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Two Korean Cases of Hereditary Spherocytosis Caused by Mutations in SLC4A1

Cho H, Lee JW, Chung NG, Lee SE, Jang W, Kim M, Han K, Kim Y

Hereditary spherocytosis (HS) is caused by mutations in the SPTA1, SPTB, ANK1, SLC4A1, and EPB42 genes, all of which encode erythrocyte membrane proteins. Mutations in SLC4A1, which encodes band 3...
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Cryohemolysis, erythrocyte osmotic fragility, and supplementary hematimetric indices in the diagnosis of hereditary spherocytosis

Emilse LA, Cecilia H, María TM, Eugenia MM, Alicia IB, Lazarte SS

BACKGROUND: Hereditary spherocytosis (HS) is a chronic hemolytic anemia characterized by microspherocytes in the peripheral blood and increased erythrocyte osmotic fragility (EOF). This study evaluated the cryohemolysis test (CHT); initial...
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Coexistence of Gilbert Syndrome and Hereditary Spherocytosis in a Child Presenting with Extreme Jaundice

Lee JH, Moon KR

Gilbert syndrome is the most common inherited disorder of bilirubin glucuronidation. It is characterized by intermittent episodes of jaundice in the absence of hepatocellular disease or hemolysis. Hereditary spherocytosis is...
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A Case of Immune Hemolytic Anemia due to Autoantibodies Against C and e Antigens in a Patient with Paroxysmal Nocturnal Hemoglobinuria and Myelodysplastic Syndrome

Park MJ, Seo YH, Park PW, Kim KH, Park SH, Jeong JH, Lee JH, Hong JS, Ahn JY

  • KMID: 1440725
  • Korean J Blood Transfus.
  • 2012 Apr;23(1):78-83.
Antiglobulin test-negative hemolytic anemia, thrombophilia, and marrow failure, such as aplastic anemia and myelodysplastic syndrome - refractory anemia (MDS-RA), are the primary clinical manifestations of paroxysmal nocturnal hemoglobinuria (PNH). Here,...
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Flow-Assisted Differential Diagnosis of Hemolytic Anemia with Spherocytosis: A Case Report

Won DI

In patients with hemolytic anemia associated with spherocytosis, differential diagnosis has to be made whether the hemolysis is immune-mediated or of non-immune origin. We report a case of hereditary spherocytosis...
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Prolonged Extreme Thrombocytosis in a Postsplenectomy Patient with Hereditary Spherocytosis

Park YM, Park J, Jo Y, Kim SH, Shin KC, Won IS, Sym SJ, Cho EK, Shin DB, Lee JH

We report a case of prolonged extreme reactive thrombocytosis in a post-splenectomy patient with hereditary spherocytosis. A 29-year-old female patient presented with gall stones detected incidentally by abdominal ultrasonography. Her...
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The Aplastic Crisis of Hereditary Spherocytosis Due to Parvovirus B19 Infection

Choi HJ, Lee JH, Lee KS

  • KMID: 2049208
  • Clin Pediatr Hematol Oncol.
  • 2006 Apr;13(1):22-31.
PURPOSE: Hereditary spherocytosis (HS) is the commonest cause of inherited hemolytic anemia in Korea. In hereditary spherocytosis patients, parvovirus B19 infection causes transient severe anemia, so called aplastic crisis. This...
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A Case of Moyamoya Syndrome Associated with Hereditary Spherocytosis

Kim JS, Yoon WS, Kim GM, Kim IS, Woo YJ, Lee JH

  • KMID: 1961855
  • J Korean Child Neurol Soc.
  • 1998 Oct;6(1):125-132.
Moyamoya is a chronic cerebrovascular disease characterized by progressive stenosis or occlusion of the terminal parts of both intermal carotid arteries with telangiectatic vascular network of collateral circulation at the...
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A Case of Transient Aplastic Crisis Induced by Human Parvovirus Bl9 in Hereditary Spherocytosis

Shin KS, Son BR, Lee DH

  • KMID: 2146194
  • Korean J Clin Pathol.
  • 1997 Dec;17(6):928-933.
Human parvovirus B19 is a single-strand DNA virus which causes erythema infectlosum, arthralgia, aplastic crisis in patients with red cell defect, chronic anemia in immunocompromised patients, and fetal hydrops in...
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Influences of Heat on Function and Morphology of Peripheral Blood Cells

Choi JW, Nahm CH, Lee JW, Kim JJ, Kim SK, Pai SH

  • KMID: 2146193
  • Korean J Clin Pathol.
  • 1997 Dec;17(6):912-927.
BACKGROUND: Heat-treated erythrocytes have been reported to be undergone fragmentation and microspherocytes transformation in vitro. However, the changes for enzyme activity of WBCs is not well known. The purpose of...
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Erythrocyte Membrane Protein Alterations by SDS-PAGE and Underlying Clinical Heterogeneity in Hereditary Spherocytosis

Yoo ES, Choi HS, Shin HY, Ahn HS, Lee YK, Cho HI

  • KMID: 1979968
  • Korean J Pediatr Hematol Oncol.
  • 1997 Oct;4(2):261-272.
BACKGROUND: Hereditary spherocytosis(HS) is a clinically and biochemically very heterogeneous disorder The purpose of this study is to detect erythrocyte membrane protein abnormalities by SDS-PAGE and to investigate the frequency...
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Splenectomy in Hereditary Spherocytosis in Childhood

Heo YS, Kim CS, Do BS, Suh BY, Hah JO

Among the erythrocyte membrane defects, hereditary spherocytosis is the most common. The erythrocyte membrane defect results from a deficiency of spectrin, the most important structural protein in red cell. Hereditary spherocytosis...
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The Effect of Amphotericin B on Erythrocyte Volume and Cation Content

Park YS, Solomon S

The effects of amphotericin B, an antifungal antibiotic, on erythrocyte volume and cation permeability were investigated by measuring the hematocrit, cell volume, cation content, fragility and osmotic behavior in rat...
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