Congenital acute myeloid leukemia with t(8;16) and t(17;19) double translocation: case presentation and literature review.

Congenital leukemia is uncommon and excluding transient myeloproliferation associated with Down syndrome, makes up approximately 1% of childhood leukemias. A newborn boy was born with multiple subcutaneous nodules and large purpuric papules. Skin biopsy revealed proliferation of atypical hematologic cells in the dermis. Bone marrow morphology was consistent with acute myeloid leukemia (M5) and cytogenetic studies revealed t(8;16) and t(17;19) double translocation. Although prognosis of congenital leukemia is known to be dismal, recent reports showed spontaneous remissions. With the fear of chemotherapy-related toxicity, to treat or not to treat may be a dilemma both to parents and pediatricians. We report our experience and review the literature.

Idarubicin plus behenoyl cytarabine and 6-thioguanine compares favorably with idarubicin plus cytarabine-based regimen for children with previously untreated acute myeloid leukemia: 10-year retrospective, multicenter study in Korea.

We investigated the outcome of idarubicin plus N(4)-behenoyl-1-beta-D-arabinofuranosyl cytosine (BHAC)-based chemotherapy (BHAC group, n=149) compared to idarubicin plus cytarabine-based chemotherapy (cytarabine group, n=191) for childhood acute myeloid leukemia (AML). Between January 1996 and December 2005, 340 children with AML from 5 university hospitals in Korea received the BHAC-based or cytarabine-based chemotherapy, with or without hematopoietic stem cell transplantation. After induction therapy, 264 (77.6%) of 340 children achieved a complete remission (CR) and 43 (12%) achieved a partial remission (PR). The CR rate in the BHAC group was higher than in the cytarabine group (85.2% vs. 71.7%, P=0.004). However, the overall response rate (CR+PR) was not different between the two groups (93.3% vs. 87.9%, P=0.139). The 5-yr estimates of overall survival (OS) of children in the two groups were similar (54.9% for the BHAC group vs. 52.4% for the cytarabine group, P=0.281). Although the results were analyzed according to the treatment type and cytogenetic risk, the OS showed no significant difference between the BHAC group and the cytarabine group. In the present study, the clinical outcomes of the BHAC-based chemotherapy, consisting of BHAC, idarubicin, and 6-TG, are comparable to that of the cytarabine-based chemotherapy for childhood AML.

Comparable outcomes of HLA-matched unrelated and HLA-identical sibling donor bone marrow transplantation for childhood acute myeloid leukemia in first remission.

We retrospectively investigated the outcomes of HLA-matched unrelated BMT (MU-BMT, n = 13) and HLA-identical sibling donor BMT (MS-BMT, n = 17) for childhood AML in CR1 between June 2002 and August 2005. Engraftment of neutrophil and platelet did not differ between the two transplant groups. The cumulative incidence of grade II-IV acute GVHD and any chronic GVHD at three yr was not different between MS-BMT and MU-BMT. Of the 30 patients, four patients experienced relapses (three with MS-BMT, one with MU-BMT) and four patients died of transplant-related complications (two with MS-BMT, two with MU-BMT). A total of 23 patients survived with a median follow-up of 43.2 months. The Kaplan-Meier estimates for EFS rates at three yr were 71% and 77% for MS-BMT and MU-BMT, respectively, and the OS rates were 76% and 77% for MS-BMT and MU-BMT, respectively. The outcome of HLA-matched unrelated BMT is comparable to that of HLA-identical sibling BMT for childhood AML in CR1. HLA-matched unrelated BMT may be recommended for patients who have AML in CR1 without an HLA-matched sibling donor.

Disseminated Kikuchi-Fujimoto disease mimicking malignant lymphoma on positron emission tomography in a child.

Kikuchi-Fujimoto disease is a rare and benign disease, characterized by regional cervical lymphadenopathy with fever of unknown origin, affecting mainly young women. This self-limiting disease of unknown etiology has rarely been reported in children, so it has been frequently confused with malignancies. We describe a case of a child who was initially mistaken for malignant lymphoma because of multiple lymph nodes with 18-fluorodeoxyglucose uptake on positron emission tomography and was finally diagnosed with Kikuchi-Fujimoto disease by excisional biopsy of the affected lymph node.

Optic disc edema as a possible complication of Imatinib mesylate (Gleevec).

BACKGROUND: Imatinib is effective for a variety of hematologic and nonhematologic malignancies. Well-known ocular side effects are periorbital edema, epiphora, extraocular muscle palsy, and blepharoconjunctivitis. However, optic disc edema has not been reported as a complication of imatinib mesylate. Herein, we describe a patient with chronic myeloid leukemia (CML) who developed optic disc edema during treatment with imatinib. CASE: A 14-year-old Korean girl was referred to ophthalmology for a 3-week history of photopsia in both eyes. She had started taking imatinib daily 2 months previously for CML. At the initial exam, slit lamp showed optic disc edema in both eyes, even though visual acuity was 20/20 and other optic nerve function evaluations were within normal limits. We recommended to the oncologist discontinuation of the imatinib treatment. OBSERVATIONS: When the patient was followed for 1 week after stopping imatinib treatment, the frequency of photopsia decreased and the optic disc edema improved. Because a second examination 3 weeks after discontinuation of imatinib revealed much improved optic disc edema, she restarted the imatinib treatment. No ocular side effects have been noted so far. CONCLUSIONS: Optic disc edema should be considered one of the complications associated with imatinib. We propose discontinuation of the treatment for a short period when optic disc edema occurs.

Isolation and characterization of a novel platelet aggregation inhibitory peptide from the medicinal mushroom, Inonotus obliquus.

This study describes the extraction and characterization of a platelet aggregation inhibitory peptide from Inonotus obliquus. Ethanol extract from I. obliquus ASI 74006 mycelia showed the highest platelet aggregation inhibitory activity (81.2%). The maximum platelet aggregation inhibitory activity was found when the mycelia of I. obliquus ASI 74006 was extracted with ethanol at 80 degrees C for 12 h. The platelet aggregation inhibitor was purified by systematic solvent fractionation, ultrafiltration, Sephadex G-10 column chromatography, and reverse-phase HPLC. The purified platelet aggregation inhibitor is a novel tripeptide with a molecular mass of 365 Da, having a sequence of Trp-Gly-Cys. The purified platelet aggregation inhibitor also showed high platelet aggregation inhibitory activity in Institute of Cancer Research (ICR) mice.

Isolation and characterization of a novel glutathione S-transferase-activating peptide from the oriental medicinal plant Phellodendron amurense.

The aim of this study was to elucidate the characteristics of glutathione S-transferase (GST)-activating compounds from medicinal plants. Among 265 kinds of medicinal plants, Phellodendron amurense showed the highest GST activity at 174.8%. The GST-activating compound of P. amurense was maximally extracted when treated with distilled water at 30 degrees C for 12 h. The compound was purified by ultrafiltration, Sephadex G-10 gel filtration chromatography, and reverse-phase HPLC. The purified GST-activating compound from P. amurense was a novel tetrapeptide with an amino acid sequence of Ala-Pro-Trp-Cys and its molecular weight was estimated to be 476 Da. It also displayed a clear detoxicative effect in 1-chloro-2,4-dinitrobenzene treated mice at a dosage of mg/kg body weight.

Characterization of beta-hydroxy-beta-methylglutaryl coenzyme A reductase inhibitor from Pueraria thunbergiana.

This study describes the extraction and characterization of an inhibitor for beta-hydroxy-beta-methylglutaryl (HMG) coenzyme A (CoA) reductase from Pueraria thunbergiana. The maximum HMG-CoA reductase inhibitory activity (IC(50) = 79 microg) was obtained when P. thunbergiana was extracted with 70% ethanol at 30 degrees C for 12 h. After purification of the HMG-CoA reductase inhibitor by means of systematic solvent extraction, silica gel column chromatography, and HPLC, an active fraction with an IC(50) of 0.9 microg (4.25 microM) and a yield of 1.3% was obtained. The purified HMG-CoA reductase inhibitor was identified as daidzein (C(15)H(10)O(4); molecular mass, 254 Da).

Effect of Ganoderma lucidum on the quality and functionality of Korean traditional rice wine, yakju.

The goal of this study was to develop a high value Korean traditional rice wine possessing the pharmaceutical functionality of Ganoderma lucidum. The effects of the fruiting body of G. lucidum on the alcohol fermentation of Korean traditional rice wine, yakju, were investigated. Optimal fermentation conditions for the preparation of G. lucidum-yakju consisted of the koji added at 15% and a fermentation period of 15 d at 25 degrees C. The effects of the amount of G. lucidum added on the acceptability and functionality of G. lucidum-yakju were investigated. G. lucidum GL-1 yakju brewed by adding 0.1% G. lucidum into the mash showed the best acceptability and its angiotensin I-converting enzyme (ACE) inhibitory activity and SOD-like activity were 63% and 42%, respectively, both of which are higher than those of yakju. The high ACE inhibitory activity of G. lucidum GL-1 yakju was found to result from ganoderic acid K in G. lucidum on the basis of physical and spectral data. However, the fibrinolytic activity and antioxidant activity of G. lucidum GL-1 yakju were very low, while tyrosinase inhibitory activity was not determined. From these results, G. lucidum GL-1 yakju may become a new functional Korean traditional rice wine with antihypertensive properties.

Derivative-assisted classification of fractured zones crossing a deep borehole.

In this study, the derivative analysis using the derivative of drawdown with respect to log-time was utilized to determine candidates for hydraulic conductor domains (HCDs). At a 500-m deep borehole in the study site, the fractured rocks crossing the borehole were first classified in fractured and nonfractured zones by core logging and geophysical loggings, such as acoustic televiewing, density, and flow loggings. After conducting the hydraulic tests such as constant head withdrawal and recovery tests at the fractured zones and the nonfractured zones, the derivative analyses were carried out, of which the results were evaluated to determine the candidates for HCDs. For the nonfractured zones, the diagnostic plot has only a big hump indicating poor connection of the background fractures to the permeable geologic media, while those of the candidates for HCDs show various flow regimes. On the basis of these results, the candidates for HCDs among the fractured zones were determined. From discussion on the results, the combination of the spacing analysis and derivative analysis following a hydraulic test is recommended for determining the candidates for HCDs rather than other geophysical loggings.

Deferasirox shows in vitro and in vivo antileukemic effects on murine leukemic cell lines regardless of iron status.

Numerous studies have shown the antiproliferative effect of iron chelating agents (ICAs), which have been used traditionally in patients with secondary iron overload (SIO). Because the in vivo model for these studies has been animals with normal iron status, the antileukemic effect of ICAs in the SIO condition has not been determined clearly. We investigated the in vitro and in vivo effects of ICAs in murine leukemic cell lines regarding the iron status. The viability of both EL4 cells and L1210 cells incubated with either deferoxamine (DFO) or deferasirox (DFX) decreased in a concentration-dependent manner. This effect was most prominent in L1210 cells treated with DFX. The viability of L1210 cells incubated with both ICAs did not change regardless of the presence of ferric chloride. The percentage of apoptosis in L1210 cells treated with DFO or DFX increased in a concentration-dependent manner; however, the expression of Fas showed no significant change. The non-SIO mice and SIO mice bearing L1210 cells showed longer survival than other groups when treated with DFX, whereas the SIO mice treated with DFO showed shorter survival than the control group. The tumor was significantly smaller in the SIO mice treated with DFX or DFO compared with the control group. The iron content of the liver or the tumor in SIO mice decreased after ICA treatment. This study indicates an antileukemic effect of DFX regardless of iron status and suggests that the use of DFX has a survival benefit for SIO leukemia murine model in terms of iron chelation and antileukemic therapy.

Prognostic implications of the NIH consensus criteria in children with chronic graft-versus-host disease.

PURPOSE: In this study, we analyzed a cohort of children with chronic graft-versus-host disease (GvHD) according to the NIH consensus classification (NCC) in order to observe whether global assessment at diagnosis correlates with GvHD-specific endpoints. We then studied the clinical course of these patients, specifically with regards to episodes of GvHD exacerbation requiring treatment escalation. MATERIALS AND METHODS: Recipients of either allogeneic bone marrow transplantation (BMT) or peripheral blood stem cell transplantation (PBSCT) from January 2006 to August 2008 at the Department of Pediatrics, The Catholic University of Korea were evaluated for chronic GvHD, which was diagnosed according to the NCC. The course of chronic GvHD in these patients was then followed. RESULTS: Of 59 evaluable patients, 23 developed chronic GvHD for a cumulative incidence of 39.3%. Upon multivariate analysis, previous acute GvHD (≥grade II) had a significant impact on chronic GvHD incidence. With a median duration of systemic treatment for chronic GvHD of 501 days, no significant relationship was found between initial global severity of chronic GvHD and either duration of immunosuppressive treatment or final clinical response to treatment. Fifteen patients (65%) experienced at least one episode of chronic GvHD exacerbation during the period of follow-up, with a median of four exacerbations in the subgroup of patients who experienced such events. Lung GvHD resulted in the highest number of exacerbations per diagnosed patient, followed by oral GvHD. CONCLUSION: Analysis of this small cohort indicates that global assessment as proposed by the NCC may have limited correlations with GvHD-specific endpoints, possibly due to the favorable response of children to treatment.

Acute colonic pseudo-obstruction in postchemotherapy complication of brain tumor treated with neostigmine.

Acute colonic pseudo-obstruction is characterized by dilatation of the large bowel without mechanical obstruction. Although the first step of the treatment is conservative management, mechanical decompression should be performed when symptoms persist. Recently, the efficacy of pharmacologic treatment has been reported in adults, but no such data have yet been reported in children for treatment of acute colonic pseudo-obstruction resulting from chemotherapy. We report a 9-year-old boy with acute colonic pseudo-obstruction caused by chemotherapy for brain tumor who did not respond to initial supportive therapy, but who was successfully treated with neostigmine.

Effect of a Pholiota adiposa Extract on Fat Mass in Hyperlipidemic Mice.

The purpose of this study was to investigate the effect of a Pholiota adiposa extract on fat mass in hyperlipidemic mice fed on a high-fat diet. The water extracts from P. adiposa (ASI 24018) were not affected in the total triglyceride contents and epididymal fat mass in mice fed on a high-fat diet, but the retroperitoneal fat mass decreased significantly. This result suggests that the P. adiposa extract may be a potential candidate for use as a functional food that can act as a prophylactic against hyperlipidemia. However, the P. adiposa extract showed no effect in the total triglyceride contents and epididymal fat mass.