Patterns of mortality in sickle cell disease in adults in France and England.

INTRODUCTION

An understanding of the causes of death among patients with sickle cell disease may be informative for both epidemiology and pathogenesis. This information should aid anticipation of dangerous clinical conditions, counselling patients and design of preventive therapies.

PATIENTS AND METHODS

All deaths known to four European sickle cell disease centres over a 10-year period were retrospectively analysed. The circumstances of death were classified as follows: (1) acute sickle related vaso-occlusion; (2) chronic organ failure related to sickle cell disease; (3) infection; and (4) miscellaneous causes.

RESULTS

Sixty-one adult patients (mean age: 32+/-11 years) died during the study period. Twelve patients suddenly died at home; most of them exhibited symptoms of vaso-occlusion but in eight patients, the cause of death was unknown. The primary cause of death in the 53 evaluable patients was sickle related vaso-occlusion (27 out of 53; 51%) which manifested mainly by acute multiorgan failure (n=13) and acute chest syndrome (n=9). Ten of the 27 patients (37%) who died in these circumstances had an apparent mild disease before their deaths. Ten patients (19%) died of documented infection. Ten of the evaluable patients (19%) died of a chronic terminal visceral involvement related to sickle cell disease which was mainly liver cirrhosis. Four patients died by suicide or because of refusal of care and two patients died of iatrogenic complication.

CONCLUSION

The primary cause of death in adults appears to be vaso-occlusive, even in patients with no overt organ-system failure. Our results emphasise that the circumstances of death in sickle cell disease are different between adults and children. The deaths among adults appear not to be easily assigned to a few preventable causes as they are in children.