A five-month-old male infant with familial hemophagocytic lymphohistiocytosis underwent cord blood transplantation using reduced-intensity conditioning. Methylprednisolone (mPSL) pulse administration was performed for marked pulmonary edema during the early phase of transplantation, followed by GVHD treatment with mPSL until day 100. CMV antigenemia was detected on days 27 and 55, but serum became negative with 2- to 3-week ganciclovir (GCV) treatment on both occasions. On day 120, ophthalmological findings included multiple bilateral white spots and a positive PCR study using anterior chamber fluid confirmed the diagnosis of CMV retinitis affecting both eyes, although CMV antigenemia was negative. Re-treatment with GCV had a minimal effect on the ophthalmological findings, while foscarnet administration markedly improved the retinitis and decreased the CMV-DNA level. Considering that a substantial proportion of patients develop CMV retinitis even when CMV antigenemia is not present, routine monitoring involving ophthalmological examinations should be conducted for hematopoietic transplant patients, especially infants, who cannot complain of ocular symptoms.
Endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) was performed as a diagnostic procedure for two pediatric patients with intra-abdominal tumors. Case 1 was an 8-year-old boy with a huge tumor in the portal-hepatic region. Case 2 was a 17-year-old girl with a history of diabetes and recurrent relapse of Burkitt lymphoma, who had a newly developing tumor in the pancreatic body. In both cases, EUS-FNA was performed as a less invasive diagnostic procedure than open biopsy or total resection of the tumor. Tumor cells were determined to be of the B cell lineage by flow cytometric and immunostaining analyses. Both cases were diagnosed as having Burkitt lymphoma based on detection of IgH/C-MYC translocation by FISH. The aspiration was successfully conducted without severe complications, and both patients were immediately given chemotherapy. EUS-FNA is a safe and minimally invasive procedure with high diagnostic value for pediatric cases with intra-abdominal tumors.
To clarify the differences in the clinical features between idiopathic interstitial pneumonia (IIP) and interstitial pneumonia associated with collagen vascular diseases (CVD-IP).Symptoms, radiographic findings, pulmonary function, blood chemistry data including autoantibody, and bronchoalveolar lavage fluid (BALF) findings were compared using multiple logistic regression analysis.The subjects were 44 patients clinically diagnosed with IIP and 33 patients with CVD-IP.The clinical features related to IIP were as follows: male sex, advanced age, past history of hypertension, presence of cough, exertional dyspnea, digital clubbing, an increased level of gamma-globulin, decreased lung volume on chest X-ray, and typical type according to the criteria for IIP on chest X-ray. Increased levels of rheumatoid factor and total cell number in BALF were related to CVD-IP.These findings are considered to be useful to differentiate IIP and CVD-IP.
A 31-year-old nulliparous Japanese woman visited the clinic due to worsening dysmenorrhea. A cystic endometriotic lesion was found in the vesico-uterine pouch. Laparoscopic surgery was chosen due to the severe dysmenorrhea. Her first oocyte retrieval attempt was performed at in-vitro fertilization clinic before the planned surgery. However, she complained of abdominal pain on day 6 after the retrieval. We diagnosed her with peritonitis with an abscessed cystic endometriotic lesion in the vesico-uterine pouch. Conservative treatment was ineffective. Therefore, laparoscopic surgery was performed. The cysts in the vesico-uterine pouch were drained of pus. No adhesions or lesions of endometriosis in the uterus, bilateral adnexa, or pelvic peritoneum were found. Although cystic endometriotic lesions in the vesico-uterine pouch are rare, they can form abscesses after oocyte retrieval. The possibility of abscesses formation risk must be considered. Moreover, following the management of endometrioma, sufficient medication should be administered to prevent this formation.
10021 Background: The Japanese Study Group for Pediatric Liver Tumor (JPLT) has conducted cooperative treatment studies on hepatoblastoma (HB) since 1991. The main aim in JPLT-2 study launched in 1999 and closed in 2012 to evaluate the efficacy of cisplatin/pirarubicin in risk-stratified HB. This protocol was launched in 1999 and closed in 2012 to evaluate the cure rate of risk-stratified HB: standard risk HB (a tumor involving three or fewer sectors of the liver), intermediate risk HB (a tumor involving all sectors of the liver or invasion into portal or hepatic vein) and high risk HB (a tumor involving all sectors of the liver or with metastasis). Methods: Until 2012, 385 HB children who were younger than 15 years of age were eligible for inclusion in the JPLT2 study in which the cisplatin/pirarubicin regimen (CITA) is kept as the first line. In this study, we examined the outcome and late effects of the HB patients by the risk-stratified three groups (standard, intermediate and high risk groups). Results: Among 385 cases, PRETEXT I was 31, II was 120, III was 145, and IV was 89 including 86 cases (18%) with metastatic tumors. The 3-year EFS/OS of the cases with standard risk HB were 94/82%, while those of the cases with Intermediate and high risk HB was 64/49% and 34/28%, respectively. Except for 40 cases who underwent primary resection, complete resection of primary after CITA was performed 86% of standard risk, 66% of intermediate risk and 56% of high risk patients. And the late phase complications were 5 cases with maldevelopment, 17 with cardiac complications, 26 with ototoxicity and 5 with second malignancies. Conclusions: As compared with other multicenter cooperative protocols, CITA regimens achieved similar rates of survival and resectability in standard risk patients. More promising strategies including adequate liver transplantation and new targeting drugs should be developed for intermediate and high risk HBs. Clinical trial information: UMIN000001116.
The SIOPEL-4 study has demonstrated that dose-dense cisplatin-based chemotherapy dramatically improves outcome in children with high-risk hepatoblastoma in western countries. However, the feasibility and safety of this regimen have not been clarified in Japanese patients.A pilot study, JPLT3-H, was designed to evaluate the safety profile of the SIOPEL-4 regimen in Japanese children with newly diagnosed hepatoblastoma with either metastatic disease or low alpha-fetoprotein.A total of 15 patients (three female) were enrolled. Median age was 2 years (range, 0-14). Three patients were PRETEXT II (where PRETEXT is PRETreatment EXTent of disease), six PRETEXT III, and six PRETEXT IV. All patients had lung metastasis, none had low alpha-fetoprotein. Eight patients completed the prescribed treatment, and seven patients discontinued therapy prematurely, four due to progressive disease and three due to causes other than severe toxicity. Grade 4 neutropenia was documented in most patients in preoperative cycles A1-3 (11/15 in A1, 9/11 in A2, and 7/11 in A3) and in all considering all cycles. Grade 3-4 thrombocytopenia and grade 3 anemia were also frequently observed. Patients experienced several episodes of grade 3 febrile neutropenia, but none had grade 4 febrile neutropenia or severe infections. One patient had grade 3 heart failure only in the first cycle. Other grade 3 or 4 toxicities were hypomagnesemia, anorexia, nausea, mucositis, liver enzyme elevation, fever, infection, and fatigue. There were no unexpected severe toxicities.The toxicity profile of JPLT3-H was comparable to that of SIOPEL-4. Dose-dense cisplatin-based chemotherapy may be feasible among Japanese patients with high-risk hepatoblastoma.
