Sa. Zimmerman et Re. Ware, Palpable splenomegaly in children with haemoglobin SC disease: Haematological and clinical manifestations, CLIN LAB H, 22(3), 2000, pp. 145-150
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.