Multiorgan dysfunction syndrome in sickle cell disease.

OBJECTIVE: Concurrent failure of multiple organs in patients of sickle cell disease (SCD) has rarely been reported. The main objective of this study was to highlight the multiorgan dysfunction (MODS) that occurs in some patients during sickle cell crisis.

METHODS: Ten episodes of multiorgan failure were identified with sickle cell disease and defining criteria of organ failure of two or more organs that is lung, liver, or renal were established according to Acute Physiological and Chronic Health Evaluation - II (APACHE-II) criteria.

RESULTS: Most episodes occurred during vaso-occlusive crisis, which was associated with a severe pain event. The onset of organ failure was associated with fever, rapid fall in haematocrit, platelet count and altered sensorium, later organ failure set in. Respiratory, hepatic, and kidney failure (all the three organs) were present in three of the ten patients who were in sickle crisis. Of this we lost one patient despite exchange transfusion and ventilator support. Of the four patients who had respiratory failure accompanied by hepatic failure, one patient died due to associated septicemia. Respiratory failure with renal involvement was seen in two patients whereas hepatic and renal failure was present in only one patient. Except for two, all other patients improved after aggressive blood transfusions.

CONCLUSION: Acute multiorgan failure is a life threatening complication of SCD, which can exhibit without any evidence of chronic organ damage and is easily reversed by prompt and aggressive transfusion therapy.