Saada Véronique, Cynober Thérèse, Brossard Yves, Schischmanoff Pierre Olivier, Sender Alfred, Cohen Henri, Delaunay Jean, Tchernia Gil
Laboratoire d'Hématologie, d'Immunologie et de Cytogénétique, Hôpital de Bicêtre, APHP, and Faculté de Médecine Paris-Sud, Le Kremlin Bicêtre, France.
Pediatr Hematol Oncol. 2006 Jul-Aug;23(5):387-97. doi: 10.1080/08880010600646662.
As most of hereditary spherocytosis-affected individuals experience jaundice at birth, it seemed of interest to evaluate the proportion of hereditary spherocytosis in 402 severely jaundiced neonates with a bilirubinemia level prompting phototherapy. Red cell dehydration, a hallmark of spherocytosis whether constitutional or acquired, was demonstrated in 74 of them, among whom 23 disclosed a typical pattern of spherocytosis upon red cell deformability studies. Acquired spherocytosis of immune origin was diagnosed in 19/23 and hereditary spherocytosis in 4, making the proportion of hereditary spherocytosis-affected individuals among a severely jaundiced population of neonates amount to 1%, an incidence at least 30-fold that of the overall population.
