Chronic graft-versus-host disease(GVHD), found in 30~50% of long-term survivors after an HLA-identical sibling transplant, in a multi-organ syndrome resembling collagen vascular diseases. Although the skin is the most commonly involved organ, generalized vitiligo with poliosis has rarely been reported as a manifestation. Patient 1 underwent an allogeneic peripheral blood stem cell transplant when late graft failure developed after initial bone marrow transplant(BMT) for aplastic anemia. She suffered from Grade III GVHD involving gut and skin, and developed cough, wheeze and dyspnea along with decreased pulmonary function. The diagnosis of bronchiolitis obliterans in association with chronic GVHD was made, and treatment with steroid and hydroxychloroquine(HCQ) resulted in some improvement. Patient 2 showed de novo chronic GVHD after allogeneic BMT for aplastic anemia. The vitiligo with premature grayness of hair and eyelash was the manifestation of chronic GVHD. Steroid and HCQ treatment stabilized the skin manifestations. HCQ treatment, though a larger study is needed, seemed to be safe adjuvant in the management of chronic GVHD in children.
Anemia, Aplastic ; Bone Marrow ; Bone Marrow Transplantation ; Bronchiolitis Obliterans* ; Bronchiolitis* ; Child ; Collagen ; Cough ; Diagnosis ; Dyspnea ; Graft vs Host Disease* ; Hair ; Humans ; Hydroxychloroquine ; Siblings ; Skin ; Skin Manifestations ; Stem Cells ; Survivors ; Transplants ; Vascular Diseases ; Vitiligo*

Early diagnosis of childhood cancer is very important for the prognosis. Ultrasonography of routine examination for pregnant woman is now a popular method. We experienced two cases of congenital Wilms tumor suspected by prenatal ultrasonography from mothers and confirmed by nephrectomy soon after birth. One of the cases was stage II with favorable histology and was treated with vincristine(2.0 mg/m2) and actinomycin D(45 microgram/kg) for 60 weeks by National Wilms Tumor Study(NWTS) IV treatment protocol, K-4 A regimen. The other was stage I with favorable histology and was treated with vincristine(1.5 mg/m2) and actinomycin D(15 microgram/kg) for 24 weeks by NWTS IV, EE regimen. The post-chemotherapy follow-up evaluation of these two children for 3 years and 2 months and 1 year and 7 months respectively were quite normal with 100% of Karnovsky scores. Early diagnosis, especially in prenatal, is very important for the prognosis of childhood cancer. Routine ultrasonographic examination in pregnant women is also desirable for the detection of solid tumor of fetus if it does not harmful for the fetus.
Child ; Clinical Protocols ; Dactinomycin ; Early Diagnosis ; Female ; Fetus ; Follow-Up Studies ; Humans ; Mothers ; Nephrectomy ; Parturition ; Pregnant Women ; Prenatal Diagnosis* ; Prognosis ; Ultrasonography ; Ultrasonography, Prenatal ; Wilms Tumor*

Angioimmunoblastic lymphadenopathy with dysproteinemia(AILD), first described at early seventies, is a clinico-pathologic syndrome which has common features of lymphadenopathy, hepatosplenomegaly, fever, rash and dysproteinemia. It is a lymphoproliferative disease, differs from malignant lymphoma by the high frequency of constitutional symptoms and by the presence of generalized lymph node enlargement, hepatosplenomegaly, cutaneous menifestations and polyclonal hypergammaglobulinemia at the time of clinical onset. Peripheral T cell lymphomas(PTCL) are morphologically and immunologically heterogeneous group of lymphoproliferative disorders that are composed of postthymic lymphocytes. There are group of cases showing features similar to AILD, and these cases are classified as AILD like PTCL. We report a rare case of AILD like PTCL in an 8 year old boy, who visited Seoul National University Hospital with fever, hepatosplenomegaly and cervical lymphadenopathy, and diagnosed as AILD like PTCL by lymph node biopsy. He did not respond to conventional chemotherapy and died 1 year after clinical onset during chemotherapy, showing feature of multiple brain infarction of unknown etiology.
Biopsy ; Brain Infarction ; Child ; Drug Therapy ; Exanthema ; Fever ; Humans ; Hypergammaglobulinemia ; Immunoblastic Lymphadenopathy* ; Lymph Nodes ; Lymphatic Diseases ; Lymphocytes ; Lymphoma ; Lymphoma, T-Cell, Peripheral* ; Lymphoproliferative Disorders ; Male ; Paraproteinemias ; Seoul

A 5-year-old boy developed painful erythematous indurated patch at vertex that rapidly progressed to central necrotic ulceration with peripheral granulation after chemotherapy for acute lymphoblastic leukemia. Cultures at the lesion sites demonstrated a fungus, Aspergillus niger, as the etiologic agent. No evidence of involvement of other organs or of systemic dissemination by Aspergillus niger was found. Surgical debridement was performed and the patient received treatment with intravenous Amphotericine B followed by oral itraconazole. No evidence of recurrent fungal infection was noted during several months of follow-up.
Amphotericin B ; Aspergillosis* ; Aspergillus niger ; Child* ; Child, Preschool ; Debridement ; Drug Therapy ; Follow-Up Studies ; Fungi ; Humans ; Itraconazole ; Male ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Skin ; Ulcer

We have recently encounted a case of Kasabach-Merritt syndrome which were characterized by hemangioma, thrombocytopenia, and disseminated intravascular coagulation. The skin of anterior neck and tongue was involved by large mass of hemangioma. Patient was taken ventilator care from upper airway obstruction of hemangioma. He was placed on combined therapy with steroid and interferon alfa-2a for 13weeks. Blood platelet count increased to acceptable range(>20000/mm3) after 10weeks of interferon alfa-2a treatment and the hemangioma decreased in size. But, he expired from failure of airway maintenance, uncontrollable bleeding and septic shock on hospital day 93. A brief review of the literature ensues with the case report.
Airway Obstruction* ; Disseminated Intravascular Coagulation ; Hemangioma ; Hemorrhage ; Humans ; Interferons* ; Kasabach-Merritt Syndrome* ; Neck ; Platelet Count ; Shock, Septic ; Skin ; Thrombocytopenia ; Tongue ; Ventilators, Mechanical

Congenital combined deficiency of vitamin K dependent coagulation factors is a rare coagulation disorder. We experienced a 20-month old boy who was found to have a congenital vitamin K dependent coagulation factor defeciency. He presented with continuous bleeding on lacerated hard palate and had a history of numerous hemorrhagic episodes with multiple bruises after birth. Laboratory finding showed prolonged prothrombin time and partial thromboplastin time. Blood coagulation work-up showed marked decreased activities of the coagulation factors II, VII, IX, X and the natural anticoagulants proteins C and S. Assay of coagulation factors in the parents and sibling were with the normal range. There's no evidence of malabsorption, liver disease or ingestion of a coumarin compound. Response to intravenous administration of vitamin K1 was not significant but transfusion of fresh frozen plasma corrected prothrombin time and partial thromboplastin time. We reported a case of congenital combined deficiency of vitamin K dependent coagulation factors.
Administration, Intravenous ; Anticoagulants ; Blood Coagulation ; Blood Coagulation Factors ; Contusions ; Eating ; Hemorrhage ; Humans ; Infant ; Liver Diseases ; Male ; Palate, Hard ; Parents ; Partial Thromboplastin Time ; Parturition ; Plasma ; Prothrombin Time ; Reference Values ; Siblings ; Vitamin K 1 ; Vitamin K* ; Vitamins*

Paroxysmal cold hemoglobinuria(PCH), a rare type of autoimmune hemolytic process caused by a complement-binding IgG anti-P biphasic hemolysin, is associated with a positive Donath-Landsteiner(D-L) test. Historically, PCH has been associated with syphilis, but is now most often seen following viral infections especially in children. Epstein-Barr virus(EBV), a member of Herpesviridae, can cause various hematologic complications such as neutropenia, thrombocytopenia, aplastic anemia, virus associated hemophagocytic syndrome and hemolytic anemia. We report a case of PCH following EBV infection confirmed by EBV panel test and D-L test. The patient, a 4-year-old male was seen at Yonsei Medical Center in April, 1997, complained high fever, jaundice and hematuria for 2 days. Direct antiglobulin test using anti-C3d monovalent antisera was strong positive, but was negative against anti-IgG monovalent antisera. D-L test showed a characteristic biphasic hemolysin pattern. EBV panel test showed primary infection with a result of EBV EA(early antigen) IgM(+), EBV EA IgG(-), EBV NA(nuclear antigen) IgG(-). He was improved with transfusion of packed red cells and discharged on 13 th hospital day. He was completely recovered and then no relapse occurred during follow up. PCH is thought to be a rare form of autoimmune hemolytic anemia, but recent studies suggest that PCH may account for a large percentage of cases of autoimmune hemolytic anemia as acute transient form, especially in children. Therefore, in unexplained hemolytic anemia, more careful serological examination and attention will result in high detection rate of PCH and cause of PCH.
Anemia, Aplastic ; Anemia, Hemolytic ; Anemia, Hemolytic, Autoimmune ; Child ; Child, Preschool ; Coombs Test ; Epstein-Barr Virus Infections ; Fever ; Follow-Up Studies ; Hematuria ; Hemoglobinuria, Paroxysmal* ; Herpesviridae ; Herpesvirus 4, Human* ; Humans ; Immune Sera ; Immunoglobulin G ; Jaundice ; Lymphohistiocytosis, Hemophagocytic ; Male ; Neutropenia ; Recurrence ; Syphilis ; Thrombocytopenia

BACKGROUND: Cord blood(CB) has been suggested as an alternate source of stem cells in transplantation because of lower incidence of graft versus host disease and ease of collection. To optimize storage space cord blood needs to be stored as separated product. Additionally, for cord blood bank to be economical and efficient, volumes smaller than that of whole packs needs to be stored. However, CB processing procedures described so far involve open systems and/or reagents which are not licensed for use in humans. Consequently, these procedures poorly match the needs for closed-system, large-scale CB banking. In our study, we use the triple bag system in which the CB is separated by centrifugation to maintain of sterility and volume reduction. And simple and effective RBC depletion method was evaluated with 10% pentastarch and 4% modified gelatin. METHODS: The collection of CB was performed from 45 full term newborns. The collection was made by gravity and uterine contraction before placental expulsion with triple transfusion bag containing CPD-A1. 10% Pentastarch(Group A) or 4% modified gelatin(Group B) is added directly to the CB containing bag. After RBC sedimentation, the WBC-rich supernatant is collected in a satellite bag. After RBC depletion, recovery of mononuclear cells(MNC), CD34+ cells were compared between two groups. RESULTS: Mean volume of collected CB and final RBC-depleted products were 68.1+/-17.0 mL and 18.1+/-4.1 mL in Group A and 68.0+/-15.8 mL, 23.1+/-6.3 mL in Group B, respectively. After RBC depletion, the number of MNC and CD 34+ cells were 2.61+/-1.51x108, 1.19+/-0.96x106 in Group A and 4.03+/-3.16x108, 1.43+/-0.86x106 in Group B. Mean recovery of MNC, CD34+ cells were 70.2+/-43.7%, 60.2+/-31.5% in Group A, and 84.7+/-39.9% and 87.7+/-7.5% in Group B, respectively. CONCLUSION: CB processing in closed-system using triple bag with 4% modified gelatin appears to be safe, easy, effective and particularly suitable for large-scale CB banking.
Centrifugation ; Fetal Blood* ; Gelatin ; Graft vs Host Disease ; Gravitation ; Humans* ; Hydroxyethyl Starch Derivatives ; Incidence ; Indicators and Reagents ; Infant, Newborn ; Infertility ; Stem Cells ; Umbilical Cord* ; Uterine Contraction

PURPOSE: Umbilical cord blood as the source of hematopoietic stem cells has several advantages over bone marrow cells for transplant purpose, but the limitation inherent in the cord blood is its limited number of hematopoietic stem cells. The responses of cord blood mononuclear cells (MNC) which were stimulated by unrelated cord blood MNC or adult peripheral blood MNC to investigate the possibility of unrelated, HLA-mismatched multiple cord blood transplantation were evaluated. METHODS: Nineteen cord blood and 7 adult peripheral blood samples were used. Each of the MNC from cord blood and adult peripheral blood was cultured before and after 2,000 cGy irradiation. Mixed MNC cultures with cord blood and irradiated different cord blood or irradiated adult peripheral blood were performed. RESULTS: The mean percentages of MNC counts compared to day 0 of day 2 and day 7 cultures of mixed MNC cultures with cord blood and irradiated different cord blood were 92.5 55.8%, 57.4 51.0% and those of mixed MNC cultures with cord blood and irradiated adult peripheral blood were 143.4 132.7%, 113.4 95.1%, respectively. The difference of the cell count changes in 2 types of MNC cultures was statistically significant (P<0.05). CONCLUSION: The proliferative responses of cord blood MNC to different cord blood MNC were significantly lower than those to adult peripheral blood MNC. The study for the investigation of clinical applicability of cord blood transplantation from multiple unrelated donors to overcome the limitation of the cell dose of cord blood transplantation and the shortage of related or unrelated HLA-matched donors is needed.
Adult* ; Bone Marrow Cells ; Cell Count ; Cell Culture Techniques* ; Fetal Blood* ; Hematopoietic Stem Cells ; Humans ; Tissue Donors ; Unrelated Donors

PURPOSE: A sensitive assay to detect minimal residual disease in neuroblastoma is necessary for accurate assessment of disease status and optimal treatment. In this study, we compared the usefulness of sensitive methods, flow cytometry and RT-PCR for the detection of minimal residual disease in neuroblastoma. METHODS: Eighteen patients who were newly diagnosed and treated at Severance Hospital since 1999 were included in this study. Samples from bone marrow, peripheral blood, and peripheral blood stem cell product were examined for tumor cell contamination by RT-PCR (TH RT-PCR) to detect tyrosine hydroxylase mRNA and by flow cytometry identifying CD9+/CD56+/CD45- cells. RESULTS: We analyzed 20 cases from 18 patients, which were assayed by both methods at the same time. Among 20 cases, 16 cases showed same results, which were compatible with histologic results and clinical course, and 4 cases showed different results. One case of them showed positive result in histology and flow cytometry, but negative result in TH RT-PCR. The other 3 cases showed negative results in flow cytometry, but positive results in TH RT-PCR, and 1 patient of them relapsed. Among 16 patients, 2 patients, showing positive results in only TH RT-PCR, relapsed. CONCLUSION: Detection of minimal residual disease using TH RT-PCR and flow cytometry was effective and useful in evaluating disease status and deciding for proper treatment.
Bone Marrow ; Flow Cytometry* ; Humans ; Neoplasm, Residual* ; Neuroblastoma* ; RNA, Messenger ; Stem Cells ; Tyrosine 3-Monooxygenase