Persistent splenomegaly and hypersplenism in sickle cell disease - abstract

ABSTRACT

Hypersplenism, arbitrarily defined as a spleen more than 3 cm below the left costal margin, associated with an haemoglobin level below 6.5 gm/dl and platelet counts below 260 x 10/1, for at least one month, occurred in 38/308 (12.3 percent) children with SS disease in a cohort study followed from birth. Comparison of clinical and haematological features in 12 patients (10 SS; 2 S§ thalassemia), with splenomegaly but without hypersplenism, indicated that hypersplenic patients had significantly shorter red cell survival (mean 3.8 days, p=0.04), lower red cell volumes (p=0.03), higher plasma volumes (p=0.003) and higher blood volumes (p=0.02). These patients also had greater diploic expansion (p = 0.05) and greater cardiomegaly (p =0.0d). Following splenectomy, there were highly significant increases in haemoglobin levels, red cell volume, red cell survival, and platelet counts, and decreases in reticulocytes and plasma volume. Height velocity over the year following splenectomy increased in 7/9 patients, with adequate data, at ages when height velocity is normally falling. Hypersplenism is characterized by severe anaemia, rapid haemolysis, and bone expansion; the metabolic cost of the erythropoietic hyperactivity may compete with the demands for normal growth. Splenectomy is the optimal method of treatment in such cases (AU)