Successful Unrelated Cord Blood Transplantation for Epstein-Barr Virus-Associated Lymphoproliferative Disease with Hemophagocytic Syndrome

We report a case of successful umbilical cord blood transplantation (CBT) for Epstein-Barr virus (EBV)-associated lymphoproliferative disease (LPD) in a 6-year-old girl. The patient had hemophagocytic syndrome with excessive circulating levels of EBV DNA that was refractory to immunochemotherapy. Multiple hepatosplenic lesions favored the diagnosis of EBV-associated LPD, although the aggressive course precluded the histopathologic diagnosis. Unrelated CB cells mismatched at 1 HLA locus were infused after patient conditioning with 900 mg/m2 etoposide, 2 g/m2 cytarabine, 16 mg/kg busulfan, and 200 mg/kg cyclophosphamide. Complete chimeric status was obtained on day 19 posttransplantation. Drug fever and acute graft-versus-host disease of the skin (grade II) were the major complications. A transient increase of EBV DNA 1 year after CBT indicated a primary EBV infection of the donor cells. The patient is alive with no evidence of disease 27 months after CBT. There has been no previous report of successful CBT for EBV-related LPD/lymphoma. CBT can be a curative treatment for the disease, even if no viral memory has been set in the stem cell source.     

Evaluation of the pharmacokinetics of linezolid in an obese Japanese patient

We evaluated the pharmacokinetics of linezolid in the case of an obese Japanese patient (body weight 116 kg; body mass index 37 kg/m(2)). Linezolid was administered at a dose of 600 mg by intravenous drip infusion for 60-90 min at 12-h intervals. The results showed increased clearance of linezolid and a reduced serum concentration compared to population pharmacokinetic parameters, with trough levels below the 90% minimum inhibitory concentration. However, linezolid was effective for improving lung infection and inflammation in our patient, which may be due to its particularly effective transfer into lung tissues. Linezolid undergoes slow non-enzymatic oxidation in vivo that may be increased in obese patients, and this may account for the greater clearance. Our findings are useful for the planning of linezolid therapy in obese patients.     

Recurrent gastric volvulus resolved surgically after therapeutic percutaneous endoscopic gastrostomy in Duchenne muscular dystrophy

A 20-year-old man with Duchenne muscular dystrophy (DMD) with recurrent gastric volvulus underwent percutaneous endoscopic gastrostomy (PEG). Four months later, he developed vomiting and consciousness disturbance. CT revealed gastric volvulus recurrence along the gastrostomy axis. Endoscopic repositioning failed and fistula perforation necessitated emergency surgery. The upper position of the stomach was twisted counter-clockwise and revolved on the gastrostomy axis sliding between the lower stomach and abdominal wall. The fistula showed necrotic perforation and was thus resected. The anterior stomach wall was fixed to the abdominal wall at 3 triangular points. Thereafter, gastric volvulus did not recur. PEG is reportedly effective for preventing gastric volvulus, but there are rare cases of postgastrostomy recurrence. This successfully managed case provides valuable clinical insights.     

Computed tomography colonography for 5 cases of pneumatosis cystoides intestinalis

Computed tomography colonography (CTC) was performed in 5 patients with pneumatosis cystoides intestinalis (PCI). The virtual colonoscopy view of CTC as well as total colonoscopy (TCS) findings showed polypoid lesions in the colon, and multiplanar reconstruction images of the colon revealed in the polypoid lesions of the colon. We confirmed the diagnosis of PCI in all cases. CTC also detected the PCI lesions in the subserosa of the colonic wall which were not detected by TCS. Accurate evaluation of the extent of PCI involvement was obtained by CT air-contrast enema images. CTC is useful for detection of PCI lesions, assessment of the exact site and final diagnosis for PCI.     

Xanthomatous meningioma: a case report with review of the literature

Xanthomatous meningioma is an extremely rare variant of meningioma that is characterized histopathologically by the presence of tumor cells with lipid-filled vacuolated cytoplasm. In this report, we describe the fifth documented case of xanthomatous meningioma and review its clinicopathological features. A 76-year-old Japanese male presented with dizziness. Magnetic resonance imaging demonstrated a well-circumscribed tumor in the left parasagittal to frontal region with attachment of the dura mater. Histopathological examination of the resected specimen revealed proliferation of polygonal to spindle cells with eosinophilic cytoplasm and bland round to oval nuclei. Whorl formation and psammomas were scattered, and mitotic figures were rarely seen. A peculiar finding was the presence of extensive xanthomatous change continuing to the above-mentioned typical meningothelial meningioma. These tumor cells had clear vacuolated cytoplasm and bland round to oval nuclei. Immunohistochemically, xanthomatous cells were positive for epithelial membrane antigen. Accordingly, an ultimate diagnosis of xanthomatous meningioma was made. Our clinicopathological analysis revealed that xanthomatous meningioma affects children to young persons or the elderly, and four of five cases were located in the supratentorial region. Although the detailed mechanism underlying the xanthomatous change has not been clarified, this change is thought to result from a metabolic abnormality of the neoplastic meningothelial cells. Further, xanthomatous change has also been reported in atypical and anaplastic meningiomas. Therefore, it is important to recognize that xanthomatous change can occur in meningiomas, and to avoid misidentifying these cells as macrophages.