Symptoms can be quite variable. The
erythrocytic fragility and hepatic enzyme immaturity may cause significant
neonatal jaundice. Whether or not occasional acute aplastic crises
(especially after parvovirus infection) is revealing; the clinical picture
is mainly that of an anemia that may vary from mild to severe.
Hyperbilirubinemia, predisposing to cholelithiasis, elevated plasma lactate
dehydrogenase, splenomegaly, endocrine dysfunctions, and acute renal failure
following hemolytic crisis, is not unusual. Splenectomy markedly improves