Splenic rupture in children with hematologic malignancies

BACKGROUND: Splenic rupture is an uncommon but life-threatening complication of leukemias and lymphomas, and is reported mostly in adults. The authors investigated the frequency with which splenic rupture is diagnosed in pediatric patients with hematologic malignancies and reviewed its clinical profile and outcome. METHODS: The data base of St. Jude Children's Research Hospital was searched for cases coded as splenic laceration or rupture, splenic infarction, or splenectomy in patients diagnosed with lymphoma or leukemia between January 1962 and December 1997. The medical records of patients with histopathologic or radiologic evidence of splenic rupture were reviewed. The time spanned by the study was divided into early (1962-1990) and recent (1991-1997) eras to reflect the availability of modern diagnostic imaging techniques. RESULTS: Seven children experienced splenic rupture. They were between ages 5-17 years. There were four males and three females. Primary diagnoses included acute myeloid leukemia (four patients), acute lymphoblastic leukemia (two patients), and Hodgkin lymphoma (one patient). Five patients were diagnosed in the recent era and two in the early era. Four patients had radiologic or bacteriologic evidence of fungal infection concomitant with the splenic event. Of five deaths, only two were related causally to splenic rupture; these occurred in the early era. All seven acute episodes of splenic rupture were managed conservatively without surgery. CONCLUSIONS: The overall frequency with which splenic rupture was detected in children with hematologic malignancy at the study institution was 0.18%. In the recent era, the frequency of detection was 9-fold higher (0.55%) than that of the early era (0.06%). Improved imaging techniques and increased utilization of imaging studies may account for the increased incidental detection of "preclinical" splenic rupture. Adolescent age group, acute myeloid leukemia (especially acute promyelocytic leukemia), a high leukocyte count, thrombocytopenia, and coagulopathy may predispose children with leukemia to pathologic splenic rupture. Fungal infection frequently was associated with splenic rupture and may play a role in its pathogenesis.