Unrelated stem cell transplantation after reduced-intensity conditioning plus rituximab for Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis with CNS involvement / 소아과

Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) with central nervous system (CNS) involvement is usually fatal unless stem cell transplant (SCT) is offered. However, SCT with conventional intensity conditioning is associated with high transplant-related mortality. We describe our experience with unrelated SCTs after reduced-intensity conditioning (RIC) for patients with EBV-HLH with progressive CNS disease. This approach was associated with minimal toxicities and might be an effective option in patients with EBV-HLH with progressive CNS disease. Moreover, the addition of rituximab to RIC appears to be safe and effective in suppressing EBV in the patients with EBV-HLH.

Diagnosis and Prediction of Clinical Outcomes in Patients with Acute Lacunar Infarction: Usefulness of Perfusion MR Imaging

PURPOSE: To correlate the findings of perfusion-weighted imaging (PWI) with clinical outcomes in patients with acute lacunar infarction. MATERIALS AND METHODS: Eleven patients (7 males and 4 females) with acute lacunar infarction who were examined within 50 (mean, 29) hours of the onset of symptoms underwent conventional MRI, diffusion-weighted imaging (DWI) and PWI. Gadolinium (0.2 mmol/kg) was injected at a rate of 2 ml/sec, and PWI was performed using a gradient-echo EPI pulse sequence and the following parameters: TR/TE, 2000/60; flip angle, 90 degree; matrix size, 128X128. Relative cerebral blood volume (rCBV) maps were derived from gadolinium bolus perfusion-weighted images where rCBV ratios between infarcted areas were detected by DWI, and contralateral control areas were obtained. In each case, the resulting rCBV ratio at a lesion site was compared with the clinical outcome determined on the basis of the difference between National Institute Health Stroke Scale (NIHSS) scores at admission and discharge. RESULTS: With the aid of the time-intensity curve obtained at PWI, the rCBV maps revealed a hypoperfused area in 10 of 11 patients, and there was positive correlation (r=0.81) with clinical outcome. CONCLUSION: Although PWI has a lower detection rate than DWI, it may be a useful modality for helping determine prognosis in cases of acute lacunar infarction.