Nonimmune hydrops fetalis due to congenital xerocytosis

María Pilar Vicente-Gutiérrez1, Inés Castelló-Almazán, Maria Dolors Salvía-Roiges

  • 1Unitat Integrada de Pediatria Hospital Clínic-Hospital Sant Joan de Déu, Barcelona, Spain.

Hereditary xerocytosis is a rare hemolytic anemia in which erythrocytes are dehydrated due to a loss of potassium and water through their cell wall membrane. In adults, this condition leads to a mild-to-moderate hemolysis. We report a case of hydrops fetalis secondary to hereditary xerocytosis. Management with intrauterine erythrocyte and albumin transfusions resulted in a favorable postnatal course.