[A case of chronic active Epstein-Barr virus infection with a pharyngeal ulcer].

Chronic active Epstein-Barr virus infection (CAEBV) is characterized by chronic or recurrent infectious mononucleosis-like symptoms, such as fever, extensive lymphadenopathy, and hepatosplenomegaly. A 44-year-old women visited our ENT clinic with a four-month history of fever and throat pain. She was diagnosed as having CAEBV based on the findings of fever, liver dysfunction, lymphadenopathy, pharyngeal ulcer, the titer for IgG to the EBV capsid and pathological findings. The whole-blood EBV DNA levels were high and above 3.7 x 10(3) copies/mL. After administration of intravenous predonine (1000 mg/day for 3 days) and oral predonine (1.5 mg/kg. 60 mg/day), the liver dysfunction and pharyngeal ulcer improved. Since the prognosis is poor in adult cases of CAEBV, chemotherapy is scheduled for this case.

Two cases of peritonsillar abscess complicated by von Willebrand disease.

Von Willebrand disease (vWD) is a common hereditary bleeding disorder resulting from a quantitative and/or qualitative deficiency of von Willebrand factor (vWF). We report two cases of peritonsillar abscess complicated by vWD. A 46-year-old Japanese man was intravenously administered factor VIII clotting antigen (500U×3 days)and platelet transfusion (10U), when before puncture was performed. After puncture, his symptoms promptly improved with the administration of the antibiotic doripenem (DRPM, 1.5g/day). He left our facility one week later and had no recurrence of symptoms. A 24-year-old Japanese woman was intravenously administered factor VIII clotting antigen (4500U×3 days) and desmopressin (DDAVP) before undergoing a puncture. Her symptoms promptly improved with DRPM treatment (1.5g/day). The patient left our facility one week later. However, the peritonsillar abscess recurred in three weeks. Afterwards, tonsillectomy was enforced three months later. Intravenous factor VIII clotting antigen (4500U×2 days) and platelet transfusion (10U×1 day) had been used before tonsillectomy. We therefore suggest that a peritonsillar abscess in patients with vWD can be safely treated by factor VIII clotting antigen and DDAVP at the appropriate disease stage and by performing paracentesis for the acute phase or tonsillectomy for the chronic phase.

Vascular endothelial growth factor produced in nasal glands of perennial allergic rhinitis.

BACKGROUND: Vascular endothelial growth factor (VEGF), a pleiotropic polypeptide that mediates endothelial cell-specific responses such as induction of angiogenesis and vascular leakage, is hyperproduced in a variety of inflammatory disorders. In asthma, VEGF hyperproduction promotes mucosal edema by enhancing vascular leakage. However, in allergic rhinitis, details of the pathophysiological importance remain unclear. This study was designed to investigate and discuss the pathophysiological significance of VEGF in nasal secretions from perennial allergic rhinitis sufferers. METHODS: Seven allergic rhinitis patients sensitized with house-dust mites and 12 chronic rhinosinusitis patients were enrolled. Nasal secretion VEGF was quantified and compared between groups. In allergic rhinitis cases, nasal lavage VEGF was estimated before and after the antigen provocation. Nasal gland VEGF was immunohistochemically investigated. VEGF messenger RNA (mRNA) levels in serous and mucous acini were analyzed by laser microdissection and light cycler-polymerase chain reaction. RESULTS: VEGF levels in nasal secretions and nasal lavage from allergic rhinitis were higher than in nonallergic rhinosinusitis, after rather than before antigen provocation. VEGF mRNA expression was higher in serous versus mucous acini. These results are consistent with the immunohistochemistry results. CONCLUSION: In allergic rhinitis, there was significant VEGF production in serous acini, which was hypersecreted after antigen provocation. VEGF may play an important role in pathophysiology of allergic rhinitis.

Ewing's sarcoma/primitive neuroectodermal tumour occurring in the maxillary sinus.

A 12-year-old boy complained of swelling of the left cheek. Fiberscopic examination revealed the presence of a soft reddish mass in the middle meatus of the left nostril. CT scan showed a large mass completely filling the left maxillary sinus. The lesion originated from the maxillary sinus and extended to the middle nasal meatus; bone destruction and invasion of the subcutaneous tissue of the cheek were noted. T2-weighted MRI images revealed a heterogeneous signal in the left maxillary sinus. Under general anaesthesia, biopsies were obtained through an intraoral incision. On pathology, atypical cells containing irregular nuclei with scanty cytoplasm were noted. The tumour cells were strongly positive for CD99 and reacted weakly with NSE however the cells were negative for synaptophysin, LCA and cytokeratin on immunohistochemical examination. Based on these findings, the tumour was diagnosed as a Ewing's sarcoma/primitive neuroectodermal tumour. The patient was treated with radiotherapy and combination chemotherapy; subsequently, the tumour's size decreased markedly. After 20 months of follow-up, the patient showed no evidence of local tumour growth or metastasis.