Association of Clinical and Hematological variables with the disease severity in Indian Sickle cell anemia patients

2021 ◽  
pp. 5254-5257
Author(s):  
L.V.K.S. Bhaskar ◽  
Smaranika Pattnaik
Keyword(s):  
Liver Function ◽  
Disease Severity ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Severe Disease ◽  
Fetal Hemoglobin ◽  
Mild Disease ◽  
Total Hemoglobin ◽  
Significant Difference ◽  
Function Test

Sickle cell anemia (SCA) is the most common genetic disorder that is caused due to mutation of the β globin gene. Although SCA is a monogenic disorder, the clinical presentation varies greatly among patients. The present study was designed to be a cross sectional study, aimed at analysing the SCA severity and its association with different clinical, biochemical and hematological variables in SCA patients of Indian origin. About 190 random homozygous SCA patients confirmed by hemoglobin electrophoresis were used in the study. Routine biochemical laboratory (liver function test and Renal function test) and hematologic tests (Total hemoglobin, fetal hemoglobin, hematocrit, MCV and MCH) were done. Values pertaining to complete blood count (CBC), Hb-HPLC and clinical investigations were collected from patient’s records. The mean age of patients with severe disease was significantly lesser than the moderate and mild disease patients. The body mass index (BMI) was also significantly lower in severe disease patients compared to the moderate and mild disease. The patients with severe disease had low levels of red blood cells, total hemoglobin (tHb) and fetal hemoglobin (HbF) compared to the other groups. There is no significant difference in the kidney and liver function among various degrees of disease severity. In summary, this study demonstrates that the tHb and HbF and total leucocyte count (TLC) are major prognostic factors for several clinical complications in SCA. Baseline measurement of these important variables is paramount in predicting important aspects of clinical course and improves the quality lives of these children.

scholarly journals The relationship between fetal hemoglobin and disease severity in children with sickle cell anemia

1987 ◽  
Vol 27 (3) ◽  
pp. 525-535 ◽  
Author(s):  
Daniel J. Odenheimer ◽  
Sharada A. Sarnaik ◽  
Charles F. Whitten ◽  
Donald L. Rucknagel ◽  
Charles F. Sing ◽  
...  
Keyword(s):  
Disease Severity ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Fetal Hemoglobin ◽  
The Relationship

scholarly journals Clinical and Hematological Evaluation of Patients with Sickle Cell Anemia Before and After Four Years of Using Hydroxyurea

10.3823/2469 ◽  
2017 ◽  
Vol 10 ◽  
Author(s):  
Ieda Maria Gonçalves Pacce Bispo ◽  
Maria Lúcia Ivo ◽  
Valter Aragão do Nascimento ◽  
Alexandra Maria Almeida Carvalho de Pinto ◽  
Olinda Maria Rodrigues de Araújo ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Hematological Parameters ◽  
Fetal Hemoglobin ◽  
Hemoglobin Concentration ◽  
Teaching Hospitals ◽  
Acute Chest Syndrome ◽  
Mean Values ◽  
Significant Difference ◽  
Before And After

Objective: Evaluating clinical and hematological-clinical parameters of patients with sickle cell anemia (SCA) before and after four years of using hydroxyurea (HU).  Method: A retrospective cohort study implementing a quantitative, descriptive and analytical approach developed in two public teaching hospitals located in the Central-West region of Brazil, from November 2010 to October 2011. Data collection was performed through medical records of 32 patients with SCA to assess clinical and hematological parameters before and after HU treatment. The study was approved by the UFMS Ethics Committee under protocol number 1890/2010. Results: All of the 32 patients were homozygous with a mean age in the prescription of hydroxyurea of 19.72±7.58 years, an initial dose of 15.59±4.27 mg/kg/day, and 22.48±5.35 mg/kg/day in the fourth year of treatment. Regarding the use of HU, average values of some hematological parameters presented a significant difference in the fourth year compared to the mean values prior to HU use, such as fetal hemoglobin (14.49±7.52%), red blood cells (2.54±0.38x1012/L), hematocrit (25.30±4.03%) and hemoglobin (9.22±3.34g/dL).  Conclusion: Treatment with hydroxyurea showed a significant increase in fetal hemoglobin levels, increased hemoglobin, hematocrit and average corpuscular hemoglobin concentration, with reduced episodes of pain, infection and acute chest syndrome in such a way as to reaffirm its efficiency in treating these patients. Keywords: Hemoglobin; Sickle Cell Anemia; Hydroxyurea.

scholarly journals Fetal Hemoglobin in Sickle Hemoglobinopathies: High HbF Genotypes and Phenotypes

2020 ◽  
Vol 9 (11) ◽  
pp. 3782
Author(s):  
Martin H. Steinberg
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Globin Gene ◽  
Point Mutations ◽  
Fetal Hemoglobin ◽  
Gene Deletions ◽  
Total Hemoglobin ◽  
Sickle Erythrocytes ◽  
Hereditary Persistence ◽  
Very High

Fetal hemoglobin (HbF) usually consists of 4 to 10% of total hemoglobin in adults of African descent with sickle cell anemia. Rarely, their HbF levels reach more than 30%. High HbF levels are sometimes a result of β-globin gene deletions or point mutations in the promoters of the HbF genes. Collectively, the phenotype caused by these mutations is called hereditary persistence of fetal hemoglobin, or HPFH. The pancellularity of HbF associated with these mutations inhibits sickle hemoglobin polymerization in most sickle erythrocytes so that these patients usually have inconsequential hemolysis and few, if any, vasoocclusive complications. Unusually high HbF can also be associated with variants of the major repressors of the HbF genes, BCL11A and MYB. Perhaps most often, we lack an explanation for very high HbF levels in sickle cell anemia.

The HBG2 rs7482144 (C > T) Polymorphism is Linked to HbF Levels but not to the Severity of Sickle Cell Anemia

2021 ◽  
Author(s):  
Bhaskar V. K. S. Lakkakula ◽  
Smaranika Pattnaik
Keyword(s):  
Disease Severity ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
High Performance ◽  
Complete Blood Count ◽  
Small Sample Size ◽  
Severe Disease ◽  
Small Sample ◽  
Total Leucocyte Count ◽  
Severity Scores

AbstractSickle cell anemia (SCA) is a severe disease characterized by anemia, acute clinical complications, and a relatively short life span. In this disease, abnormal hemoglobin makes the red blood cells deformed, rigid, and sticky. Fetal hemoglobin (HbF) is one of the key modulators of SCA morbidity and mortality. Interindividual HbF variation is a heritable trait that is controlled by polymorphism in genes linked and unlinked to the hemoglobin β gene (HBB). The genetic polymorphisms that determine HbF levels are known to ameliorate acute clinical events. About 190 well-characterized homozygous SCA patients were included in this study. Complete blood count (CBC), high-performance liquid chromatography (HPLC), and clinical investigations were obtained from patient's records. Severity scores were determined by using the combination of anemia, complications, total leucocyte count, and transfusion scores. HBG2 rs7482144 polymorphism was genotyped by using the polymerase chain reaction and restriction fragment length polymorphism. The association between HBG2 rs7482144 polymorphism and HbF levels as well as the disease severity of SCA were assessed. SCA patients carrying TT genotype were found to have higher HbF levels. In addition, SCA patients with increased severity showed significantly lower levels of hemoglobin, HbF, and hematocrit values. However, the genotypes of HBG2 rs7482144 polymorphism were not found to be associated with the risk of disease severity. In summary, this study demonstrated that HBG2 rs7482144 polymorphism is linked with HbF levels, but it does not affect disease severity. The sample sizes used and the pattern of association deduced from our small sample size prevents us from extrapolating our findings further.

scholarly journals COVID-19 Disease Severity and Determinants among Ethiopian Patients: A study of the Millennium COVID-19 Care Center

2020 ◽  
Author(s):  
Tigist W. Leulseged ◽  
Kindalem G. Abebe ◽  
Ishmael S. Hassen ◽  
Endalkachew H. Maru ◽  
Wuletaw C. Zewde ◽  
...  
Keyword(s):  
Diabetes Mellitus ◽  
Disease Severity ◽  
Multinomial Logistic Regression ◽  
Care Center ◽  
Severe Disease ◽  
P Value ◽  
Age Group ◽  
Disease Category ◽  
Mild Disease ◽  
Significant Difference

ABSTRACTBackgroundUnderstanding determinants of developing severe COVID-19 disease is important as studies show that severe disease is associated with worse outcomes.ObjectiveThe study aimed to assess the determinants of COVID-19 disease severity among COVID-19 patients admitted to Millennium COVID-19 Care Center in Ethiopia.MethodsA cross-sectional study was conducted from June to August 2020 among randomly selected 686 patients. Chi-square test was used to detect the presence of a statistically significant difference in the characteristics of the patients based on disease severity (Mild Vs Moderate Vs Severe), where p-value of <0.05 was considered as having a statistically significant difference. A Multivariable multinomial logistic regression model was used to assess the presence of a significant association between the independent variables and COVID-19 disease severity where Adjusted Odds ratio (AOR), 95% CIs for AOR and P-values were used for testing significance and interpretation of results.ResultsHaving moderate as compared with mild disease was significantly associated with having hypertension (AOR= 2.302, 95% CI= 1.266, 4.184, p-value=0.006), diabetes mellitus (AOR=2.607, 95% CI= 1.307, 5.198, p-value=0.007 for diabetes mellitus), fever (AOR= 6.115, 95% CI= 2.941, 12.716, p-value=0.0001) and headache (AOR= 2.695, 95% CI= 1.392, 5.215, p-value=0.003). Similarly, having severe disease as compared with mild disease was associated with age group (AOR= 4.428, 95% CI= 2.497, 7.853, p-value=0.0001 for 40-59 years and AOR=18.070, 95% CI=9.292, 35.140, p-value=0.0001 for ≥ 60 years), sex (AOR=1.842, 95% CI=1.121, 3.027, p-value=0.016), hypertension (AOR= 1.966, 95% CI= 1.076, 3.593, p-value=0.028), diabetes mellitus (AOR= 3.926, 95% CI= 1.964, 7.847, p-value=0.0001), fever (AOR= 13.218, 95% CI= 6.109, 28.601, p-value=0.0001) and headache (AOR= 4.816, 95% CI= 2.324, 9.979, p-value=0.0001). In addition, determinants of severe disease as compared with moderate disease were found to be age group (AOR= 4.871, 95% CI= 2.854, 8.315, p-value=0.0001 for 40-59 years and AOR= 18.906, 95% CI= 9.838, 36.334, p-value=0.0001 for ≥ 60 years), fever (AOR= 2.161, 95% CI= 1.286, 3.634, p-value=0.004) and headache (AOR= 1.787, 95% CI= 1.028, 3.107, p-value=0.039).ConclusionsBeing old, male sex, hypertension, diabetes mellitus, and having symptoms of fever and headache were found to be determinants of developing a more severe COVID-19 disease category. We recommend a better preventive practice to be set in place so that these groups of patients can be protected from acquiring the disease. And for those who are already infected, a more careful follow-up and management should be given so that complication and death can be prevented. Furthermore, considering the above non respiratory symptoms as disease severity indicator could be important.

Fetal hemoglobin in sickle cell anemia: a glass half full?

Blood ◽  
2014 ◽  
Vol 123 (4) ◽  
pp. 481-485 ◽  
Author(s):  
Martin H. Steinberg ◽  
David H. K. Chui ◽  
George J. Dover ◽  
Paola Sebastiani ◽  
Abdulrahman Alsultan
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Severe Disease ◽  
Fetal Hemoglobin ◽  
Cell Disease ◽  
Sickle Hemoglobin ◽  
F Cells ◽  
The Mean ◽  
Number Of Cells

Abstract Fetal hemoglobin (HbF) modulates the phenotype of sickle cell anemia by inhibiting deoxy sickle hemoglobin (HbS) polymerization. The blood concentration of HbF, or the number of cells with detectable HbF (F-cells), does not measure the amount of HbF/F-cell. Even patients with high HbF can have severe disease because HbF is unevenly distributed among F-cells, and some cells might have insufficient concentrations to inhibit HbS polymerization. With mean HbF levels of 5%, 10%, 20%, and 30%, the distribution of HbF/F-cell can greatly vary, even if the mean is constant. For example, with 20% HbF, as few as 1% and as many as 24% of cells can have polymer-inhibiting, or protective, levels of HbF of ∼10 pg; with lower HbF, few or no protected cells can be present. Only when the total HbF concentration is near 30% is it possible for the number of protected cells to approach 70%. Rather than the total number of F-cells or the concentration of HbF in the hemolysate, HbF/F-cell and the proportion of F-cells that have enough HbF to thwart HbS polymerization is the most critical predictor of the likelihood of severe sickle cell disease.

scholarly journals Longitudinal Analysis of Hydroxyurea and Fetal Hemoglobin in Adult Patients with Sickle Cell Anemia

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 2054-2054
Author(s):  
Aryeh Pelcovits ◽  
Giancarlo Riotto ◽  
Katie Cherenzia ◽  
Patrick T. McGann ◽  
John L Reagan
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Conflicts Of Interest ◽  
Surrogate Marker ◽  
Fetal Hemoglobin ◽  
P Value ◽  
Significant Difference ◽  
Dosing Strategies ◽  
Over Time ◽  
Painful Crisis

Abstract Introduction: Hydroxyurea (HU) was the first FDA-approved therapy for sickle cell anemia (SCA) and remains the most well-established disease-modifying therapy, reducing painful crisis and improving morbidity and mortality. HU improves outcomes through the upregulation of fetal hemoglobin (HbF). Dosing is highly variable with doses ranging from &lt;10-35 mg/kg/day. Most dosing strategies escalate with a goal of mild myelosuppression, commonly defined using an absolute neutrophil count (ANC) between 2,000-4,000 and platelets &gt;80,000. Macrocytosis is often used as a surrogate marker for compliance and effect. Despite its well-established effectiveness, clinical use remains limited with only 12% of adults with SCA taking HU. The reasons for this are multifactorial but include skepticism by both providers and patients regarding its effectiveness in the adult population. Some experts suggest up to 30% of adults are non-responders with no significant HbF change, and many believe that the effect of HU fades with time. Dosing strategies are usually quite conservative to prevent excessive myelosuppression, though dose is highly correlated with clinical effect and ultimate %HbF. There is very limited real-world data regarding long term effectiveness of HU in adults with SCA over time. Methods: We retrospectively analyzed the records of the 109 adults with SCA currently treated in our multidisciplinary sickle cell clinic. Data from 1/1/2011-6/30/2021 was collected, including genotype, HU prescription status, current and maximum laboratory values (HbF, MCV, ANC), and number of admissions for painful crisis. We performed Wilcoxon rank sum testing between pts prescribed and not prescribed HU and measures of HbF (peak, average and current) and number of admissions for painful crisis over the study period. Results: Among 106 pts (3 excluded from analysis, 2 for lack of data and 1 for post-transplant status), 79 are currently prescribed HU (75%). Among our 63 pts with HbSS genotype 58 are prescribed HU (92%). Average HbF over time for all pts prescribed HU was 11.9%. Average peak HbF was 18.1% and average current HbF is 12.4%. In the pts not prescribed HU HbF average, peak and current levels were 5.5%, 6.7%, and 5.0% respectively. HU prescription was significantly associated with increased HbF at peak, average, and current levels (p-value &lt;0.001, Figure A). HU prescription was also significantly associated with decreased number of admissions for painful crisis (p-value 0.001). Among pts prescribed HU, MCV levels &gt;100 at time of peak HbF were associated with higher peak levels than pts with MCV &lt;100 (p-value &lt;0.001, Figure B). Larger peak HU doses were also correlated with higher MCVs at the time of peak HbF levels (Figure C). Larger current HU doses were also significantly associated with higher current HbF levels (Figure, D). Doses &gt;9.9m/kg were associated with significantly higher HbF levels however there was no significant difference between dose levels 10-19.9mg/kg and 20-29.9 or 20-29.9 and 30-39.9mg/kg (p-values 0.02, 0.68 and 0.84 respectively). Among pts prescribed HU, 34 obtained ANC levels &lt;4000 at the times of peak HbF and 24 at the current dosing level (43% and 30% respectively). 24 pts obtained both MCV&gt;100 and ANC &lt;4000 at time of peak HbF and only 11 achieved both at current dosing levels (30% and 14% respectively). Conclusion: In our adult multidisciplinary sickle cell clinic prescribing rates are well above current HU usage for adults with SCA. Our data notes that elevated HbF levels can be maintained over long periods of time. HU prescription was significantly associated with increased HbF levels and reduced admissions for painful crisis. This was despite a majority of pts not meeting target prescribing levels of ANC &lt;4000 and MCV &gt;100. While higher peak HbF levels were significantly associated with higher HU doses, this was only true for doses above 9.9 mg/kg. Further investigation is needed to explore the factors contributing to suboptimal HbF and MCV response, including careful assessment with medication adherence and dose selection. Overall, these data illustrate the importance of dosing and suggest that one size dosing of HU for adults with SCA does not fit all. We hypothesize that there may be a role individualized dosing strategies in adults with SCA, incorporating factors such as pharmacokinetics and renal function to achieve the optimal dose for each patient. Figure 1 Figure 1. Disclosures No relevant conflicts of interest to declare.

scholarly journals Blood Types and Severity of COVID-19

2021 ◽  
Vol 10 (4) ◽  
pp. 211-218
Author(s):  
Burcu Ozdemir ◽  
Levent Ozdemir ◽  
Bilge Akgunduz ◽  
Murat Celik ◽  
Senem Urfali ◽  
...  
Keyword(s):  
Disease Severity ◽  
Severe Disease ◽  
Type A ◽  
Blood Groups ◽  
Blood Type ◽  
Control Group ◽  
Retrospective Case ◽  
Blood Types ◽  
Mild Disease ◽  
Significant Difference

Aim: Since blood types first appeared, their association with diseases caused by microorganisms has been further investigated with several studies for many years. The bond of blood groups described as A, B, AB, and O with coronavirus has been the research subject in many countries.We aimed to elucidate whether there was a relationship between blood types and Rh factor and contracting COVID-19 disease and disease severity. Methods: The study was designed as a retrospective case-control study. Between March 2020 - February 2021, 1110 patients were included (538 cases, 572 controls). Disease severity was classified according to where patients were treated: those who were outpatients considered as “mild disease”, hospitalized in a hospital ward considered as “moderate disease”, and treated in the intensive care unit were considered as “severe disease”. Results: The number of people with blood type A was 447 (40.3%), blood type B was 197 (17.7%), blood type AB was 90 (%8), and blood type O was 376 (33.9%). There was no significant difference between the case and control groups according to the blood types. A 3.93 times increase of developing mild illness was detected compared to the control group in Rh-positive individuals. The rate of developing a severe disease was higher in females with blood type A than a mild disease, and A blood type caused the disease to be severe compared to other blood groups in females. Conclusion: We concluded that blood type A caused more severe disease than other blood types in females, and females with B blood type survived the disease as outpatients. Our study can shed light on pathophysiological investigation of the relationship between COVID-19 disease causing a pandemic with high mortality and virulence and blood types. Keywords: COVID-19 virus, blood group, disease

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