Publication

Abstract : Very severe acquired aplastic anemia (VSAA) is a uniformly fatal disease if untreated. The standard of care now is allogeneic stem cell transplantation or immunosuppressive therapy including antithymocyte globulin (ATG).[1] We describe an interesting case of posterior reversible encephalopathy syndrome (PRES) developing post ATG in a case of very severe aplastic anemia. A 10-year-old male child presented with a fever, neutropenia and mucosal bleeding. He was diagnosed with VSAA 6 months back and received oral cyclosporine with no response. He was admitted, cyclosporine was stopped and intravenous antibiotics were started with blood product support. After 1 month of this episode, he was started on horse ATG (ATGAM, Pharmacia) at a dose of 40 mg/kg/day for 4 days. Prednisolone was initiated at 1 mg/kg on day +1. Apheresis platelets were transfused daily. On day +4, he developed neck pain and global headache. His blood pressure (BP) was normal and there was no focal deficit. Injectable tramadol was given for pain relief. He completed the last day of ATG with worsening of neck pain at the end of infusion. There was no associated vomiting and the platelet count was 67 × 10 9 /L. On day +5, he had a generalized tonic clonic seizure controlled by intravenous phenytoin. BP was elevated (174/100 mm of Hg) for which he was given oral amlodipine. Serum electrolytes were normal. Non-contrast head computed tomography showed a small old bleed in the right occipital cortex with areas of hypodensity in right parieto-occipital region. A presumptive diagnosis of PRES was considered and magnetic resonance imaging Brain was performed. T2 and fluid-attenuated inversion recovery hyperintensities were seen in bilateral occipital white matter adjacent to left occipital horn of lateral ventricle [Figure 1]. His BP normalized and remained normal until discharge without any further antihypertensive therapy. Phenytoin was tapered and the patient was switched to levetiracetam for seizure prophylaxis. Steroids were continued in view of generalized itching and maculopapular rash suggestive of serum sickness. The family requested for discharge due to the financial reasons. At the time of discharge, he was on levetiracetam and prednisolone. There had been no seizure recurrence until discharge. He presented a day later in hypertensive encephalopathy with subarachnoid hemorrhage and acute left frontoparietal hemorrhage. He developed repeated seizures and was intubated. He went into cardiac arrest and died on day +17 of ATG.