Introduction: Thalassemias are common monogenic autosomal recessive hemoglobin disorders. The usually asymptomatic heterozygotes (β-thalassemia traits, βTT) may rarely develop non-transfusion-dependent-thalassemia (NTDT) due to co-inheritance of supernumerary α-globin genes. Literature on phenotypic/genotypic features of these rare combinations is limited. Materials and Methods:We studied the demographic, clinical, and laboratory data from 47 persons with co-inherited βTT + supernumerary α-globin genes. HBB mutations were tested for by ARMS-PCR and/or Sanger sequencing, ααα (anti3.7) /ααα (anti4.2) and deletional α-thalassemia testing by multiplex gap-PCRs, and Xmn1 G γ genotyping by PCR-RFLP. Results:The 47 cases comprised 0.08% of 61 010 hemoglobinopathy screenings during the study period. Mean age was 31.9 ± 14.7 years (range 5.5-83 years), with 57.4% males. Thirty (63.8%) had NTDT-phenotype, 16 (34%) were asymptomatic/ minimally symptomatic, and 1 became transfusion-dependent at the age of 20 years.Anemia/pallor and jaundice were the commonest complaints (76% each); 40% had required blood transfusions. Twenty-one had splenomegaly, 14 had hepatomegaly.Mean hemoglobin was 9.0 ± 1.9 g/dl (range 4.0-13.0). HbA2 was 5.1 ± 0.7% (3.4%-6.3%) and HbF% 4.2 ± 3.2% (0.5%-18.4%). Forty-four (93.6%) had ααα anti3.7 , while 3 (6.4%) had ααα anti4.2 triplications. HBB:c.92+5G>C (47%), HBB:c.27_28insG (14.9%), and HBB:c.47G>A (8.5%) were the commonest β-globin mutations. One case showed HBB:c.-138C>T (β ++ ), while the rest had β 0 or severe-β + mutations. Symptomatic cases had significantly lower hemoglobins and higher HbF% than asymptomatic ones.
Hypereosinophilia (HE) can be caused by a wide variety of non-hematologic (secondary or reactive) and hematologic (primary, clonal) disorders. Diagnosing hypereosinophilia/hypereosinophilic syndrome (HE/HES) is challenging due to the complex nature of disease manifestations and numerous underlying etiologies. Knowing that only rare cases are clonal, it is wise to rule out reactive conditions and proceed with molecular and other advanced tools. The exclusion of secondary causes needs a detailed clinical evaluation followed by a wide range of serological and imaging investigations. Once reactive eosinophilia has been ruled out, the diagnosis of primary HE/HES is made using a combination of morphologic examination of the blood and bone marrow, conventional cytogenetics, fluorescent in situ hybridization, flow-cytometry, and T-cell clonality evaluation to look for histopathologic or clonal evidence of an underlying hematological disorder. The accurate diagnosis of clonal eosinophilia-causing myeloid and lymphoid neoplasms and the identification of numerous gene rearrangements significantly enhance patient outcomes, because a proportion of these patients (such as PDGFRA and PDGFRB rearrangements) responds well to tyrosine kinase inhibitors. Considering the complex etiopathologies, the cost of testing, and the time involved, the workup needs to be tailored according to the urgency of the situation and the resources available. In urgent situations with organ damage, it is crucial to initiate appropriate management without waiting for the results of investigations. In contrast, in a resource-limited situation, it is acceptable to employ step-by-step rather than comprehensive testing to rule out the most common causes first. Here, we discuss various laboratory investigations employed in diagnosing HE/HES, highlighting their importance in different situations.
Andrographis echioides leaves were used to isolate a flavonoid (medicarpin) that inhibited mild steel corrosion in 1M HCl. Preliminary phytochemical analysis revealed that the A.E leaves were rich in flavonoids. Following a room temperature extraction with methanol, the constituents were fractionated using a Soxhlet apparatus. Chromatographic techniques were used to isolate the constituents from the leaves of Andrographis echioides. Flavonoid compounds were characterized using Spectral studies. Weight loss and corrosion studies were investigated the inhibition activity of an isolated flavonoid (medicarpin). The inhibition efficiency of isolated flavonoid was highest (85.78 percent) at 800 ppm concentration, according to weight loss and electrochemical measurements. The influence of temperature on the corrosion activities of base metal was calculated in the temperature range 293-303K. The inhibitory activity increases with rising inhibitor concentration but decreases with rising temperature. The activation and free energies for the inhibition response sustain the method of physisorption. The adsorption of isolated flavonoid on the base metal is an endothermic process and it follows the Langmuir adsorption isotherm. The potentiostatic study illustrates that isolated flavonoid is a mixed type inhibitor. Surface Morphology examination by SEM and EDAX to reveals the adherence of isolated flavonoid on the mild steel surface.
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