Unusual intracranial hemorrhagic complications of sickle cell disease after multiple blood transfusions: One or multiple clinical - Radiological entity?

Abstract

Cerebrovascular complications in sickle cell disease (SCD) are usually attributed to vasculopathy, endothelial dysfunction, pathological angiogenesis, and aneurysm formation. The nonaneurysmal subarachnoid haemorrhage (SAH) and intracerebral haemorrhage (ICH) as complications in SCD are less well understood and less frequent. To present a type of an unusual hemorrhagic intracranial (SAH/ICH) complications associated with additional clinical-radiological phenomena in the patient with SCD developed after multiple blood transfusions in the recovery period from sickle cell crises. A 32-years-old female known to have HbSS genotype sickle cell disease (SCD), who had a good clinical recovery from the SCD crises with multiple blood transfusions, presented with thunderclap headache and rapid clinical neurological deterioration. Nonaneurysmal SAH and convexital ICH had been diagnosed associated with reversible cerebral vasoconstriction syndrome (RCVS) in combination with a patchy vasogenic cerebral edema, resembling atypical posterior reversible encephalopathy syndrome (PRES). The patient recovered well neurologically after four weeks of hospital course and neuroimaging sign of CRVS and PRES like changes regressed completely. The patient was discharged with a mild residual pyramidal sign and a residual sign of convexital ICH on brain MRI. To the best of our knowledge this is a second case description of nonaneurysmal SAH and ICH associated with RCVS and radiological features reminding PRES developed after blood transfusions in a patient with SCD.