Akira Morimoto

..This was particularly evident for patients with multifocal SS disease, but half of those with MS disease also benefited...

....

..Several studies have suggested that Langerhans cell histiocytosis (LCH) is responsive to treatment with bisphosphonates (BPs). However the efficacy and safety of BPs therapy for childhood LCH is unknown...

..Thereafter, she successfully received hematopoietic stem cell transplantation from an HLA-matched sibling donor. Development of t-APL associated with NHL in children appears to be rare...

..However, results of previous reports have been controversial. This study was undertaken to clarify the influence of GST polymorphism on the outcome of childhood B-precursor acute lymphoblastic leukemia (ALL)...

..To assess the etiology, prognosis, and appropriate treatment of hemophagocytic lymphohistiocytosis (HLH) in neonates...

..On the other hand, in the patients having reactivation while off therapy, 6 of 7 patients showed good response. These findings suggest that 2-CdA is effective for reactivated LCH while off therapy...

..To overcome these issues, the Japan LCH Study Group-96 (JLSG-96) protocol was conducted prospectively from 1996 to 2001 in Japan...

....

..These results suggest that EVI1 overexpression was the major factor contributing to leukemogenesis, and the late appearance of the Ph chromosome is closely associated with the progression to an aggressive form of leukemia...

..Although HLH can be also observed after hematopoietic stem cell transplantation (HSCT), the incidence and clinical features of HLH after HSCT remain obscure...

..Post-transplant outcomes of hemophagocytic lymphohistiocytosis (HLH) patients were analyzed in Japan where Epstein-Barr virus (EBV)-associated severe forms are problematic...

..In the near future, an entire pathogenesis should be clarified in order to establish appropriate therapies including immunotherapy, HSCT and gene therapy...

....

..This single case report suggests that pulse Cy may be a promising therapy for steroid-refractory GVHD, especially hepatitic GVHD, but needs to be further tested in clinical trials...

..None had homozygous nonsense mutations. Our data suggest that nonsense perforin gene mutations yield early onset and missense mutations late onset in FHL2 cases...

..Classification of familial hemophagocytic lymphohistiocytosis (FHL) into FHL2, FHL3, and other subtypes based on genetic abnormalities has recently become possible. We studied the phenotypic differences among these subtypes in Japan...

..We suspect that conventional/real-time PCR failed to detect AdV due to nine silent single base substitutions in the extracted AdV genome. Treatment with 1 mg/kg cidofovir (CDV) intravenously three times a week was effective...

..Our new prognostic definition (R1, R2, R3) incorporating day 14 marrow findings is useful to tailor early-phase treatments for better therapeutic results in childhood ALL...

..In this study, the pattern of cytokine gene polymorphisms was comparatively analyzed in EBV-related diseases...

..To improve the prognosis in children with newly diagnosed acute myeloid leukemia (AML) by introducing a dose-dense intensive chemotherapy regimen and an appropriate risk stratification system...

..The patient continues to be well and in remission 3 years after stem cell transplantation...

..CML patients who respond slowly to imatinib may still be candidates for allo-SCT, even when a major cytogenetic response is obtained...

..However, detailed analyses of relapses in aplastic anemia children are limited...

..Our case suggests that non-TCD HLA-haploidentical 3-loci mismatched BMT can be safely performed from mother to offspring even when feto-maternal microchimerism is barely detectable with the current detection procedure...

..The authors investigated whether rapidly treating children with AAP by continuous regional arterial infusion (CRAI) of protease inhibitor and antibiotic would quickly resolve AAP...

..While several recent studies have focused on the role of the JAK2 V617F mutation in pediatric ET, the frequency of pediatric ET cases with this mutation and the associated clinical features remain unclear...

..These results suggest an association between the type of genetic mutation in FHL cases and the magnitude of CTL cytolytic activity and age at onset...

..Careful management is required to prevent such episodes in patients with markedly decreased coagulation factors and increased D-dimer levels following L-asparaginase administration...

..Rituximab may be a successful therapy for refractory DBA where B cells play a crucial role in the pathogenesis of the severe anemia...

....

..These findings support the diagnosis of Ewing sarcoma family of tumor. After adjuvant multidrug chemotherapy, the patient has shown no evidence of disease for more than 2 years...

..To determine useful biochemical markers in Langerhans cell histiocytosis (LCH), we analyzed the serum levels of soluble CD154 (sCD154), IL2 receptor (sIL2-R), receptor activator of NF-kappaB ligand (sRANKL), and osteoprotegerin (OPG)...

....

..These aberrant values in PB were reversed by DEX pulse therapy and correlated well with the neurological symptoms. A prospective study that assesses the efficacy of this promising and inexpensive treatment for OMS is warranted...

..T-cell subsets analysis at the onset of hepatitis might be useful for predicting development of hepatitis-associated aplastic anemia...

..The LCH-based multiagent chemotherapy including cytarabine, vincristine, methotrexate, and prednisolone ameliorated the symptoms rapidly. She was treated for 12 months and is currently doing well as a normally developing 2-year-old...

..Through a similar mechanism, we believe that MOZ, fused to an unidentified partner gene at 2p23, may have caused an alteration in histone acetylation, resulting in the development of tMDS in this patient...

..This reduced the frequency and severity of bleeding and ended the need for hospitalization. This, together with a decreased requirement for bypass agents, APCC treatment significantly reduced the cost of treatment for this patient...

..HS and Evans' syndrome have different mechanisms of pathophysiology one another. We reported the quite rare case of an infant who had these diseases concurrently. Possible explanations of the unexpected complication are discussed...

..The patient's symptoms and hypercytokinemia disappeared soon after corticosteroid treatment was started. This case demonstrates that overproduction of IL-6 is common to systemic JIA and multicentric Castleman's disease...

..To evaluate a less intensive chemotherapeutic regimen specifically designed for patients with Down syndrome (DS) and acute myeloid leukemia (AML), and to determine the prognostic factors for event-free survival...

..Conquest of aspergillus pneumonia in SCID infants could be achieved by CD34+ bone marrow cell transplantation together with appropriate anti-fungal treatment...

....

..After that treatment, X-ray photographs of the patient's body showed dense metaphyseal band sign. There have been few such cases reported until now...

..This approach may provide important clues for improving outcomes and clarifying the exact etiology in cases of often fatal, infection-associated HPS...

..Sequence analysis detected a heterozygous K196E mutation in the PROS1 gene. Type 2 PS deficiency was diagnosed. This is the first report of mesenteric vein thrombosis in a child with a type 2 PS deficiency...

..Only one case of T lineage ALL expressed a small-sized isoform of Helios (designated as He6). It was also found that the expression of Ai1 and He1 was low in Ik6-positive patients (Fisher's exact test; Ai1 P = 0.005, Hel P = 0.035)...

..The patient developed the full clinical symptoms of PGK1 deficiency including haemolytic anaemia, myopathy, central nervous system disorder and growth retardation, which is unusual...