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Severe hemolytic anemia in black children with glucose-6-phosphate dehydrogenase deficiency
Insights
Hemolytic anemia in black children with glucose-6-phosphate dehydrogenase (G-6-PD) deficiency can be severe, often triggered by infections. Distinctive red blood cell changes like eccentrocytes are common findings.
Area of Science:
- Pediatric Hematology
- Clinical Genetics
- Biochemistry
Background:
- Hemolytic anemia due to oxidant stress in glucose-6-phosphate dehydrogenase (G-6-PD) deficient Black children is not well-documented in pediatric literature.
- G-6-PD deficiency is an X-linked genetic disorder affecting red blood cells, leading to hemolysis upon exposure to certain triggers.
Purpose of the Study:
- To describe the clinical spectrum of hemolytic anemia in G-6-PD deficient Black children.
- To identify common triggers and characteristic clinical and laboratory findings in these patients.
Main Methods:
- A retrospective study of 14 hospitalized Black children with G-6-PD deficiency and moderate to severe hemolytic reactions over 3.5 years.
- Analysis of patient demographics, clinical presentation, triggers, treatment (including blood transfusions), and peripheral blood smear morphology.
Main Results:
- The majority of affected children (13/14) were boys under 3 years old.
- Infections (bacterial and viral) were the most common trigger (11/14 episodes), with viral syndromes associated with more severe hemolysis.
- Naphthalene exposure caused 3 episodes; oxidant drugs were not implicated. Distinctive red blood cell morphology, including eccentrocytes, was observed in most subjects. Nine patients required blood transfusions.
Conclusions:
- Hemolytic reactions in G-6-PD deficient Black children can be severe and are most frequently associated with infections.
- Characteristic erythrocyte morphology, such as eccentrocytes, is a common finding and aids in diagnosis.
- Early recognition and avoidance of triggers are crucial for managing this condition in pediatric populations.
Abstract:
The clinical spectrum of hemolytic anemia as a consequence of oxidant stress in black children deficient in erythrocyte glucose-6-phosphate dehydrogenase (G-6-PD) has not been well described in the pediatric literature. During a 3 1/2-year period 14 hospitalized black G-6-PD-deficient children with moderate to severe hemolytic reactions were studied. The vast majority (13/14) were boys and were less than 3 years of age. Nine of the patients required blood transfusion. Eleven of the 14 episodes occurred with infection (five bacterial, six viral); those children with viral syndromes tended to have more severe hemolysis. Naphthalene was responsible for three episodes, but oxidant drugs were implicated in no instances. Findings on the blood smears of most subjects included irregular dense misshapen erythrocytes with asymmetrical distribution of hemoglobin and an adjacent membrane-bound clear zone ("eccentrocytes"). It is concluded that hemolytic reactions in the black G-6-PD-deficient child may be severe, are most commonly associated with infection, and are frequently characterized by distinctive erythrocyte morphology.