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Haematology and oncology
Published in Jagdish M. Gupta, John Beveridge, MCQs in Paediatrics, 2020
Jagdish M. Gupta, John Beveridge
9.12. Which of the following statements is/are true of hereditary spherocytosis (HS)?About 50% of affected infants have moderately severe neonatal jaundice.The presence of spherocytes on examination of a blood film in the newborn confirms the diagnosis.Aplastic crises occur due to parvovirus infection.Intravascular haemolysis is a common feature.Red blood cell osmotic fragility is increased.
Human Erythroenzymopathies Of The Anaerobic Embden-Meyerhof Glycolytic And Associated Pathways
Published in Ronald L. Nagel, Genetically Abnormal Red Cells, 2019
Ernst R. Jaffé, William N. Valentine
The laboratory features of symptomatic PK deficiency may be categorized as those due to hemolysis itself and those secondary to the molecular lesion. The former include variable reticulocytosis, often becoming much increased after splenectomy, and indirect reacting hyperbilirubinemia. The hemogram reveals anemia. The Wright’s stained blood film exhibits variable polychromasia, lack of spherocytes, and a scattering of acanthocyte-like cells (probably those metabolically depleted and whose destruction is imminent). Such cells are seen in varying degree in other erythroenzymopathies where disturbed energy metabolism is associated with hemolytic syndromes. The osmotic fragility test done on fresh blood is normal. The sterile incubation of fresh blood for 48 hr yields variable findings depending on the molecular lesion. In severe deficiencies, a common pattern is a striking increase in hemolysis not corrected by glucose additives (Type II of Selwyn and Dacie).6
Targeted next-generation sequencing identified a novel ANK1 mutation associated with hereditary spherocytosis in a Chinese family
Published in Hematology, 2019
Qing Sun, Yao Xie, Penghui Wu, Shuo Li, Ying Hua, Xintian Lu, Weihong Zhao
A 4-year-old boy with fever, anaemia, and jaundice was referred to our centre. He had fever for five days before being referred to our department. Pale lips and jaundice were observed soon after he was admitted to the hospital. Hepatomegaly and splenomegaly were identified by physical examination. The boy had a medical history of mild anaemia of unknown cause, of approximately 90 g/L–100 g/L, since infancy. His anaemia and jaundice had become rapidly worse during the past few days. The results of blood tests at the time of admission to our hospital are shown in Table 1, which indicated that the boy suffered from haemolytic anaemia and hyperbilirubinemia. No abnormalities were observed by glucose-6-phosphate dehydrogenase (G6PD) activity analysis, direct Coombs test, or haemoglobin electrophoresis. Erythrocyte osmotic fragility was increased. Densely stained spherical red blood cells (RBC) were frequently observed in the peripheral blood smear (Figure 1A). Then, the proband was clinically diagnosed with HS. The proband’s father had shown similar symptoms, such as anaemia and jaundice, since he was young. A haematological investigation of the proband’s father showed the possibility of compensated haemolysis (Table 1). Splenomegaly was detected by B-ultrasound. Clinical manifestations and haematological data of the proband’s father indicated a high possibility of HS (Figure 2).
Umbilical Cord Blood Screening for the Detection of Common Deletional Mutations of α-Thalassemia in Bangladesh
Published in Hemoglobin, 2020
Saeed Anwar, Jarin Taslem Mourosi, Md. Kamrul Hasan, Mohammad Jakir Hosen, Md. Faruque Miah
We divided all the collected samples into four aliquots. For complete blood counts and Hb concentration, one of the aliquots was analyzed immediately. We left one aliquot at 4 °C for 45 min. and then centrifuged at 4500 g for 15 min. After centrifugation, the serum was carefully separated and stored at –20 °C until subsequent analysis. We measured serum iron (Fe) and zinc (Zn) levels using spectrophotometric and colorimetric methods, following standard laboratory protocols [26,27]. We carried out osmotic fragility tests (OFTs) using another aliquot following a modified protocol from the one previously described [28]. All samples and solutions were processed in sterile conditions.
Role of Oxidative Stress and the Protective Effect of Fermented Papaya Preparation in Sickle Cell Disease
Published in Hemoglobin, 2022
Prashant P. Warang, Nikhil S. Shinde, Vinod D. Umare, Prajyot V. Deshmukh, Kanjaksha Ghosh, Manisha R. Madkaikar, Roshan B. Colah, Malay B. Mukherjee
The inhibition action of FPP on hemolysis of normal RBCs based on osmotic fragility test was also studied. Red cell suspension with FFP was suspended in different concentrations of NaCl and incubated at 37 °C for 24 hours. The extent of hemolysis was measured by the quantity of Hb released into the supernatant. Hemoglobin in the supernatant was measured at 405 nm, using an enzyme-linked immunoassay (ELISA) reader. This showed decreased osmotic fragility (0.380 ± 0.0169%) as compared to the red cells suspension without FPP (0.44 ± 0.0307%). Decreased osmotic fragility suggests prevention of hemolysis.