Although the survival rate in childhood cancer is increased with current improvements of diagnostic and therapeutic methods, the incidence of second malignancy is now increasing. Therefore close follow-up with high index of suspicion for second malignancies are important for cancer survivng patients. We report our experiences of 3 second malignancies which were glioblastoma multiforme after treatment of acute lymphoblastic lymphoma, Philadelphia positive leukemia after treatment of osteosarcoma and acute myelogenous leukemia occuring in the course of chemotherapy for acute lymphoblastic leukemia. It is imperative that survivors of childhood cancer be closely followed for the detection of not only the relapse of original disease but also the occurrence of second malignancy.
Drug Therapy ; Follow-Up Studies ; Glioblastoma ; Humans ; Incidence ; Leukemia ; Leukemia, Myeloid, Acute ; Neoplasms, Second Primary* ; Osteosarcoma ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Radiotherapy ; Recurrence ; Survival Rate ; Survivors

Hepatoblastoma is a rare pediatric malignancy which frequently presents at an advanced unresectable stage. Complete surgical resection after chemotherapy is the definitive treatment for hepatoblastoma. Liver transplantation should be considered for children who have unresectable hepatoblastoma. We report a case of a 18-month-old boy with unresectable hepatoblastoma who had transplantation with pre- and post-operative chemotherapy.
Child ; Drug Therapy ; Hepatoblastoma* ; Humans ; Infant ; Liver Transplantation* ; Liver* ; Living Donors* ; Male

We report a 4.7 kg infant who received a therapeutic leukapheresis as an immediate treatment for acute lymphoblastic leukemia with severe hyperleukocytosis. By decreasing the number of circulating white blood cells, therapeutic leukapheresis helps prevent the risks of hyperviscosity and cerebrovascular and pulmonary leukostasis. In addition, it potentially reduces metabolic and renal complications associated with rapid cell lysis when applied before chemotherapy. This six-week-old female presented with vomiting for 15 days. Initial WBC count was 1,532,800/muL. After placement of 4 french two-lumen central venous catheter in both femoral vein, the CS 3000 plus was primed with 250 mL of paternal whole blood mixed with 150 mL of normal saline. After therapeutic leukapheresis, the CBC showed WBC count of 560,000/muL. Our successful experience in performing this procedure suggests that therapeutic leukapheresis be a feasible treatment even for very young infants with hyperleukocytosis.
Central Venous Catheters ; Drug Therapy ; Female ; Femoral Vein ; Humans ; Infant* ; Leukapheresis* ; Leukocytes ; Leukostasis ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Vomiting

Acute sensorineural hearing loss is unusual as initial manifestations in a child with acute lymphoblastic leukemia (ALL), even though facial or oculomotor nerve palsy as early finding of leukemia was reported. The pathology of sensorineural hearing loss in leukemia showed as leukemic cell infiltration, hemorrhage, infection, and local ischemia due to hyperviscosity. A 12-year-old boy with ALL was admitted due to multiple cervical lymphadenopathy with left sided sensorineural deafness. He complained gross hematuria and purpura on whole body. His initial complete blood cell counts were hemoglobin 11.9 g/dL, hematocrit 34.3%, white blood cells 164,000/muL (segmented neutrophils 3%, lymphocytes 11%, monocytes 2%, immature blast 84%), and platelet 28,000/muL. Pure tone audiogram revealed profound sensorineural hearing loss of the left ear at all frequencies. His brain MRI showed no definite abnormal findings without hemorrhage or infarction in inner ear or temporal lobe. He received induction chemotherapy and total 4 times of transtympanic steroid injection with 1 week interval. His hearing power at complete remission was more improved than admission, but not completely recovered until 5 months. We proposed that hearing impairment might be an initial manifestation in acute leukemia with hyperleukocytosis.
Blood Cell Count ; Blood Platelets ; Brain ; Child* ; Deafness ; Ear ; Ear, Inner ; Hearing ; Hearing Loss ; Hearing Loss, Sensorineural* ; Hematocrit ; Hematuria ; Hemorrhage ; Humans ; Induction Chemotherapy ; Infarction ; Ischemia ; Leukemia ; Leukocytes ; Lymphatic Diseases ; Lymphocytes ; Magnetic Resonance Imaging ; Male ; Monocytes ; Neutrophils ; Oculomotor Nerve Diseases ; Pathology ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Purpura ; Temporal Lobe

Antiphospholipid antibodies are a family of autoantibodies directed against certain phospholipids or phospholipid-binding proteins. Antiphospholipid antibody syndrome can occur as a secondary event to an underlying autoimmune disease, most commonly systemic lupus erythematosus and also occur in certain infectious diseases and drug reactions. We experienced a patient with Kawasaki disease who had anticardiolipin antibodies accompanied by coagulation abnormalities. A 4-month-old female patient was admitted due to fever, irritability, lymph node swelling on the right submandibular area and maculopapular skin rashes on trunk and both extremities. She had prolonged activated partial thromboplastin time (aPTT). Further investigations revealed a positive anticardiolipin antibody detected by ELISA. She had right lateral sinus thrombosis on parieto-occipital area on brain MRI and thrombotic microangiopathy, resulting in necrosis of toe. She showed considerable improvement in affected skin after dexamethasone treatment for 3 weeks. Thrombosis was resolved completely 2 months later.
Antibodies, Anticardiolipin ; Antibodies, Antiphospholipid ; Antiphospholipid Syndrome ; Autoantibodies ; Autoimmune Diseases ; Brain ; Communicable Diseases ; Dexamethasone ; Enzyme-Linked Immunosorbent Assay ; Exanthema ; Extremities ; Female ; Fever ; Humans ; Infant ; Lateral Sinus Thrombosis ; Lupus Erythematosus, Systemic ; Lymph Nodes ; Magnetic Resonance Imaging ; Meningitis* ; Mucocutaneous Lymph Node Syndrome* ; Necrosis ; Partial Thromboplastin Time ; Phospholipids ; Skin ; Thrombosis ; Thrombotic Microangiopathies ; Toes

Evans syndrome is the combination of direct Coombs' positive hemolytic anemia and immune thrombocytopenic purpura, in the absence of a known underlying etiology. Being reported rarely in pediatric patients, the syndrome is characterized by periods of remission and exacerbation with viable responses to therapy. Management of the disease remains a challenge despite a variety of therapeutic trials. We experienced a 11-years old male patient of Evans syndrome who was initially presented as having an autoimmune hemolytic anemia 17 months before. Over the 5 years of follow-up, he had a chronic, relapsing courses, showing partial responses to a variety of therapeutic trials, including IVIG, oral prednisolone, methylprednisolone pulse therapy, cyclosporine A and vincristine. A brief review of the literature ensues with the case report.
Anemia, Hemolytic ; Anemia, Hemolytic, Autoimmune ; Child ; Cyclosporine ; Follow-Up Studies* ; Humans ; Immunoglobulins, Intravenous ; Male ; Methylprednisolone ; Prednisolone ; Purpura, Thrombocytopenic, Idiopathic ; Vincristine

PURPOSE: Intracranial hemorrhage (ICH) in hemophilia patients is the most common cause of death in Korea. Early suspicion and prompt treatment of ICH is a very important for saving their life and minimizing neurologic sequelae. We investigate the prognosis of the hemophilia patients with ICH who registered in Daegu & Kyungpook area. METHODS: We evaluated the clinical courses, laboratory findings, brain image, effect of treatment and prognosis of ICH. Nine of 161 patients registered in our Department of Pediatrics suffered 16 episodes of ICH from Dec. 1996 to Mar. 2002. RESULTS: All were male hemophilia A patients (severe; 5, moderate; 3 and mild; 1) with median age of 48 (17~312) months at diagnosis of ICH. Two patients who had inhibitor became negative and one patient who had found inhibitor on annual routine follow-up has had inhibitor until now. One had trauma history and seven had family history. The median time interval from first symptom to hospital visit was 7 hours (15 minutes~10 days). Chief complaints were vomiting in 6 patients, headache in 4, seizure in 3 and mental change in 1. All patients except one were initially given factor VIII concentrate, 50 units/kg and then continuous infusion, 2~3 units/kg/hour. One patient who had factor VIII inhibitor was given factor IX concentrates, 100 units/kg with activated prothrombin complex (FEIBA ), 75 units/kg at every 12 hours. All except one with hematoma in cerebellar vermis and the third ventricle are alive without any neurologic sequela. CONCLUSION: ICH in hemophilia patients can be severe with no trauma history, or can be recurred several times with life-threatening event. So, it is desirable to have early treatment, prophylaxis, regular follow-up and patient education about abnormal symptoms to reduce the complications of them.
Brain ; Cause of Death ; Daegu ; Diagnosis ; Factor IX ; Factor VIII ; Follow-Up Studies ; Gyeongsangbuk-do ; Headache ; Hematoma ; Hemophilia A* ; Humans ; Intracranial Hemorrhages* ; Korea ; Male ; Patient Education as Topic ; Pediatrics ; Prognosis ; Prothrombin ; Seizures ; Third Ventricle ; Vomiting

PURPOSE: Retrovirs-mediated transduction of target genes into bone marrow progenitor cells or peripheral lymphocytes has been less optimal due to low efficiency and minimal expression on long-term analysis. This study aims to establish an efficient 4-day culture condition for the increased transduction efficacy into CD34+ cells selected on umbilical cord blood by comparing combination of various cytokines. METHODS: CD34+ cells from umbilical cord blood selected by Isolex-50R were incubated with supernatant containing XM5/PA317 vector for 96 hours. Cytokine combinations were used including IL-6+SCF, IL-6+IL-3+SCF, and IL-6+IL-3+SCF+TPO. Methylcellulose colony assay was done after culture. The data were expressed as mean+/-SD with 3 experiments. The efficiency of gene transfer was assessed by the ability of transduced CFU-GM to grow in the presence of G418 and PCR analysis of individual CFU-GM. RESULTS: The mean recovery rate of CD34+ cells after purification was 22%, and the purity of the final CD34+-enriched fraction was 82+/-13% (mean+/-SD). After a 4-day culture, the cell number increased 5~10 fold in each culture condition. The transduction efficiency evaluated by both G418-screened CFU-GM and PCR-positive CFU-GM with the above cytokine combinations was 46% and 64%, 41% and 57%, and 28% and 45%. However, there were no significant differences of colony counts between the cytokine combinations. CONCLUSION: We were unable to establish the best recipe of cytokine combination as the number of experiments was small and we tried only a fixed concentration of cytokines. For the future, the study of developing a novel vector, a better condition of transduction, and better combination of cytokines is warranted to attain the goal of highly effective, long-lasting method of gene transfer.
Bone Marrow ; Cell Count ; Cytokines ; Fetal Blood* ; Granulocyte-Macrophage Progenitor Cells ; Lymphocytes ; Methylcellulose ; Polymerase Chain Reaction ; Stem Cells ; Umbilical Cord*

Pichia ohmeri is an yeast-like fungus used in the food industry for fermentation. This organism has been implicated in human disease only in a few case reports. We describe herewith two cases of Pichia ohmeri fungemia in immunocompromised pediatric patients with central venous catheters. A 7-year-old patient with Burkitt's lymphoma undergoing chemotherapy and a newborn with low birth weight developed fungemia during hospitalizations. Both patients were receiving parenteral nutrition through central venous catheters. Both patients succumbed despite empiric treatment with amphotericin B in Case 1. A brief review of the literature ensues with the case reports.
Amphotericin B ; Burkitt Lymphoma ; Central Venous Catheters ; Child ; Drug Therapy ; Fermentation ; Food Industry ; Fungemia* ; Fungi ; Hospitalization ; Humans ; Infant, Low Birth Weight ; Infant, Newborn ; Parenteral Nutrition ; Pichia*

Self-healing reticulohistiocytosis is a perinatal disease characterized by rapidly developing and involuting benign histiocytic infiltration of the skin and other organ. We had recently experienced a case of a self-healing reticulohistiocytosis in 99-day-old infant who presented with multiple erythematous nodular lesions on face and trunk and pancytopenia. Our case differed from those previously reported in that extracutaneous involvement was found. The patient had pancytopenia, hypertriglyceridemia, hypofibrinogenemia hyperferritinemia and hepatosplenomegaly as well as skin lesions. Bone marrow showed a marked lymphocytosis with many histiocytes. We described a detailed clinical features of this case and reviewed the literatures.
Bone Marrow ; Histiocytes ; Humans ; Hypertriglyceridemia ; Infant ; Lymphocytosis ; Pancytopenia ; Skin