scholarly journals Sickle Cell Anemia: A review

2021 ◽  
Vol 32 (3) ◽  
pp. 513-527
Author(s):  
Alfonso J. Ayala Viloria ◽  
Henry J. González Torres ◽  
Gabriel J. David Tarud
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Calidad De Vida ◽  
Hb S

La anemia hemolítica más frecuente en la población mundial es la anemia de células falciformes, con una incidencia de 1/600 recién nacidos en Estados Unidos y en España algunas regiones con incidencia de 1/5000 neonatos; en Colombia no hay registros con respecto a la incidencia y prevalencia. La transmisión de la ACF es autosómica dominante. Los homocigotos (SS), no sintetizan Hb A y poseen eritrocitos con un 90% de Hb S. El portador o heterocigoto (AS) tiene hematíes con Hb A mayor al 50% y Hb S de 20 – 40% y son usualmente asintomáticos. La Hb S se debe a una mutación en el gen de la cadena beta de globina, lo que conlleva a la polimerización de la Hb en condiciones de baja oxigenación, originándose un cambio en la morfología del eritrocito adquiriendo la forma falciforme. La sintomatología es secundaria a la anemia hemolítica crónica, la vaso–oclusión en los diferentes órganos y la asplenia funcional la cual predispone a la infección. Otras manifestaciones asociadas son el secuestro esplénico, la aplasia eritroide y las complicaciones órgano – especificas, que disminuyen la calidad de vida y predisponen a mayor mortalidad. Su manejo debe realizarse en centros de referencia donde haya un manejo integral incluyendo el recurso humano y físico, ya que el manejo inadecuado y sus complicaciones disminuyen la sobrevida la cual no es superior a los 45 años según reportes.

scholarly journals A assistência de enfermagem em crianças e adolescentes portadores de anemia falciforme

2012 ◽  
Vol 2 (5) ◽  
pp. 5 ◽  
Author(s):  
Aline Barbosa Soares ◽  
Débora Rita Gobbi ◽  
André Moreno Silva ◽  
Gisele Duarte da Silva ◽  
Isabel Cristina Gomes Leite de Siqueira ◽  
...  
Keyword(s):  
Children And Adolescents ◽  
Sickle Cell ◽  
Nursing Care ◽  
Sickle Cell Anemia ◽  
Childhood And Adolescence ◽  
Nursing Interventions ◽  
Crisis Prevention ◽  
Calidad De Vida ◽  
High Prevalence ◽  
Prevention And Treatment

Anemia Falciforme é uma doença autossômica recessiva com elevada prevalência e morbimortalidade no Brasil. Apresenta uma variedade de complicações que se manifestam de forma peculiar em cada paciente, principalmente na infância e na adolescência. Levantar os diagnósticos e intervenções de enfermagem mais relevantes às crianças e aos adolescentes portadores da doença para proporcionar uma melhor qualidade de vida. Realizou-se o levantamento bibliográfico sobre o tema nas bases de dados SciELO, LILACS e AAFESP, além de dados sobre mortalidade da doença no portal DATASUS. Em 2008, 38,6% das mortes por Anemia Falciforme no Brasil compreendeu a faixa etária entre 0 e 19 anos, evidenciando a necessidade de uma assistência de enfermagem específica, baseada na prevenção e no tratamento das principais complicações da doença. O preparo do enfermeiro é fundamental para individualizar essa assistência junto às crianças e adolescentes portadores da doença para que se obtenha sucesso na prevenção das crises e no tratamento para amenizar os sintomas.Descritores: Enfermagem, Anemia Falciforme, Crianças e Adolescentes. Nursing care in children and adolescents with sickle cell anemiaAbstract: Sickle cell anemia is an autosomal recessive disease with a high prevalence, morbidity and mortality in Brazil. Features a variety of complications that manifest in a peculiar way in each patient, especially in childhood and adolescence. Raise the diagnoses and nursing interventions most relevant to children and adolescents with the disease to provide a better quality of life. We carried out the literature on the subject in the databases SciELO, LILACS and AAFESP, plus data on mortality rates in DATASUS portal. In 2008, 38.6% of deaths from sickle cell disease in Brazil comprised the age group between 0 and 19 years, highlighting the need for specific nursing care based on prevention and treatment of major complications of the disease. The preparation of nurses is critical to individualize this intervention with children and adolescents with the disease in order to achieve success in crisis prevention and treatment to alleviate the symptoms.Descriptors: Nursing, Sickle Cell Anemia, Children and Adolescents. Enfermería de atención a la infancia y la adolescencia con anemia de células falciformesResumen: La anemia falciforme es una enfermedad autosómica recesiva, con una alta prevalencia, morbilidad y mortalidad en Brasil. Cuenta con una variedad de complicaciones que se manifiestan de una manera peculiar en cada paciente, especialmente en la infancia y la adolescencia. Elevar los diagnósticos e intervenciones de enfermería más relacionados con los niños y adolescentes con la enfermedad de proporcionar una mejor calidad de vida. Hemos llevado a cabo la literatura sobre el tema en la bases de datos SciELO, LILACS y AAFESP, además de datos sobre las tasas de mortalidad en DATASUS portal. En 2008, el 38,6% de las muertes por la enfermedad de células falciformes en Brasil comprende el grupo de edad entre 0 y 19 años, destacando la necesidad de cuidados de enfermería específicos basados en la prevención y tratamiento de las principales complicaciones de la enfermedad. La preparación de las enfermeras es fundamental para individualizar la intervención con niños y adolescentes que padecen la enfermedad con el fin de lograr el éxito en la prevención de crisis y el tratamiento para aliviar los síntomas.Descriptores: Enfermería, Anemia de Células Falciformes, la Niñez y la Adolescencia.

β-Thalassemia, HB S-β-Thalassemia and Sickle Cell Anemia Among Tunisians

Hemoglobin ◽  
1991 ◽  
Vol 15 (1-2) ◽  
pp. 11-21 ◽  
Author(s):  
S. Fattoum ◽  
F. Guemira ◽  
C. Öner ◽  
R. Öner ◽  
H-W. Li ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Hb S

Correlation of Iron Levels with Asthma and Lung Function in Pediatric Patients with Sickle Cell Anemia

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 12-12
Author(s):  
Yusra D Shaikh ◽  
Nataly Apollonsky ◽  
Bruce Bernstein
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Serum Iron ◽  
Pediatric Patients ◽  
Pulmonary Complications ◽  
Acute Chest Syndrome ◽  
Iron Level ◽  
Obstructive Sleep ◽  
History Of ◽  
Hb S

Introduction:Significant morbidity and mortality in patients with sickle cell disease (SCD) is attributed to the pulmonary sequalae of the disease. Patients with SCD often suffer airway hyper-reactivity, acute chest syndrome (ACS), chronic lung disease, pulmonary hypertension (PHTN), and obstructive sleep apnea (OSA). Recent literature has provided evidence supporting the strong association between asthma and airway hyper-reactivity in SCD. One of the factors linked to chronic inflammation and asthma is iron status. The present study examined whether iron levels are associated with pulmonary complications in pediatric patients with SCD. Method:Through retrospective review of electronic medical records (EMR) we evaluated patients with diagnosis of asthma and SCD. All patients with available PFT (3/21/2013-3/11/2020) and iron studies were included in the analysis. Chi square and ANOVA tests were used to explore relationships of respiratory conditions with lab data and relevant medical history. Results:The analysis reviewed information of 100 patients with SCD -- 56 males and 44 females The sample population had the following genotypes: 63% Hemoglobin (Hb) SS, 23% Hb SC, 2% Hb S Beta Zero Thalassemia, and 12% Hb S Beta Thalassemia. 38% of these patients were receiving treatment via hydroxyurea. The results generated found that patients with a large airway obstruction (LAO) had a marginally statistically significantly higher serum iron level than those with no LAO (p=0.067.) Patients with homozygous Hb S disease were four times as likely to have a history of ACS (p=0.004) than those without and were marginally significantly more likely to be SS and SB0Thal (p=0.052). Patients with history of ACS had a significantly higher mean iron saturation and lower total iron binding capacity (TIBC.) Patients with PHTN had significantly higher serum iron levels (p=0.029). Conclusion:Our findings reveal that while iron might play a more significant role in the development of PHTN and ACS in patients with SCD, the role in asthma is borderline in our sample. These findings, although of borderline statistical significance p=0.067, are clinically noteworthy. These results may open a new window for therapy targeted at maintaining iron in normal physiologic ranges to decrease pulmonary complications in patients with sickle cell anemia. Further studies with larger samples are necessary to clarify the meaning of our marginally significant findings. Disclosures No relevant conflicts of interest to declare.

scholarly journals Qualidade de vida dos portadores de doença falciforme

2019 ◽  
Vol 13 (2) ◽  
pp. 24
Author(s):  
Kamila Tuany Lacerda Leão Lima ◽  
João Otávio Ferreira Pereira ◽  
Paulo Roberto De Melo Reis ◽  
Keila Correia De Alcântara ◽  
Flávia Melo Rodrigues
Keyword(s):  
Quality Of Life ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Functional Capacity ◽  
Sickle Cell Anemia ◽  
Physical Aspect ◽  
Cell Disease ◽  
Calidad De Vida ◽  
Sf 36

RESUMOObjetivo: avaliar a qualidade de vida de portadores de células falciformes atendidos pelo Programa de Anemia Falciforme. Método: trata-se de um estudo quantitativo, descritivo e analítico em 20 portadores de anemia falciforme e 40 indivíduos não falciformes. Coletaram-se os dados por meio de entrevistas no período entre fevereiro a maio de 2015. Avaliou-se a qualidade de vida por meio de SF-36 e WHOQOL-BREF. Apresentaram-se os resultados em forma de tabelas. Resultados: constata-se que a maioria dos pacientes com doença falciforme se declara como negros e castanhos e com baixo nível de escolaridade; os aspectos físicos e a capacidade funcional tiveram os piores resultados e, com a idade, o aspecto físico se torna mais comprometido. Mostrou-se, pelo questionário SF-36, que, entre os escores, os indivíduos com DF apresentavam dor, capacidade funcional, vitalidade, aspectos físicos, emocionais e de saúde mental como os mais prejudicados em relação ao grupo de pacientes sem DF. Conclusão: apresentou-se, pela avaliação WHOQOL-BREF, comprometimento significativo da qualidade de vida física e geral entre os pacientes com DF; já os participantes com doença falciforme sofrem um impacto negativo na qualidade de vida, o que interfere e influencia a saúde dessas pessoas. Descritores: Qualidade de Vida; Doença Crônica; Perfil de Impacto da Doença; Anemia Falciforme; Anemia Hemolítica; Eritrócitos. ABSTRACT Objective: to evaluate the quality of life of sickle cell patients treated by the Sickle Cell Anemia Program. Method: this is a quantitative, descriptive and analytical study in 20 patients with sickle cell anemia and 40 non-sickle individuals. Data was collected through interviews between February and May 2015. Quality of life was evaluated through SF-36 and WHOQOL-BREF. Results were presented in the form of tables. Results: the majority of patients with sickle cell disease declare themselves as black and brown with a low level of schooling; the physical aspects and the functional capacity had the worst results and, with age, the physical aspect becomes more compromised. The SF-36 questionnaire showed that, among the scores, individuals with FD presented pain, functional capacity, vitality, physical, emotional and mental health aspects as the most impaired in relation to the group of patients without SCD. Conclusion: the WHOQOL-BREF evaluation showed a significant impairment of physical and general quality of life among patients with DF; participants with sickle-cell disease have a negative impact on quality of life, which interferes with and influences the health of these people. Descritores: Quality of Life; Chronic Disease; Sickness Impact Profile; Sickle Cell Anemia; Anemia Hemolytic; Erythrocytes.RESUMEN Objetivo: evaluar la calidad de vida de portadores de células falciformes atendidos por el Programa de Anemia Falciforme. Método: se trata de un estudio cuantitativo, descriptivo y analítico en 20 portadores de anemia falciforme y 40 individuos no falciformes. Se recogieron los datos a través de entrevistas en el período entre febrero a mayo de 2015. Se evaluó la calidad de vida por medio de SF-36 y WHOQOL-BREF. Se presentaron los resultados en forma de tablas. Resultados: se constata que la mayoría de los pacientes con enfermedad falciforme se declara como negros y castaños y con bajo nivel de escolaridad; los aspectos físicos y la capacidad funcional tuvieron los peores resultados y, con la edad, el aspecto físico se vuelve más comprometido. Se mostró, por el cuestionario SF-36, que entre los escores, los individuos con DF presentaban dolor, capacidad funcional, vitalidad, aspectos físicos, emocionales y de salud mental como los más perjudicados en relación al grupo de pacientes sin DF. Conclusión: se presentó, por la evaluación WHOQOL-BREF, un compromiso significativo de la calidad de vida física y general entre los pacientes con DF; ya los participantes con enfermedad falciforme sufren un impacto negativo en la calidad de vida, lo que interfiere e influye en la salud de esas personas. Descritores: Calidad de Vida; Enfermedad Crónica; Perfil de Impacto de la Enfermedad; Anemia de Células falciformes; Anemia Hemolítica; Eritrocitos. 

Newborn Screening for Sickle Cell Disease: Effect on Mortality

PEDIATRICS ◽  
1989 ◽  
Vol 83 (5) ◽  
pp. 834-834
Author(s):  
Elliott Vichinsky ◽  
Deborah Hurst ◽  
Ann Earles ◽  
Klara Kleman ◽  
Bertram Lubin
Keyword(s):  
Sickle Cell Disease ◽  
Mortality Rate ◽  
Newborn Screening ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Comprehensive Treatment ◽  
Cell Disease ◽  
Screening Programs ◽  
Hb S ◽  
Overall Mortality

Newborn screening for sickle cell disease has been recommended as a method of decreasing patient mortality. However, its effectiveness in accomplishing this has not been reliably measured. To help determine the effectiveness, 10 years of experience in newborn screening have been summarized. The effects of early patient enrollment in a comprehensive treatment program on long-term morbidity and mortality are reported. From 1975 to 1985, 84,663 newborns were screened regardless of race or ethnic background. Hb Bait's was present in 5%, hemoglobin AS in 2.6%, and hemoglobin AC in 0.75%. Excluding Bart's, approximately 3.6% of all newborns were carriers for hemoglobinopathy. Sickle cell disease occurred in 1:951 births (58 Hb 55, 25 Hb FSC, three Hb S-β+-thalassemia, and three Hb S-β°-thalassemia). In addition, one in every 4,233 newborns had a clinically significant thalassemia syndrome (eight Hb FE, ten Hb F only, two Hb H). Compared with other newborn screening programs in California (congenital hypothyroidism, 1:3,849; phenylketonuria, 1:22,474; galactosemia, 1:74,103), hemoglobinopathies are the most prevalent congenital disease. Eighty-one newborns with sickle cell disease were followed up for 7.2 years. Patients experienced 513 hospitalizations, including 13 episodes of sepsis with or without meningitis and ten acute sequestration crises. The overall mortality rate for patients with sickle cell anemia diagnosed in the newborn period was 1.8%. In comparison, the clinical course of 64 patients with sickle cell anemia diagnosed after 3 months of age and followed up for an average of 9.4 years was analyzed. Five of these patients died. In two of these, sickle cell anemia was diagnosed at the time of the death. Overall mortality rate in this group was 8%. In summary, the data indicate that newborn screening, when coupled with extensive follow-up and education, will significantly decrease patient mortality.

scholarly journals SPLENIC FUNCTIONS IN NON NEGRO PATIENTS WITH SICKLE-CELL ANEMIA AND HB-S-THALASSEMIA

1978 ◽  
Vol 12 (1) ◽  
pp. 69-69 ◽  
Author(s):  
Sinasi Özsovlu ◽  
Necdet Altinoz ◽  
Yahya Lâleli
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Hb S

scholarly journals Sickle Cell Anemia with Two Adult Hemoglobins—Hb S and Hb GPhiladelphia/S

Blood ◽  
1964 ◽  
Vol 23 (2) ◽  
pp. 206-215 ◽  
Author(s):  
REGINALD P. PUGH ◽  
THOMAS V. MONICAL ◽  
VIRGINIA MINNICH
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Hb S ◽  
Α Chain ◽  
Β Chain

Abstract Hemoglobin studies have been presented on a patient with clinically typical sickle cell anemia who was found to possess two major adult hemoglobins, Hb S and hybrid Hb GPhil./S. Four hemoglobins were demonstrated in his mother, Hb A, GPhil., S and GPhil./S, in somewhat unexpected and as yet unexplained proportions. To our knowledge the propositus represents the first description of an individual with a homozygous β chain defect accompanied by a heterozygous α chain abnormality.

Difficulties in the diagnosis of Hb S/Beta thalassemia: Really a mild disease?

2021 ◽  
Author(s):  
Süheyl UCUCU
Keyword(s):  
Sequence Analysis ◽  
Dna Sequence ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Dna Sequence Analysis ◽  
Current Status ◽  
Beta Thalassemia ◽  
Carrier Status ◽  
Laboratory Findings ◽  
Hb S

OBJECTIVE: HbS/β cases having clinical, hematologic and electrophoretic similarities cannot be sufficiently distinguished from sickle cell anemia cases, and are misdiagnosed as sickle cell anemia. This study will investigate the congruence between the HPLC thalassemia scanning tests and the laboratory findings in comparison with the DNA sequence analysis results of the patients diagnosed with SCA between 2016 and 2020. This study also aims to indicate the current status to accurately diagnose sickle cell anemia and HbS/β in the light of hematologic, electrophoretic and molecular studies. MATERIAL METHOD: Fourteen patients who were diagnosed with SCA in hospitals at different cities in Turkey and followed by the Thalassemia Diagnosis, Treatment and Research Center, Muğla Sıtkı Koçman University were included in this retrospective study. The socio demographic characteristics, hemogram, hemoglobin variant analysis results and DNA chain analysis results of the patients were taken from the database of the center and then examined. The informed consents were taken from the patients. The patients were administered a survey containing questions about transfusion history and diagnostic awareness. The Beta-Thalassemia mutations were analyzed using DNA sequencer (Dade Behring, Germany) based on the Sanger method. RESULTS: According to the DNA sequence analysis results of these patients diagnosed with SCA in hospitals in different cities of Turkey: Of 14 patients, 8 had Hb S/β0 and Hb S/β+ and one had Hb S carrier, and one had Hb-O, and three had SCA. The patient with HbS carrier status also contains three additional mutations all of which are heterozygous. We discovered that although two of three mutations, which are c.315+16G>C and c.316-185C>T, are previously reported as benign, at least one of the two mentioned mutations, when combined with Hb S, causes transfusion-dependent Hb S/β. CONCLUSION: Briefly, HbSS and HbS/β thalassemia genotypes cannot be definitely characterized by electrophoretic and hematologic data, resulting in misdiagnosis. c.315+16G>C and c.316-185C>T, are previously reported as benign, at least one of the two mentioned mutations, when combined with Hb S, causes transfusion-dependent Hb S/β.  In undeveloped or some developing countries, molecular diagnosis methods and genetic analyses cannot be used. If mutation analyses could be performed, then such differential diagnosis errors would reduce. However, if mutation analysis cannot be performed, other methods such as HPLC, capillary electrophoresis absolutely be sought to have insight into the parental carriage status.

High Mortality Among Children with Sickle Cell Anemia and Stroke Who Discontinue Chronic Blood Transfusion After Transition to An Adult Program

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 1629-1629 ◽  
Author(s):  
Samir K. Ballas
Keyword(s):  
Blood Transfusion ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Gene Deletion ◽  
Adult Care ◽  
Adult Programs ◽  
Blood Exchange ◽  
Hb S ◽  
Alpha Gene ◽  
The Mean

Abstract Abstract 1629 Introduction: The Comprehensive Sickle Cell Disease study (CSCD) of 4082 patients showed that the likelihood of developing stroke was 11% by the age of 20 years for patients with sickle cell anemia (SS).The stroke prevention trials (STOP I and STOP II) in SS showed that blood transfusion prevents the occurrence of primary stroke in children with abnormal TransCranial Doppler (TCD) velocities (STOP1) and discontinuation of transfusions after 30 months resulted in a high rate of reversion to abnormal TCD velocities and stroke (STOP II). To that end all children with abnormal TCD velocities are managed with chronic blood transfusion without interruption as long as they are in pediatrics. The problem arises when they are due to be transferred to adult care after the age of 18 years. The transition process from pediatrics to adult programs is marred with several issues that often result in interruption or discontinuation of the quality care the patients had as children. There is little or no information in the literature about the outcome of children with stroke after transition to adult programs. In this study we report the outcome of 22 patients, 10 males and 12 females, with SS and stroke who were referred to our adult program from several Children's hospitals between 1993 and 2009. Patients & Methods: Records were kept prospectively. The mean age of patients at transition was 22.4 ± 3.10 years. Blood bank data were performed and computerized according to FDA and AABB regulations. Blood counts and % Hb S were done before and after each transfusion. Hb S was kept below 30% or 50% after transfusion. Metabolic profiles were done every 6 months or more often if needed. Molecular diagnostics including alpha genotypes and beta globin haplotypes were done by described methods. Statistical analysis was by the 2-tailed t test. Results: All patients had ischemic strokes during childhood and one had hemorrhagic stroke superimposed on cerebral infarction. Two patients developed moyamoya after transition. No alpha gene deletion was detected in 18 patients, 4 had one alpha gene deletion and none had 2 alpha gene deletion. The Ben/CAR haplotype was found in 7 patients, Ben/Ben in 5, Ben/Sen in 4 and other combinations in 6 patients; 21 patients were heterozygous for the Ben haplotype. Hb F was 1.0–7.0% except for one patient on hydroxyurea (HU) with 20% Hb F. All patients were kept on the same regimen of transfusion they had as children; 15 were kept on exchange transfusion two of whom were non-compliant, two were kept on simple transfusion plus HU and the remaining 5 patients refused to have chronic blood transfusion and were kept on normal care. Some of the causes for refusal were due to alloimmunization and difficult venous access. One of these 5 patients agreed to be on HU. Patients on blood exchange required an average of 55 ± 13.5 (40-98) units of RBC annually. Those on simple transfusion required up to 24 units annually. Alloimmunization was present in 10 patients with the number of alloantibodies between 1 and 6 per patient. The most common alloantibodies were ant-E and anti-K. Patients who were compliant with blood transfusion were rarely hospitalized for painful crises. Patients who were noncompliant with blood transfusion or who refused to be maintained on chronic transfusion were hospitalized for painful crises 5.7 ± 5.00 times annually (p<.025). All 5 patients who refused to be maintained on chronic blood transfusion died within 3 years after transition. Cause of death was massive intraventricular hemorrhage in one patient proven by autopsy, massive hemispheric infarct proven by imaging in the second patient leading to coma and death in the hospital and sudden death during hospitalization for painful crises in the remaining 3 patients. Two patients who were not compliant with chronic blood transfusion died within 2 years after transition due to ARDS. Thus the overall rate of death after transition was 32% and the major cause was discontinuation of chronic blood transfusion. The mean age at death was 27.0 ± 3.16 years whereas the mean age of patients who are alive is 35.4 ± 5.92 years (p<.001). Conclusion: Our data indicate that about one third of patients with SS and stroke die within 3 years after transition especially if chronic blood transfusion is discontinued or if compliance was poor. It is imperative that all efforts should be made to maintain adequate chronic blood transfusion for children with SCD and stroke after transition to adult care. Disclosures: No relevant conflicts of interest to declare.

Evaluation of a commercial kit for microchromatographic quantitation of hemoglobin A2 in the presence of hemoglobin S.

1981 ◽  
Vol 27 (7) ◽  
pp. 1244-1247 ◽  
Author(s):  
R M Baine ◽  
H G Brown
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Sickle Cell Trait ◽  
Accurate Method ◽  
Beta Thalassemia ◽  
Coefficients Of Variation ◽  
Hb S ◽  
Slow Moving ◽  
Commercial Kit ◽  
Hemoglobin A2

Abstract Commercial microcolumns introduced in 1976 by Helena Laboratories ("Hb A2 Quik Column") and by Isolab, Inc. ("Quik-Sep") provide a rapid, simple, accurate method for quantitation of hemoglobin A2 (Hb A2). However, these kits cannot be used for the quantitation of Hb A2 in the presence of slow-moving variants such as Hb S. Recently, Isolab, Inc., produced a new kit ("Quik-Sep Improved Hb A2 Test") for quantitation of both Hb A2 and Hb S. We compared results obtained with the new Isolab kit to results obtained with the original Tris/HCl method for quantitation of Hb A2 and Hb S. Blood was drawn from persons with sickle cell trait (A/S), sickle cell anemia (S/S), sickle cell/beta+ thalassemia (S/beta+ thal) and sickle cell/beta 0 thalassemia (S/beta 0 thal) and percentages of Hb A2 and Hb S were determined by each method. We found no significant differences in Hb A2 percentages by the two methods, and the coefficients of variation were similar. Both methods showed only slight overlap of Hb A2 values from subjects with some form of beta thalassemia and those with A/S or S/S. However, the Tris/HCl method consistently gave values for Hb S that were higher and closer to those expected, suggesting that the Isolab kit does not accurately quantitate Hb S.

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