Acute splenic complications in children with sickle cell-hemoglobin C disease

To determine the frequency and severity of acute splenic complications in children and adolescents with sickle cell (SC) hemoglobin C disease.

The medical records of 271 patients with SC disease seen at our center were reviewed to evaluate the incidence and severity of acute complications involving the spleen.

Sixteen (6%) children had acute splenic complications. Thirteen (5%) had 16 episodes of acute splenic sequestration (ASSC), with the initial event occurring at a mean age of 8.9 years (range, 2 to 17 years). Splenomegaly had been noted before the initial event in 6 (46%) of the 13 cases, and 3 (23%) had a history of painful splenic infarction. Two young children (aged 4 and 6 years) had a hemoglobin value less than 2 gm/dl, one without history of splenic enlargement. Three (23%) children had a second episode of ASSC. Three additional patients had a history of acute painful splenic infarction, two of whom also had splenic hemorrhage. Eight (3%) of the 271 children required splenectomy (1 after the initial episode of ASSC, 3 after a second episode of ASSC, 2 as a result of pain accompanying chronic infarction and ASSC, and 2 because of splenic hemorrhage). No deaths resulted from ASSC.

We conclude that (1) acute splenic complications in children and adolescents with SC disease are relatively uncommon, (2) most episodes of ASSC occur in preadolescents, (3) ASSC can be life threatening, even in younger children, and (4) prior splenomegaly is not a good predictor of ASSC. Thus it is vital that the parents of all children with SC disease be instructed to palpate their child's spleen regularly.