Vascular leak syndrome(VLS) is characterized by endothelial damage, which causes extravasation of plasma proteins and fluid from capillaries into the extravascular space. It has been suggested that the increased vascular permeability is the result of an IL-2-induced suppression of endothelin-1 secretion by endothelial cells, an IL-2-induced activation of the complement cascade or TNF-alpha release from IL-2-activated T-cells. A 13-year-old male patient visited our hospital with abdominal pain. He was diagnosed with acute lymphoblastic leukemia(T cell type) by bone marrow study on his 2 nd day in hospital. On the 3 rd day of induction chemotherapy(prednisone, L-asparaginase, vincristine, intrathecal methotrexate), pulmonary edema and pleural effusion, ascites and generalized edema developed and lasted for 53 days without responding to supportive care. The laboratoy finding was that TNF-alpha was increased without evidence of infection and hypoalbuminemia was noted. It was suggested that the patient's clinical feature was induced by VLS.
Abdominal Pain ; Adolescent ; Ascites ; Blood Proteins ; Bone Marrow ; Capillaries ; Capillary Permeability ; Complement System Proteins ; Edema ; Endothelial Cells ; Endothelin-1 ; Humans ; Hypoalbuminemia ; Induction Chemotherapy* ; Male ; Pleural Effusion ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Pulmonary Edema ; T-Lymphocytes ; Tumor Necrosis Factor-alpha ; Vincristine

Common variable immunodeficiency (CVID) is a heterogenous syndrome characterized by hypogammaglobulinemia, various immunologic abnormalities and recurrent bacterial infections. Associated immunologic abnormalities consists of various kinds of autoimmune diseases and lymphoproliferative disorders. The lymphoproliferative disorder take several forms, such as malignant lymphoma, atypical lymphoid hyperplasia, and beniegn lymphoid hyperplasia. Lymphocytic interstitial pneumonia (LIP), which is a kind of atypical lymphoid hyperplasia, develop in young age groups and has controversy on its clonality. We experienced a 14-year-old female patient with LIP and CVID. We analysed the third complementarity-determining region (CDR3) of the immunoglobulin heavy chain gene for clonality analysis. Clonality analysis of lung biopsy specimen revealed that 6 of 13 colony and 4 of 13 colony have identical sequences respectively. We speculate that one of these 2 lymphoid cell clone may develop into malignant lymphoma in the future.
Adolescent ; Agammaglobulinemia ; Autoimmune Diseases ; Bacterial Infections ; Biopsy ; Clone Cells ; Common Variable Immunodeficiency* ; Complementarity Determining Regions ; Female ; Humans ; Hyperplasia ; Immunoglobulin Heavy Chains ; Lip ; Lung ; Lung Diseases, Interstitial* ; Lymphocytes ; Lymphoma ; Lymphoproliferative Disorders