1.Glucose-6-phosphate Dehydrogenase Deficiency
Clinical Pediatric Hematology-Oncology 2015;22(1):1-7
Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common enzyme disorder. There are more than 400 million people worldwide with G6PD deficiency, and its distribution is similar to that of malaria. G6PD deficiency is an X-linked recessive disorder. Most patients with G6PD deficiency may be asymptomatic throughout their lives. They may present as neonatal jaundice, or acute and chronic hemolysis. The most important point in the management of G6PD deficiency is to avoid oxidative stress. The prevalence of G6PD deficiency in Korea is about 0.9%. However, a nationwide survey has revealed that the number of patients with enzymopathy is increasing. Immigration of different ethnicities into Korea, and the rise of interracial marriages will likely lead to an increase in the number of patients with G6PD deficiency.
Anemia, Hemolytic, Congenital
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Anemia, Hemolytic, Congenital Nonspherocytic
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Emigration and Immigration
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Favism
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Glucosephosphate Dehydrogenase
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Glucosephosphate Dehydrogenase Deficiency
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Hemolysis
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Humans
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Infant, Newborn
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Jaundice, Neonatal
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Korea
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Malaria
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Marriage
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Oxidative Stress
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Prevalence
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Splenectomy
2.Autoimmune hemolytic anemia with a paroxysmal nocturnal hemoglobinuria-like defect: report of one case.
Juxian TONG ; Wei KOU ; Qi CHEN ; Duan XIAO
Journal of Southern Medical University 2013;33(12):1864-1865
Both autoimmune hemolytic anemia and paroxysmal nocturnal hemoglobinuria are common hemolytic diseases. The former causes hemolysis because of immune disorder, and the latter is an acquired clonal hematologic disorder of stem cells. The two entities are often separate diseases, but can also occur concomitantly or secondary to each other. paroxysmal nocturnal haemoglobinuria-like defect-like defect is a special type of autoimmune haemolytic anaemia and should be distinguished from typical paroxysmal nocturnal haemoglobinuria-like defect.
Anemia, Hemolytic, Autoimmune
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Hemoglobinuria, Paroxysmal
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Humans
3.Hemolytic anemia in pediatrics.
Korean Journal of Pediatrics 2007;50(6):511-518
To understand the hemolytic anemia (HA) in children, the diagnostic approach and management of hereditary and acquired HA are described. The hereditary hemolytic anemia (HHA) can be classified according to the pathogenesis into three types:RBC membrane defects, hemoglobinopathies, and RBC enzymopathies. Clinical characteristics, laboratory findings and molecular defects of these three types are presented briefly. In Korea, HHA due to the RBC membrane defect, hereditary spherocytosis had been reported often but HHA due to hemoglobinopathies and RBC enzymopathies had been thought to be relatively rare. With recent development in the molecular diagnosis, beta thalassemia, mostly heterozygote, G6PD and pyruvate kinase deficiency have been reported with gene characterization. If the patients with microcytic hypochromic anemia show unproportionally low MCV or MCH or refractory to the iron therapy, hemoglobin electrophoresis and gene analysis for thalassemia or other unstable hemoglobinopathies need to be done accordingly. The global movement of the population especially from the region prevalent of hemoglobinopathies or enzymopathies to Korea warrants considering broad spectrum of etiology for the diagnosis of HHA. Aquired HA resulting from extracellular factors such as autoimmune HA from warm antibody, cold agglutinin and paroxysmal cold hemoglobinuria as well as nonimmune HA are described briefly.
Anemia, Hemolytic*
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Anemia, Hemolytic, Autoimmune
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Anemia, Hemolytic, Congenital
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Anemia, Hypochromic
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beta-Thalassemia
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Child
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Diagnosis
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Electrophoresis
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Hemoglobinopathies
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Hemoglobinuria, Paroxysmal
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Heterozygote
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Humans
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Iron
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Korea
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Membranes
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Pediatrics*
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Pyruvate Kinase
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Thalassemia
4.A Case of Congenital Nonspherocytic Hemolytic Anemia.
Tae Sook KIM ; Sang Bae JEON ; Bok Yang PYUN
Journal of the Korean Pediatric Society 1985;28(6):597-603
No abstract available.
Anemia, Hemolytic, Congenital Nonspherocytic*
5.Comparison of hemolytic characteristics among paroxysmal nocturnal hemoglobinuria, autoimmune hemolytic anemia and hereditary spherocytosis.
Wei Wang LI ; Jun SHI ; Zhen Dong HUANG ; Neng NIE ; Ying Qi SHAO ; Xing Xin LI ; Mei Li GE ; Jing ZHANG ; Jin Bo HUANG ; Peng JIN ; Min WANG ; Yi Zhou ZHENG
Chinese Journal of Hematology 2018;39(4):299-304
Objective: To determine the valuable hemolytic characteristics in differential diagnosis of paroxysmal nocturnal hemoglobinuria (PNH), autoimmune hemolytic anemia (AIHA) and hereditary spherocytosis (HS). Method: The clinical and hemolytic characteristics of 108 PNH patients, 127 AIHA patients and 172 HS patients diagnosed from January 1998 to April 2017 were compared. Results: ①Reticulocyte percentage (Ret%) of PNH patients [6.70% (0.14%-22.82%)] was significantly lower than that of AIHA [14.00%(0.10%-55.95%), P<0.001] and HS patients [11.83%(0.60%-57.39%), P<0.001]. The Ret% in PNH patients were significantly lower than those in AIHA and HS patients at the same levels of anemia, except for in mild anemia between PNH and AIHA patients. However, when comparing the Ret% between AIHA and HS patients, there was significant difference only in mild anemia [7.63%(1.87%-29.20%)% vs 11.20%(3.31%-22.44%), z=-2.165, P=0.030]. ②The level of TBIL in HS patients was significantly higher than that in AIHA and PNH patients [79.3 (11.2-244.0) μmol/L vs 57.6 (7.6-265.0) μmol/L, z=5.469, P<0.001; 79.3(11.2-244.0) μmol/L vs 26.2(4.6-217.7) μmol/L, z=-2.165, P<0.001], and the proportion of HS patients with TBIL more than 4 times the upper limit of normal (ULN) (64.1%) was significantly higher than that of AIHA (37.7%, χ(2)=19.896, P<0.001) and PNH patients (4.6%, P<0.001). ③The LDH level of PNH patients was significantly higher than that of AIHA and HS [1 500 (216-5 144) U/L vs 487 (29-3 516) U/L, z=-9.556, P<0.001; 1 500 (216-5 144) U/L vs 252 (132-663) U/L, z=-11.518, P<0.001], and the proportion of PNH patients with LDH more than 1 000 U/L (79.1%) was significantly higher than that of AIHA patients (13.0%, χ(2)=93.748, P<0.001) and HS patients (0, P<0.001). ④Splenomegaly occurred in 43.5% of PNH patients, including 16.0% with severe splenomegaly. In contrast, the occurrence of splenomegaly was 98.6% in AIHA patients and 100.0% in HS patients (P<0.001), and 63.0% of AIHA patients (P<0.001) and 90.4% of HS patients (P<0.001) were with severe splenomegaly. ⑤The prevalence of cholelithiasis in HS patients was up to 43.1%, significantly higher than that in AIHA patients (10.5%, P<0.001) and PNH patients (2.9%, P<0.001). Conclusion: The comprehensive assessment of the five hemolytic characteristics is simplified, practical and efficient, with great clinical significance, providing specific indicators for differential diagnosis and efficient approach for making further work-up.
Anemia, Hemolytic, Autoimmune
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Diagnosis, Differential
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Hemoglobinuria, Paroxysmal
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Hemolysis
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Humans
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Spherocytosis, Hereditary
6.Red Blood Cell Enzymopathies Causing Hereditary Hemolytic Anemia
Clinical Pediatric Hematology-Oncology 2012;19(1):1-6
The RBC enzyme deficiencies causing hereditary hemolytic anemia (HHA) can be divided into three groups: those participating in the glycolytic (E-M) pathway; those involved with the maintenance of a high ratio of reduced to oxidized glutathione; one enzyme in the nucleotide degradation and salvage pathway. Although RBC enzyme deficiencies causing HHA are rare, 3 of the 15 kinds of important and relatively frequently reported enzyme deficiencies such as pyruvate kinase, glucose-6-phosphate-dehydrogenase and pyrimidine-5'-nucleotidase deficiencies are briefly reviewed. The molecular genetics, clinical symptoms, diagnosis and therapeutic approaches of each enzyme deficiencies are summerized. As these enzyme deficiencies are reported throughout the world as well as in Korea with the identification of the mutations, considering a broad spectrum of etiologies for the diagnosis of HHA seems to be warranted.
Anemia, Hemolytic, Congenital
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Erythrocytes
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Glucosephosphate Dehydrogenase Deficiency
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Korea
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Molecular Biology
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Pyruvate Kinase
7.Diagnosis and treatment of autoimmune hemolytic anemia: classic approach and recent advances.
Blood Research 2016;51(2):69-71
No abstract available.
Anemia, Hemolytic, Autoimmune*
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Diagnosis*
8.A Case of Autoimmune Hemolytic Anemia Caused by Warm Antibody.
Sei Hee CHOI ; Mee Kyung NAMGOONG ; Baek Keun LIM
Journal of the Korean Pediatric Society 1986;29(8):86-89
No abstract available.
Anemia, Hemolytic, Autoimmune*
9.Acute exacerbation of cold agglutinin disease during operation.
Jinyoung JEONG ; Hye Kyoung LEE ; Eun Su CHOI ; Wonsik AHN
Korean Journal of Anesthesiology 2013;65(6 Suppl):S125-S126
No abstract available.
Anemia, Hemolytic, Autoimmune*
10.Acute exacerbation of cold agglutinin disease during operation.
Jinyoung JEONG ; Hye Kyoung LEE ; Eun Su CHOI ; Wonsik AHN
Korean Journal of Anesthesiology 2013;65(6 Suppl):S125-S126
No abstract available.
Anemia, Hemolytic, Autoimmune*