Palpable splenomegaly in children with haemoglobin SC disease: Haematological and clinical manifestations

This study aimed to investigate the prevalence of palpable splenomegaly in children with haemoglobin SC (Hb SC) disease, and to determine the haematol ogical and clinical manifestations of splenomegaly in this patient populati on. We performed a retrospective chart review of 100 patients with Hb SC ov er 2 years of age followed by the Duke University Paediatric Sickle Cell Pr ogram with serial physical examinations and laboratory measurements. Palpable splenomegaly was present in 34% of patients and was more common in males (P = 0.029). Children with splenomegaly had a significantly lower av erage haemoglobin concentration (10.3 vs. 10.8 g/dl, P = 0.011) and lower p latelet count (237 vs. 314 x 10(9)/l, P < 0.001) than those without splenom egaly. Children with measurements both before and after the onset of spleno megaly had a significant decrease in the platelet count (279 vs. 216 x 10(9 )/l, P < 0.001) and white blood cell count (9.1 vs. 7.9 x 10(9)/l, P = 0.04 ) after splenomegaly was identified. Clinical complications included acute splenic sequestration in 12% of children (median age 5.4 years), and hypers plenism with chronic thrombocytopenia in another 10% of patients (median ag e 10.6 years). Splenomegaly is a common physical finding in children with Hb SC disease an d is often associated with mild cytopenias. Clinical complications of splen omegaly include acute splenic sequestration in younger patients and hypersp lenism with chronic thrombocytopenia in older children.