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Fatal Bronchiolitis Obliterans with Very Severe Subcutaneous Emphysema and Pneumomediastinum after Unrelated BMT.

Sun Hee KIM ; Hoon KOOK ; So Youn KIM ; Kyoung Ran SOHN ; Eun Song SONG ; Hyun Jung KIM ; Dong Kyun HAN ; Ho Song NAM ; Tai Ju HWANG

Korean Journal of Pediatric Hematology-Oncology.2003;10(2):299-304.

Bronchiolitis obliterans (BO) is a particularly severe complication that occurs in 10% to 15 % of the patients with extensive graft-versus-host disease (GVHD) and is often refractory to treatment. We report herewith a child with chronic GVHD and BO after unrelated BMT. A 8 year-old girl with Ph ALL (Philadelphia-chromosome positive acute lymphoblastic leukemia) underwent an unrelated BMT in the first complete remission. Engraftment was uneventful. Acute GVHD of the skin developed, and was treated with methylprednisolone. No evidence of GVHD flare was documented, and immunosuppression was tapered off by 7 months posttransplant. On 9 months posttransplant, chronic GVHD involving skin, liver, mouth, eyes, gastrointestinal tract, and lungs developed. Despite conservative managements, the patient developed pneumomediastinum and subcutaneous emphysema secondary to severe BO. Her condition continued to deteriorate, and she died of respiratory failure 10 months after transplant. Further studies are required to identify risk factors and to develop newer methods of effective treatment for this rare complication.
Bone Marrow Transplantation ; Bronchiolitis Obliterans* ; Bronchiolitis* ; Child ; Female ; Gastrointestinal Tract ; Graft vs Host Disease ; Humans ; Hydrogen-Ion Concentration ; Immunosuppression ; Liver ; Lung ; Mediastinal Emphysema* ; Methylprednisolone ; Mouth ; Respiratory Insufficiency ; Risk Factors ; Skin ; Subcutaneous Emphysema*

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Dyskeratosis Congenita in a Girl.

Jun Sun YI ; Hoon KOOK ; Hee Jo PAIK ; So Youn KIM ; Ik Sun CHOI ; Suk Joo KIM ; Kyoung Ran SOHN ; Ho Song NAM ; Tai Ju HWANG

Korean Journal of Pediatric Hematology-Oncology.2003;10(2):293-298.

Dyskeratosis congenita (DC) is a rare genetic disorder encompassing abnormal skin pigmentation, dystrophic nails, leukoplakia of mucous membranes and others. Bone marrow failure is the cause of early mortality. Moreover, DC is known for its predisposition to malignancy. X-linked recessive, autosomal dominant and autosomal recessive forms of the disease are recognized. We describe here a rare case of DC in a 4-year-old girl showing dark skin, dystrophic toe nails, and mild bone marrow failure. Autosomal recessive disease was suggested as the patient is female, and tests for DKC1 and hTR mutations were negative. Intermittent treatment with oxymetholone and prednisolone for about 26 months resulted in stable hemoglobin and platelet response.
Blood Platelets ; Bone Marrow ; Child, Preschool ; Dyskeratosis Congenita* ; Female* ; Humans ; Leukoplakia ; Mortality ; Mucous Membrane ; Oxymetholone ; Prednisolone ; Skin ; Skin Pigmentation ; Toes

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Three Cases of Childhood Renal Cell Carcinoma Including 2 Cases with Systemic Immunotherapy.

Hye Rim CHUNG ; Eun Sil PARK ; Hyoung Soo CHOI ; Hee Young SHIN ; Hyo Seop AHN

Korean Journal of Pediatric Hematology-Oncology.2003;10(2):287-292.

Renal cell carcinoma (RCC) is a malignancy arising from the tubular epithelial cell. RCC is a rare disease in children with an incidence of 0.1~0.3% of all childhood cancer. It is known that RCC in children is different from its adult counterpart in some aspects. Although conventional treatment of RCC is radical nephrectomy with lymph node dissection, we tried systemic treatment for advanced disease. Because metastasis is common at diagnosis and RCC is usually resistant to radiotherapy or chemotherapy, immunotherapy such as interferon-alpha (IFN-alpha) or interleukin-2 (IL-2) is investigated and clinically used. We have experienced 3 children with RCC and two with stage IV disease were treated with systemic immunotherapy. One who had stage IV, recurred RCC is followed-up for 16 months without the evidence of disease progression with IFN-alpha and IL-2. We report these 3 cases with a brief review of literatures.
Adult ; Carcinoma, Renal Cell* ; Child ; Diagnosis ; Disease Progression ; Drug Therapy ; Epithelial Cells ; Humans ; Immunotherapy* ; Incidence ; Interferon-alpha ; Interleukin-2 ; Lymph Node Excision ; Neoplasm Metastasis ; Nephrectomy ; Radiotherapy ; Rare Diseases

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A Case of Allogeneic Stem Cell Transplantation for Chronic Granulomatous Disease.

Jae Hee LEE ; Mee Jung LEE ; Eun Seok CHOI ; Soo Jong HONG ; Chan Jeoung PARK ; Jong Jin SEO

Korean Journal of Pediatric Hematology-Oncology.2003;10(2):282-286.

Chronic granulomatous disease (CGD) is a rare primary immunodeficiency in children caused by an abnormal function of the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase in the phagocytic cells, which results in an increased susceptibility to severe bacterial and fungal infections. Prophylactic trimethoprim-sulfamethoxazole improves medium-term survival, but cannot prevent inflammatory sequelae. It still shows high morbidity and mortality. Bone marrow transplantation (BMT) is currently the only curative treatment for CGD. We report on a 29-month-old boy with CGD who was successfully treated with allogeneic BMT from an HLA-identical unrelated donors following a conditioning regimen consisting of busulfan and cyclophosphamide. One year after post-transplantation, the boy is in excellent clinical and hematological condition with complete chimerism and normal nitroblue tetrazolium (NBT) test.
Bone Marrow Transplantation ; Busulfan ; Child ; Child, Preschool ; Chimerism ; Cyclophosphamide ; Granulomatous Disease, Chronic* ; Humans ; Male ; Mortality ; NADP ; Nitroblue Tetrazolium ; Oxidoreductases ; Phagocytes ; Stem Cell Transplantation* ; Stem Cells* ; Trimethoprim, Sulfamethoxazole Drug Combination ; Unrelated Donors

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Evans Syndrome Following Unrelated Bone Marrow Transplantation.

Young Myoung KIM ; Bong Jin KIM ; Young Ho LEE

Korean Journal of Pediatric Hematology-Oncology.2003;10(2):277-281.

Thrombocytopenia and Coombs' positive hemolytic anemia (Evans syndrome) after hematopoietic stem cell transplantation (HSCT) rarely occurs, especially in childhood. We experienced a case of Evans syndrome following unmanipulated unrelated bone marrow transplantation, which promptly responded to steroid pulse therapy. Immune-mediated cytopenia should be considered for evaluation of post-engraftment cytopenia following HSCT.
Anemia, Hemolytic ; Bone Marrow Transplantation* ; Bone Marrow* ; Hematopoietic Stem Cell Transplantation ; Thrombocytopenia

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A Case of Idiopathic Hypereosinophilic Syndrome Treated Successfully with Hydroxyurea and Interferon alpha-2b.

Gi Hoon YANG ; Eun Young PARK ; Hye Lim JUNG ; Deok Soo KIM ; Jae Won SHIM ; Jung Yeon SHIM ; Moon Soo PARK

Korean Journal of Pediatric Hematology-Oncology.2003;10(2):269-276.

The idiopathic hypereosinophilic syndromes are rare hematologic disorders showing a wide spectrum of diseases with eosinophilia and associated organ damage. Three criteria for diagnosis include persistent eosinophilia of at least 1, 500 eosinophils/mm3 for longer than 6 months, or death before 6 months with signs and symptoms of hypereosinophilic disease; lack of evidence for parasitic, allergic or other known causes of eosinophilia; and signs and symptoms of organ involvement. We report a case of a 8 month-old male patient who had persistent marked eosinophilia reaching peak eosinophil count more than 100, 000/mm3, and organ dysfunction involving the bone marrow, heart, brain, lung and spleen. This patient, who did not respond to administration of prednisolone or hydroxyurea alone, has been treated successfully with hydroxyurea and interferon alpha-2b combination therapy.
Bone Marrow ; Brain ; Diagnosis ; Eosinophilia ; Eosinophils ; Heart ; Humans ; Hydroxyurea* ; Hypereosinophilic Syndrome* ; Infant ; Interferons* ; Lung ; Male ; Prednisolone ; Spleen

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The Effect of Vasoactive Intestinal Peptide on Cord Blood CD34 (+) Cells.

Mi Kyong YEO ; Hwa Young BAE ; Min Kyu HUR ; Jae Sun RA ; In Seob HAN ; Min Keong KIM ; Soon Ki KIM ; Sang Kyu PARK

Korean Journal of Pediatric Hematology-Oncology.2003;10(2):262-268.

PURPOSE: We investigated the expression of vasoactive intestinal peptide (VIP), VIP receptor 1 (VPAC1), VIP receptor 2 (VPAC2) genes in the human umbilical cord blood CD34 cells, and the ability of VIP to stimulate human primitive as well as monopotent hematopoietic progenitors. METHODS: We isolated RNA from umbilical cord blood CD34 cells, and then performed RT-PCR, and sequencing. The umbilical cord blood CD34 cells were cultured with the various concentrations of VIP for burst-forming unit of erythrocyte (BFU-E), colony-forming unit of granulocyte/monocyte (CFU-GM), colony-forming unit of graulocyte/erythrocyte/monocyte/megakaryocyte (CFU-GEMM), and colony-forming unit of megakaryocyte (CFU-Mk). RESULTS: The RNA coding for VPAC1 was detected in human umbilical cord blood CD34 cells. VIP significantly stimulated the growth of CFU-GEMM and CFU-Mk. CONCLUSION: The present results suggest that VIP is an important neuropeptide in the early proliferation of human primitive as well as megakaryocyte progenitors.
Clinical Coding ; Erythrocytes ; Fetal Blood* ; Humans ; Megakaryocyte Progenitor Cells ; Megakaryocytes ; Myeloid Progenitor Cells ; Neuropeptides ; Receptors, Vasoactive Intestinal Peptide ; RNA ; Stem Cells ; Vasoactive Intestinal Peptide*

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The Diagnostic Significance of Various Laboratory Findings in Hemophagocytic Lymphohistiocytosis: A Receiver Operating Characteristic Curve Analysis.

Jin Youl CHO ; Joo Hee HONG ; Joo Phil UM ; Byoung Ho CHA ; Hwang Min KIM ; Young UH

Korean Journal of Pediatric Hematology-Oncology.2003;10(2):255-261.

PURPOSE: The clinical findings of almost all of the hemophagocytic lymphohistiocytosis (HLH) patients are not significant. Early enforcement of bone marrow study for quicker detection of laboratory abnormalities in routine laboratory test, which is perfomed in febrile patients, is critical for early diagnosis of HLH. In this study, we tried to elucidate more significant laboratory test for the early diagnosis of HLH. METHODS: The study group and control group consisted of 13 HLH patients, and 46 febrile patients with causes like fever of unknown origin, kawasaki disease or acute lymphoblastic leukemia, respectively. Receiver operating characteristic (ROC) curve analysis of various laboratory findings of hyperpyrexic patients on the first day of admission were performed. RESULTS: The total WBC count, platelet count, serum triglyceride, serum transaminase, and serum ferritin revealed statistically significant differences between the study and the control group (P< 0.05). The ROC curve analysis revealed that serum triglyceride was the most efficient test for the differential diagnosis between HLH and febrile patients with other causes. And the serum transaminase, triglyceride and ferritin revealed a statistically significant difference between the study and the leukemia patients group (P< 0.05). The ROC curve analysis revealed that serum transaminase was the most efficient test for differential diagnosis between HLH and acute leukemia. CONCLUSION: In patients with persistent hyperpyrexia, the elevated serum triglyceride level is an important clue for the early diagnosis of HLH. In patient with persistent hyperpyrexia, and abnormal CBC findings, the elevated serum transaminase level is an important clue for the early diagnosis of HLH.
Bone Marrow ; Diagnosis, Differential ; Early Diagnosis ; Ferritins ; Fever of Unknown Origin ; Humans ; Leukemia ; Lymphohistiocytosis, Hemophagocytic* ; Mucocutaneous Lymph Node Syndrome ; Platelet Count ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; ROC Curve* ; Triglycerides

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The Clinical Manifestations of Hemophagocytic Lymphohistiocytosis.

Sun Young KIM ; Ju Yeon YOON ; Soon Ju LEE ; Nak Gyun CHUNG ; Pil Sang JANG ; Bin CHO ; Dae Chul JEONG ; Hack Ki KIM

Korean Journal of Pediatric Hematology-Oncology.2003;10(2):244-254.

PURPOSE: Hemophagocytic lymphohistiocytosis (HLH) is an important differential diagnosis in infants and children who present with prolonged fever, hepatosplenomegaly, marked hypertriglyceridemia and cytopenia. HLH is currently curable with immunomodulatory therapy, chemotherapy and stem cell transplantation. We evaluated the clinical characteristics and treatment outcomes in patients with HLH. METHODS: We reviewed retrospectively the medical records of 22 children with HLH from January 1996 to June 2003 at Catholic University St. Mary's Hospital. RESULTS: Among the 22 patients, 11 patients were male and the others were female. The median age of onset was 5.5 (0.6~14.4) years including 6 cases presenting before 2 years of age. Family history of suspicious HLH was observed in 3 patients and Epstein Barr virus (EBV) was demonstrated in 10 patients. The most frequent manifestation was fever and the median duration of fever at diagnosis was 18 (6~46) days. Common laboratory findings were neutropenia, anemia, thrombocytopenia, hypertriglyceridemia, hypofibrinogenemia and abnormal liver function tests. Bone marrow examinations showed significant hemophagocytic lymphohistiocytosis in all of these patients. No treatment was needed in 3 patients and 6 patients were treated with high dose immunoglobulin and antiviral agents, 9 cases with chemotherapy and 4 patients were transplanted with sibling bone marrow or unrelated cord blood. The causes of death were refusal to treatment in 1 patient, disease progression in 6 and pulmonary hemorrhage in 1. CONCLUSION: If the disease is familial or relapsing, progressive or persistent even without family history, hematopoietic stem cell transplantation from the best available donor is strongly recommended. In less severe secondary HLH cases, either no treatment or a short duration of therapy might suffice, but future studies are necessary to define these subsets, possibly with additional genetic markers.
Age of Onset ; Anemia ; Antiviral Agents ; Bone Marrow ; Bone Marrow Examination ; Cause of Death ; Child ; Diagnosis ; Diagnosis, Differential ; Disease Progression ; Drug Therapy ; Female ; Fetal Blood ; Fever ; Genetic Markers ; Hematopoietic Stem Cell Transplantation ; Hemorrhage ; Herpesvirus 4, Human ; Humans ; Hypertriglyceridemia ; Immunoglobulins ; Immunomodulation ; Infant ; Liver Function Tests ; Lymphohistiocytosis, Hemophagocytic* ; Male ; Medical Records ; Neutropenia ; Refusal to Treat ; Retrospective Studies ; Siblings ; Stem Cell Transplantation ; Thrombocytopenia ; Tissue Donors

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A Case of Malignant Melanoma in Children.

Joong Hyun BIN ; Pil Sang JANG ; Soh Yeon KIM ; Nak Gyun CHUNG ; Bin CHO ; Soon Ju LEE ; In Goo LEE ; Hack Ki KIM

Korean Journal of Pediatric Hematology-Oncology.2001;8(2):378-381.

Malignant melanoma is a very rare disease in children. There is an increased risk for the development of malignant melanoma in patient with giant congenital melanocytic nevus. The manifestations of distant metastases in malignant melanoma commonly indicate a poor prognosis. First line treatment of malignant melanoma is excision, but when associated with giant congenital melanocytic nevus, excision is very difficult due to the site and the size of the lesions. However, malignant melanoma is not sensitive to chemotherapy, but a child is more sensitive than an adult. We report a case of unresectable childhood malignant melanoma associated with a giant congenital melanocytic nevus in a 3 year-old female treated with chemotherapy.
Adult ; Child* ; Child, Preschool ; Drug Therapy ; Female ; Humans ; Melanoma* ; Neoplasm Metastasis ; Nevus, Pigmented ; Prognosis ; Rare Diseases

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