Reliability and Validity of the Japanese Pediatric Version of Memorial Symptom Assessment Scale.

Context: Few instruments in Japanese assess health-related quality of life in pediatric cancer patients.

Objectives: To translate the Memorial Symptom Assessment Scale (MSAS) into Japanese pediatric and proxy versions (MSAS-J 7-12, MSAS-J 13-18, and MSAS-J-Proxy) and assess validity and reliability.

Methods: Phase I comprised forward-backward translation and pilot testing in 13 children and 16 guardians.

Rhinocerebral Mucormycosis With Temporal Artery Thrombosis in an Adolescent Following HLA-haploidentical Stem Cell Transplantation.

Rhinocerebral mucormycosis (RCM) can lead to internal carotid artery thrombosis. Here, we report the first case of RCM with temporal artery thrombosis following HLA-haploidentical stem cell transplantation in an adolescent presenting with low-grade fever, right mandibular pain, and right jaw claudication. This case suggests that RCM can cause temporal artery thrombosis and should be considered as a differential diagnosis in severely immunocompromised patients with maxillary sinusitis presenting with jaw claudication.

Inflammatory serum cytokines and chemokines increase associated with the disease extent in pediatric Langerhans cell histiocytosis.

Objective: Langerhans cell histiocytosis (LCH) is characterized by immature dendritic cell proliferation, infiltration of LCH lesions by various inflammatory cells, and a lesional cytokine storm. It is classified into three groups on the basis of disease extent, namely, multisystem with risk-organ involvement (MS+), multisystem without risk-organ involvement (MS-), and single-system (SS) disease. We comprehensively analyzed whether serum levels of cytokines/chemokines reflect the disease extent.

[Successful allogeneic bone marrow transplantation with reduced intensity conditioning regimen for a pediatric relapsed ALK positive anaplastic large cell lymphoma].

Pediatric anaplastic large cell lymphoma (ALCL) is a chemosensitive malignancy, but about 30% of patients experience relapse. In most of these patients, a second complete remission is obtainable with salvage chemotherapy, though relapse free survival rates are as low as 30-60%. Herein, we report a 6-year-old boy with relapsed anaplastic lymphoma kinase (ALK) positive ALCL successfully treated with vinblastine monotherapy followed by allogeneic hematopoietic stem cell transplantation (allo-HSCT), with a reduced intensity conditioning (RIC) regimen, from his father.

An Infant With Medulloepithelioma Successfully Treated by High-dose Chemotherapy Followed by Autologous Peripheral Blood Stem Cell Transplantation Without Radiotherapy.

Medulloepithelioma of the central nervous system (CNS) is a rare primitive neuroectodermal tumor characterized by highly malignant behavior occurring in early childhood. Few cases have been reported and optimal management remains unknown. Here, we report a case of CNS medulloepithelioma successfully treated with high-dose chemotherapy (HDCTX) followed by autologous stem cell transplantation (auto-SCT) without radiotherapy.

Monitoring of anti-L-asparaginase antibody and L-asparaginase activity levels in a pediatric patient with acute lymphoblastic leukemia and hypersensitivity to native Escherichia coli L-asparaginase during desensitization courses.

A patient with acute lymphoblastic leukemia who was hypersensitive to native Escherichia coli L-asparaginase (L-asp) underwent readministration of the L-asp without serious adverse effects for 11 doses using a desensitization protocol every time. Monitoring of anti-L-asp antibody and L-asp activity levels revealed that the serum L-asp activity was below the effective levels during the administration of first 6 to 7 doses because of extremely high levels of anti-L-asp IgG. Sustained L-asp activity was attained since the eighth dose was administered, when the antibody levels were <5 U/mL.

Successful treatment with pulse cyclophosphamide of a steroid-refractory hepatitic variant of liver acute graft-vs.-host disease in a child.

A 13-yr-old boy with recurrent acute myeloid leukemia underwent HSCT using cells from an unrelated donor who matched all HLA antigens except one. Forty-two days later, the patient developed a steroid-refractory hepatitic variant of liver GVHD with peak ALT and T.Bil values of 1406 mU/mL and 10.

Cost effectiveness of prophylactic treatment with activated prothrombin complex concentrate in a patient with inhibitor-positive hemophilia A.

Patients with hemophilia and high titers of inhibitors are hard to treat during bleeding events and consequently are more likely to incur high treatment costs and to experience deterioration in quality of life. We report here the case of a boy with hemophilia A and high titers of inhibitors who responded well to prophylactic activated prothrombin complex concentrate (APCC) treatment. Previously, he had to be hospitalized frequently because of painful bleeding of target joints of the knee and ankle.

A marked decrease in CD4-positive lymphocytes at the onset of hepatitis in a patient with hepatitis-associated aplastic anemia.

A 10-year-old Japanese boy developed acute hepatitis with high levels of serum Torque teno virus DNA and marked lymphocytopenia, especially CD4 T-lymphocytopenia. Although the total lymphocyte counts rose as the patient recovered from hepatitis, this was largely because of a marked rise in CD8 cells. In contrast, CD4 cells recovered poorly, resulting in a further striking fall in the CD4/8 ratio.

Unilaterally and rapidly progressing white matter lesion and elevated cytokines in a patient with Tay-Sachs disease.

We report the case of a girl with Tay-Sachs disease who had convulsions and deteriorated rapidly after an upper respiratory infection at the age of 11 months. At the age of 16 months, her seizures became intractable and magnetic resonance imaging of the brain showed high signal intensity on T2-weighted images and marked swelling in the white matter and basal nucelei of the right hemisphere. Her seizures and right hemisphere lesion improved with glycerol and dexamethasone treatment.