Severe pyruvate kinase deficiency anemia. A case report.
BACKGROUND
Pyruvate kinase deficiency is a rare cause of hemolytic anemia and, in its most severe form, requires splenectomy in childhood. During pregnancy, severe cases have been traditionally managed with prophylactic blood transfusions to keep the hemoglobin concentration above arbitrary thresholds of 7-8 g/dL.


METHODS
A case of severe pyruvate kinase deficiency anemia was managed conservatively without blood transfusions even though the hemoglobin concentration reached a nadir of 6.8 g/dL. The perinatal outcome was good.


CONCLUSIONS
In cases of severe pyruvate kinase deficiency anemia, pregnancy per se might not be an indication for prophylactic blood transfusions.
Ghidini
A
A
Perinatal Diagnostic Center, Alexandria Hospital, Virginia, USA.
Korker
V L
VL
D000328:Adult; D000740:Anemia / Q000209:etiology* / Q000628:therapy; D005260:Female; D006454:Hemoglobins; D006801:Humans; D011247:Pregnancy; D011248:Pregnancy Complications / Q000517:prevention & control*; D011256:Pregnancy Outcome; D011295:Prenatal Care; D011770:Pyruvate Kinase / Q000172:deficiency*
D006454:Hemoglobins; D011770:Pyruvate Kinase