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Glucose-6-phosphate dehydrogenase deficiency and hematopoietic stem cell transplantation
1Department of Medicine, University of Hong Kong, Queen Mary Hospital, Hong Kong.
Insights
Glucose-6-phosphate dehydrogenase (G6PD) deficiency is an X-linked condition. Testing all stem cell transplant donors and recipients for G6PD deficiency is recommended, especially in susceptible populations, due to its potential impact.
Area of Science:
- Hematology
- Genetics
- Enzymology
Background:
- Glucose-6-phosphate dehydrogenase (G6PD) deficiency is a common X-linked enzymopathy.
- It affects approximately 3% of Southern Chinese males, presenting as a hemolytic condition.
Purpose of the Study:
- To investigate the incidence and clinical implications of G6PD deficiency in adult hematopoietic stem cell transplantation (SCT) recipients and donors.
- To assess the impact of G6PD deficiency on engraftment and hemolysis post-SCT.
- To explore the prevalence of G6PD deficiency in female patients with chronic myeloid leukemia (CML).
Main Methods:
- Retrospective analysis of 275 allogeneic and 107 autologous SCT cases.
- Screening for G6PD deficiency in both donors and recipients.
- Monitoring for hemolysis and red cell engraftment post-transplantation.
- Statistical comparison of G6PD deficiency incidence in different patient groups.
Main Results:
- G6PD deficiency was identified in 1.8% of allogeneic SCT pairs and 3.7% of autologous SCT patients.
- Female CML patients showed a significantly higher incidence of G6PD deficiency compared to the general female population.
- No significant hemolysis or delayed engraftment was observed in most G6PD-deficient individuals; most converted to donor G6PD status post-SCT.
Conclusions:
- G6PD deficiency screening for all SCT donors and recipients in susceptible populations is advisable.
- There is a potential association between G6PD deficiency and multi-lineage clonal marrow disorders, particularly in females.
- While generally well-tolerated post-SCT, G6PD deficiency warrants consideration in transplant protocols.
Abstract:
Glucose-6-phosphate dehydrogenase (G6PD) deficiency is an X-linked hemolytic enzymopathy affecting 3% of Southern Chinese males. Among 275 adult allogeneic hematopoietic stem cell transplantations (SCT), five cases (1.8%) each of donors and recipients were G6PD deficient. Among 107 autologous SCT, four patients (3.7%) were G6PD deficient. All subjects were male, except for two female patients with chronic myeloid leukemia (CML). The incidence of G6PD deficiency in female CML patients was significantly higher than the background female incidence (P = 0.004), but comparable with that in the males (P = 0.664). There was no significant hemolysis or delay in red cell engraftment, and all but one patient converted to donor G6PD screening status. One female patient achieved partial correction of her G6PD status and relapsed at 10 months. We suggest that G6PD deficiency should be tested for in all marrow donors and recipients in susceptible populations. From our data, there is a suggestion of increased clinical incidence of G6PD deficiency in female patients with multi-lineage clonal marrow disorders.