Online ISSN: 2515-8260

Keywords : sickle cell disease


Haemoglobin among patients with Sickle Cell Disease with high performance liquid chromatography

Manoj Kumar Mohapatra, Prafulla Kumar Bariha, Kshetramohan Tudu, Nawal Kishore Jajodia

European Journal of Molecular & Clinical Medicine, 2022, Volume 9, Issue 3, Pages 602-608

Background: Measurement of HbA2 in sickle cell illness is critical for distinguishing
between sickle cell anaemia (HbSS) and Hb S/0-thalassemia. The goal of the current
research is of assessing the magnitude of HbA2 among sufferers diagnosed with SC
hemoglobinopathy and also HbSS, with or without associated alpha thalassemia, with
the help of High-Performance Liquid Chromatography (HPLC).
Methods: In the current retrospective study, 242 children belonging to the ages of two
to six years old who had HbSS or HbSC diagnosis were involved. The haemoglobin was
tested with the help of HPLC. Polymerase chain reaction (PCR) was instrumentalised
for detecting alpha thalassemia. Patients were categorised into three groups:
homozygous (3.7/3.7), heterozygous (3.7/), and homozygous wild-type (3.7/3.7). The
mean HbA2 values with alpha thalassemia were compared using variance analyses.
Results: The HbA2 concentrations in the HbSS group (n = 135) were 3.68 0.65 percent
on average (standard deviation). Individuals with HbSS who were heterozygous (n = 28)
or homozygous (n = 3) for alpha thalassemia had mean values of 3.98 and 4.73
respectively. The mean HbA2 of all HbSC sufferers (n = 107) was 4.01 0.507, with 4.29
0.41 percent heterozygous for alpha thalassemia (n = 23) and 4.91 0.22 percent
homozygous for alpha thalassemia (n = 7) respectively. HbA2 values were above 3.5
percent in all patients homozygous for alpha thalassemia. HbA2 values greater than 5.2
percent were seen in sufferers with HbSS and HbSC, regardless of the presence of alpha
thalassemia.
Conclusion: HbA2 levels are higher in patients with HbS or HbC, and alpha
thalassemia genotypes have a direct impact.

Fetal haemoglobin: A novel prognostic determinant in sickle cell anaemia

Dr. Obaid Noman; Dr. Arvind Bhake; Dr. Shweta Bahadure; Dr. Nihar Gupta

European Journal of Molecular & Clinical Medicine, 2020, Volume 7, Issue 2, Pages 2003-2008

Background-Acknowledgement of instrumental action of fetal hemoglobin(HbF)
in improving the clinical condition of sickle cell disease (SCD) patients, began around 7
decades back, by Janet Watson who found that infants with SCD had lesser symptoms and
their deoxygenated RBCs did not sickle or deform as aggressively as their mothers having
sickle cell trait. She proposed high HbF levels in infant blood as compared to mothers for
the observations of her study. Reduced incidence of crisis like vaso-occlusive, haemolytic,
aplastic, megaloblastic, infectious and others have been found with higher HbF levels.
With this backdrop the present study is being undertaken to know the reciprocity between
HbF levels and the state of crisis in sickle cell. The study is expected to fill the missing link
between “Why a few patients with HbS with haemoglobin pattern of SS suffer less from
crisis than that of others with a similar haemoglobin pattern of SS”
Objectives:
• To know HbF levels in pateints with SCD with Hb (SS) pattern
• To draw the comparisons between HbF levels in SCD with Hb(SS) pattern in
subjects 1. With sickle cell crisis and 2. Without sickle cell crisis
• To find out cut-off level of Hb F at its protective effect against the crisis in SCD
Methodology
• Study design - Observational and analytical study
• Sample size - Total of 30 Patients of sickle cell Disease (Hb SS)
• Study duration - 1 year
Expected Results: Incidence of Crisis in HbSS patients are expected to have a inverse
relationship with HbF levels. A cut-off level of HbF above which episodes of crisis are
lower can be expected to be found. Conclusion: Conclusions will be drawn from results
obtained and an attempt to establish a cut-off HbF value which renders a protective action
against crisis in SCD will be made.