Sheikh M. Saeed, MD,* Raymond W. Monto, MD,** and Ernest Beutler, MD' Hereditary hemolytic anemia associated with glucose in a 56-year-old woman. Steroid therapy seemed to resolve phosphate isomerase (GPI) deficiency was first reported in our patient's acute stage. Since it has not been mentioned 1967. Since then, about 30 cases have been reported in the previously, further evaluation is necessary. Consideration of literature; their ages ranged between 1 and 26 years. We this deficiency may be helpful in investigating hemolytic present a case of glucose phosphate isomerase deficiency anemia, regardless of the patient's age. -
Compound Heterozygous Beta Thalassemia With
Scholars Journal of Applied Medical Sciences (SJAMS) ISSN 2320-6691 (Online) Sch. J. App. Med. Sci., 2014; 2(6D):3097-3098 ISSN 2347-954X (Print) ©Scholars Academic and Scientific Publisher (An International Publisher for Academic and Scientific Resources) www.saspublisher.com Case Report Compound Heterozygous Beta Thalassemia with Heredietary Persistence of Fetal Haemoglobin: A Rare Haematological Combination and Different Spectrum of Thalassemia Jaivinder Yadav1, Deepak Sharma2*, Hanish Bajaj Mittal1, Suman Yadav3, Sweta Shastri4, Aakash Pandita2 1Pt. B.D Sharma, PGIMS, Rohtak, Haryana, India 2Department of Neonatology, Fernandez Hospital, Hyderabad, India 3Department of Anatomy, University College of Medical Sciences, Delhi, India 4ACPM Medical College, Dhule, Maharashtra, India *Corresponding author Deepak Sharma Email: Abstract: 5 year old male child presented with progressive abdominal distention, pallor, and growth failure since the age of 9 months. The foe did not respond to hematinic and required one blood transfusion for anemia. Liver and spleen were enlarged on abdominal exam. Peripheral smear showed features of haemolytic anemia and neonatal red blood cells. HPLC studies of patient revealed that father was a carrier for hereditary persistence of fetal hemoglobin (HPFH) and the mother was thalassemia trait. The child was compounded heterozygous for beta thalassemia and HPFH which resulted in relatively minor clinical severity as compared to beta thalassemia major. Keywords: Beta Thalassemia, Fetal hemoglobin INTRODUCTION examination reviled hepatosplenomegaly with liver Beta thalassemia with HPFH is a rare disease with a palpable 9 cm below costal margin with a span of 13 clinical presentation different from thalassemia major cm, firm in consistency, sharp border, non-tender and and HPFH.