Castleman's disease (CD) is a lymphoproliferative disorder of unknown origin and rarely occurs in children. It has been further classified into two major subgroups: solitary or localized and multicentric CD. Furthermore, there are two main histological variants: hyaline-vascular, plasma cell types. Clinically, hyaline-vascular type is rarely associated with systemic symptoms, but the plasma cell type is frequently associated with the constitutional symptoms of fever, malaise, night sweat and the abnormal laboratory markers. Surgical excision of the affected lymph node plays an important role in the treatment of this disease. We encountered a case of the hyaline-vascular type CD located in the mesentery with systemic involvement. The clinical and biochemical abnormal findings improved after surgical resection of the involved lymph node.
Biomarkers ; Child ; Fever ; Giant Lymph Node Hyperplasia* ; Humans ; Lymph Nodes ; Lymphoproliferative Disorders ; Mesentery ; Plasma Cells ; Sweat

Recombinant activated factor VII (rFVIIa, NovoSeven (R)) was initially developed for the treatment of bleeding in patients with hemophilia having antibodies against factor VIII or IX, and factor VII deficiency. Although the precise mode of action is still elusive and there are just several hypotheses, recently case reports have suggested a role of rFVIIa in the management of intractable or life-threatening bleeding in some non-hemophilic patients who do not respond to conventional treatments. We report the successful use of rFVIIa in a pediatric patient with intractable gastrointestinal bleeding.
Antibodies ; Factor VII Deficiency ; Factor VIIa* ; Factor VIII ; Gastrointestinal Hemorrhage* ; Hemophilia A ; Hemorrhage ; Humans

Totally implanted vascular access devices are excellent for delivering chemotherapeutic agents and prolonged intravenous infusions in patients with cancer. Catheter disconnection and migration are rare but potentially serious complications of these widely used devices. Retrieval of the migrated fragment is generally indicated but may not be possible. We hereby report three cases of catheter disconnection and migration of totally implanted vascular access devices into the heart along with the review of literatures.
Catheters ; Heart ; Humans ; Infusions, Intravenous ; Vascular Access Devices*

Despite the availability of effective antiviral drugs, human cytomegalovirus (CMV) infection is still a serious life-threatening complication in recipients of allogeneic hematopoietic stem cell transplantation. Ganciclovir (GCV) preemptive therapy is currently the usual treatment for CMV disease and antigenemia. However, prolonged GCV therapy results in the emergence of drug-resistant virus. Most GCV-resistant clinical CMV isolates contain a mutation in the UL97 phosphotransferase gene. We report a case that GCV-resistant CMV antigenemia by UL97 phosphotransferase mutant strain at codon 460 and 605 which was improved by reduction of immunosuppressants for posttransplant lymphoproliferative disease after unrelated cord blood transplantation.
Antiviral Agents ; Codon ; Cytomegalovirus ; Fetal Blood* ; Ganciclovir ; Hematopoietic Stem Cell Transplantation ; Humans ; Immunosuppressive Agents

Alveolar soft part sarcoma (ASPS) is a rare soft tissue tumor that accounts for 5% of pediatric non-rhabdomyosarcoma soft tissue sarcomas. The disease presents as a indolent asymptomatic mass and affects primarily younger patients. The clinical course is often indolent and late metastases can develop even after prolonged disease-free intervals. Radiotherapy and chemotherapy play a very limited role in the treatment. We report a pediatric case of ASPS with a brief review of the literature.
Child* ; Drug Therapy ; Humans ; Neoplasm Metastasis ; Radiotherapy ; Sarcoma ; Sarcoma, Alveolar Soft Part* ; Viperidae

Selective IgA deficiency is one of the most common primary immunodeficiency. Some patients with IgA deficiency also have deficits in one or more immunoglobulin G subclasses. It has been estimated that up to 25% of patients with certain primary immunodeficiencies will develop tumors, primarily B-cell lymphomas during their lifetime. We hereby present 2 cases of malignant lymphomas, one diffuse large cell lymphoma and another mixed cellularity Hodgkin's disease, respectively, which developed in patients with selective IgA and IgG subclass deficiency.
Child* ; Hodgkin Disease ; Humans ; IgA Deficiency ; Immunoglobulin A* ; Immunoglobulin G* ; Lymphoma* ; Lymphoma, B-Cell ; Lymphoma, Large B-Cell, Diffuse ; Lymphoma, Non-Hodgkin

Chloroma, a tumor consisted of primitive myeloid cells and located in extramedullary tissues, usually develops during or preceding the course of systemic leukemia, particularly acute myelogenous leukemia (FAB M2) with translocation between chromosomes 8 and 21. Leukemic retinopathy which observed rarely in children than adults, generally related to thrombocytopenia and anemia in leukemic patients. Bronchiolitis obliterans organizing pneumonia (BOOP) is unusual clinicopathologic syndrome in association with a variety of causes or conditions, and histologically defined by the presentation of granulation tissue plugs consisting of fibroblasts and collagen within the lumen of the distal air space. We experienced a 12-year old girl who presented with weakness and ocular pain. She was diagnosed with acute myeloid leukemia with chloroma and retinopathy, and treated with chemotherapy (daunomycin, ara-C, thioguanine, etoposide. mitoxantrone, cyclosporin). Five weeks after the chemotherapy, she developed coughing and persistent fever, and diagnosed with BOOP, which resolved completely after prednisolone therapy.
Adult ; Anemia ; Bronchiolitis Obliterans* ; Bronchiolitis* ; Child ; Collagen ; Cough ; Cryptogenic Organizing Pneumonia* ; Cytarabine ; Drug Therapy ; Etoposide ; Female ; Fever ; Fibroblasts ; Granulation Tissue ; Humans ; Leukemia ; Leukemia, Myeloid, Acute* ; Mitoxantrone ; Myeloid Cells ; Prednisolone ; Sarcoma, Myeloid* ; Thioguanine ; Thrombocytopenia

There have been advances for the treatment of childhood cancers since the recent several decades, which caused increased incidence of secondary malignancy in the populations of the long term survivors from the primary cancers. The authors report a case of acute myeloid leukemia as a secondary malignancy in a 19-year-old female after successful chemotherapy for osteosarcoma, with a brief review of related literatures.
Drug Therapy* ; Female ; Humans ; Incidence ; Leukemia, Myeloid, Acute* ; Osteosarcoma* ; Survivors ; Young Adult

We report the first case of immune mediated thrombocytopenia and concurrent hemolytic anemia (Evans syndrome) that developed during valproic acid (VPA) therapy. The 6 years-old girl had been stable with VPA therapy for seizure disorder for 5 years. She was admitted due to purpura and petechiae on both legs. Her blood test was only significant for thrombocytopenia with normal hemoglobin level. Her thrombocytopenia was not caused by VPA toxicity, because of positive anti-platelet antibody. Thrombocytopenia was improved after steroid and intravenous globulin. However, hemoglobin was shown gradual drop with increased reticulocytes counts, high level of lactic dehydrogenase, and a positive direct Coombs test. She had a complete clinical recovery with negative anti-platelet antibody and direct Coombs test after discontinuation of VPA. VPA has a lot of side effects including hematologic toxicity, although this drug is used for seizure disorders. This report is the first Evans syndrome due to VPA, even though VPA induced pure red cell aplasia with positive anti-nuclear antibody or thrombocytopenia by direct toxicity.
Anemia, Hemolytic ; Child ; Coombs Test ; Epilepsy ; Female ; Hematologic Tests ; Humans ; Leg ; Oxidoreductases ; Purpura ; Red-Cell Aplasia, Pure ; Reticulocytes ; Thrombocytopenia ; Valproic Acid*

PURPOSE: Reactive oxygen species have been recognized as a common signaling mediator in diverse stimuli-induced apoptosis and hydrogen peroxide is a natural one of those reactive oxygen species. This study was performed to investigate the role of caspases in hydrogen peroxide-induced apoptosis of HL 60 cells. METHODS: Apoptosis was induced in HL 60 cells by treating 50microM hydrogen peroxide for 2, 4, and 6 hrs and induction of apoptosis was confirmed by flow cytometry and DNA fragmentation analysis. Caspase substrate assay was used to show the activity of caspases and then protein levels of caspase and its substrate were analyzed using immunoblotting. RESULTS: During the apoptosis, caspase substrates assay showed the increased activity of caspase-3, -7, -10, but not that of caspase-8 nor caspase-9, and immunoblotting analysis showed decreasing procaspase-3 protein with the progression of apoptosis. Furthermore, with progression of apoptosis, analysis of caspase substrates showed retinoblastoma protein decreased while cleaved 89kD fragment of poly (ADP-ribosyl) polymerase protein increased. CONCLUSION: These results suggest that hydrogen peroxide-induced apoptosis in HL 60 cells is not associated with the activation of caspase-8 nor caspase-9. Rather, caspase-3 is directly activated and responsible for hydrogen peroxide-induced apoptosis of HL 60 cells.
Apoptosis* ; Caspase 3* ; Caspase 8 ; Caspase 9 ; Caspases ; DNA Fragmentation ; Flow Cytometry ; HL-60 Cells* ; Humans* ; Hydrogen Peroxide ; Hydrogen* ; Immunoblotting ; Leukemia* ; Reactive Oxygen Species ; Retinoblastoma Protein