A New Glucose-6-Phosphate Dehydrogenase Variant G6PD Sugao (826C→T) Exhibiting Chronic Hemolytic Anemia With Episodes of Hemolytic Crisis Immediately After Birth

2001 ◽  
Vol 74 (2) ◽  
pp. 153-156 ◽  
Author(s):  
Masashi Taki ◽  
Akira Hirono ◽  
Momoko Kawata ◽  
Miwako Den ◽  
Yachiyo Kurihara ◽  
...  
Keyword(s):  
Hemolytic Anemia ◽  
Hemolytic Crisis ◽  
Glucose 6 Phosphate Dehydrogenase ◽  
Chronic Hemolytic Anemia

G6PD Huntsville: a new glucose-6-phosphate dehydrogenase associated with chronic hemolytic anemia

1988 ◽  
Vol 79 (1) ◽  
pp. 90-91
Author(s):  
Kelley Hall ◽  
Marshall T. Schreeder ◽  
Josef T. Prchal
Keyword(s):  
Hemolytic Anemia ◽  
Glucose 6 Phosphate Dehydrogenase ◽  
Chronic Hemolytic Anemia

Glucose-6-phosphate dehydrogenase Durham: A de novo mutation associated with chronic hemolytic anemia

1997 ◽  
Vol 131 (2) ◽  
pp. 284-287 ◽  
Author(s):  
Sherri A. Zimmerman ◽  
Russell E. Ware ◽  
Linda Forman ◽  
Beryl Westwood ◽  
Ernest Beutler
Keyword(s):  
Hemolytic Anemia ◽  
De Novo ◽  
De Novo Mutation ◽  
Glucose 6 Phosphate Dehydrogenase ◽  
Chronic Hemolytic Anemia

scholarly journals Chronic hemolytic anemia is associated with a new glucose-6-phosphate dehydrogenase in-frame deletion in an older woman

2011 ◽  
Vol 46 (4) ◽  
pp. 288-293 ◽  
Author(s):  
Licínio Manco ◽  
Janet Pereira ◽  
Luís Relvas ◽  
Umbelina Rebelo ◽  
Ana Isabel Crisóstomo ◽  
...  
Keyword(s):  
Hemolytic Anemia ◽  
Older Woman ◽  
Glucose 6 Phosphate Dehydrogenase ◽  
Chronic Hemolytic Anemia

ENZYME DEFICIENCY IN ERYTHROCYTES IN CONGENITAL NONSPHEROCYTIC HEMOLYTIC ANEMIA

PEDIATRICS ◽  
1959 ◽  
Vol 24 (2) ◽  
pp. 245-253
Author(s):  
Nasrollah T. Shahidi ◽  
Louis K. Diamond
Keyword(s):  
Hemolytic Anemia ◽  
Reduced Glutathione ◽  
Stability Test ◽  
Enzyme Deficiency ◽  
Injurious Effect ◽  
Metabolic Defects ◽  
Glucose 6 Phosphate Dehydrogenase ◽  
Low Activity ◽  
Chronic Hemolytic Anemia

Two cases of congenital nonspherocytic hemolytic anemia are reported. The patients were brothers and both showed severe metabolic defects in the erythrocytes, consisting of a low content of reduced glutathione, markedly abnormal reduced glutathione stability test, and a very low activity of glucose-6-phosphate dehydrogenase. These metabolic defects were also found in the erythrocytes from the mother but to a milder degree. While both children showed evidences of chronic hemolytic anemia, the mother was found to be healthy. This clinical discrepancy between mother and children is thought to be the result of the degree of the severity of the metabolic defects. The occurrence of the chronic hemolytic process in these patients in the absence of exposure to drugs indicates that the erythrocyte deficient in glucose-6-phosphate dehydrogenase does not necessarily require the injurious effect of a drug to undergo hemolysis. In view of the above findings, it is suggested that reduced glutathione stability test and an assay of glucose-6-phosphate dehydrogenase be performed in all instances where the diagnosis of congenital nonsphenocytic hemolytic anemia is made.

scholarly journals Autoimmune Hemolytic Anemia as a Complication of Congenital Anemias. A Case Series and Review of the Literature

2021 ◽  
Vol 10 (15) ◽  
pp. 3439
Author(s):  
Irene Motta ◽  
Juri Giannotta ◽  
Marta Ferraresi ◽  
Kordelia Barbullushi ◽  
Nicoletta Revelli ◽  
...  
Keyword(s):  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Case Series ◽  
Intravenous Immunoglobulins ◽  
Point Of View ◽  
First Line ◽  
Human Erythropoietin ◽  
Immune Mediated ◽  
Hemolytic Crisis ◽  
Life Threatening

Congenital anemias may be complicated by immune-mediated hemolytic crisis. Alloantibodies are usually seen in chronically transfused patients, and autoantibodies have also been described, although they are rarely associated with overt autoimmune hemolytic anemia (AIHA), a serious and potentially life-threatening complication. Given the lack of data on the AIHA diagnosis and management in congenital anemias, we retrospectively evaluated all clinically relevant AIHA cases occurring at a referral center for AIHA, hemoglobinopathies, and chronic hemolytic anemias, focusing on clinical management and outcome. In our cohort, AIHA had a prevalence of 1% (14/1410 patients). The majority were warm AIHA. Possible triggers were recent transfusion, infection, pregnancy, and surgery. All the patients received steroid therapy as the first line, and about 25% required further treatment, including rituximab, azathioprine, intravenous immunoglobulins, and cyclophosphamide. Transfusion support was required in 57% of the patients with non-transfusion-dependent anemia, and recombinant human erythropoietin was safely administered in one third of the patients. AIHA in congenital anemias may be challenging both from a diagnostic and a therapeutic point of view. A proper evaluation of hemolytic markers, bone marrow compensation, and assessment of the direct antiglobulin test is mandatory.

Henoch-Schönlein Purpura and Human Parvovirus Infection

PEDIATRICS ◽  
1986 ◽  
Vol 78 (1) ◽  
pp. 183-184
Author(s):  
J. J. LEFRÈRE ◽  
A. M. COUROUCÉ ◽  
J. P. SOULIER ◽  
M. P. CORDIER ◽  
M. C. GUESNE GIRAULT ◽  
...  
Keyword(s):  
Abdominal Pain ◽  
Hemolytic Anemia ◽  
Joint Pain ◽  
Past History ◽  
Henoch Schonlein Purpura ◽  
Erythema Infectiosum ◽  
Aplastic Crisis ◽  
Fifth Disease ◽  
Schonlein Purpura ◽  
Chronic Hemolytic Anemia

To the Editor.— Human parvovirus is already known to be responsible for aplastic crisis in chronic hemolytic anemia,1 for erythema infectiosum or fifth disease,2 and for arthropathies,3,4 and it has recently been isolated from the serum of patients with vascular purpura.5 We report the case of Henoch-Schonlein purpura associated with human parvovirus infection. H. T., a 6-year-old girl, without any significant past history, was admitted on March 8, 1985, for joint pain and swelling (wrists, knees, ankles) associated with intense abdominal pain.

scholarly journals The Course of Experimentally Induced Hemolytic Anemia in a Primaquine-Sensitive Caucasian

Blood ◽  
1965 ◽  
Vol 25 (1) ◽  
pp. 92-95 ◽  
Author(s):  
IVO PANNACCIULLI ◽  
ALBERTO TIZIANELLO ◽  
FRANCO AJMAR ◽  
EMANUELE SALVIDIO
Keyword(s):  
Red Blood Cells ◽  
Hemolytic Anemia ◽  
Blood Cells ◽  
Drug Induced ◽  
Glucose 6 Phosphate Dehydrogenase ◽  
Experimentally Induced

Abstract Two severe hemolytic crises, in a month’s period, were induced by primaquine in a glucose-6-phosphate dehydrogenase deficient Sardinian male. Young red blood cells tagged with Fe59 10 to 16 days earlier were destroyed in the second hemolytic episode. The implications of these experiments on the nature of drug-induced hemolysis in Caucasians are briefly discussed.

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