Congenital hypoplastic anemia (CHA) associated with congenital absence of the spleen

A 19-year-old boy with congenital hypoplastic anemia (CHA) treated with long-term steroid therapy, presented with septic shock caused by Streptococcus pneumoniae. Peripheral blood smears revealed Howell-Jolly bodies and postmortem examination revealed the absence of the spleen, splenic vein and splenic artery. We have found no association of CHA and congenital asplenia previously reported in the literature or in a review of 12 additional patients with CHA at this institution. The case is reported to bring attention to the concurrence of these two rare conditions in one individual and to discuss the possible implications of this association.