Acute splenic sequestration and hypersplenism in the first five years in homozygous sickle cell disease.

Abstract

SUMMARY A cord blood screening programme initiated in June 1973 had screened 68 000 normal deliveries by February 1979 with the detection of 216 cases of homozygous sickle cell disease. Regular review of these children in the Medical Research Council paediatric clinic has identified acute splenic sequestration as a major cause of morbidity and mortality in the first 5 years of life. In addition to classical episodes characterised by peripheral circulatory failure, minor episodes of increasing anaemia associated with an enlarging spleen and an active marrow were also common. These minor episodes appeared to have predictive value in children who later developed severe life-threatening episodes of acute splenic sequestration. Sustained hypersplenism was also appreciably more common in children developing minor or major episodes of acute splenic sequestration compared with those without such a history. It is proposed that the classification of acute splenic sequestration be expanded to include these minor episodes, and that consideration be given to prevention of recurrences by splenectomy particularly in patients who also develop sustained hypersplenism.

Acute splenic sequestration crisis in sickle cell disease: cohort study of 190 paediatric patients

“…ASSC is defined as acute splenic enlargement with a fall in the haemoglobin (Hb) level of at least 20 g/l and a normal basal reticulocyte count (Topley et al, 1981). To date, no factors predicting ASSC have been identified.…”

Sequestro esplênico agudo em coorte de crianças com anemia falciforme

“…8,13,14 There was no statistically significant association between sex and development of ASS, similar to the findings by other authors. 4 The probability curve for occurrence of the first episode of ASS ( Figure 1) indicates that 27.1, 33 and 37% of the children are likely to have suffered the first episode of ASS at the age of 2, 3 and 5 years, respectively. The Jamaican estimate, 8 using a similar methodology, was 22.5, 26.5 and ASS = acute splenic sequestration.…”

“…4,[8][9][10][11] There is no evidence of a seasonal pattern suggesting an infectious cause. Clinically, the episodes are considered a medical emergency: patients may show signs of hypovolemic shock, progressing to death within a few hours.…”

“…[3][4][5][6][7] The episodes show prevalence of 7.5 to 30%, occurring more frequently between 3 months and 5 years of age (76% before 2 years of age) and in patients with homozygous SS hemoglobinopathy.…”

“…Some reports demonstrate high recurrence and mortality rates. 4,[6][7][8][12][13][14][15][16][17][18][19] There is still controversy concerning subsequent management. Options include: careful clinical observation, periodic packed red blood cell transfusion regimens and splenectomy.…”