title: Diffuse thoracic lymphangiomatosis with disseminated intravascular coagulation in a child.
abstract: An 8.5-year-old girl who presented with chronic cough and hemoptysis underwent a CT scan of the chest showing diffuse mediastinal and parenchymal infiltration and pleural effusion, and laboratory findings showed disseminated intravascular coagulation. Disseminated lymphangiomatosis was diagnosed after an open-lung biopsy. She was treated by systemic steroids, interferon, tamoxifen, chemotherapy, and radiation but died of respiratory failure and disseminated intravascular coagulation 2 years after the diagnosis. This patient represents a rare presentation of diffuse pulmonary lymphangiomatosis together with disseminated intravascular coagulation, involving both the mediastinum and pulmonary parenchyma, in a child.
authors.affiliation: Division of Pediatric Allergy and Chest Diseases, Department of Pediatrics, Istanbul University, Istanbul Medical School, Turkey. eztamay@yahoo.com
authors.forename: Zeynep
authors.initials: Z
authors.lastname: Tamay
authors.forename: Ebru
authors.initials: E
authors.lastname: Saribeyoglu
authors.forename: Ulker
authors.initials: U
authors.lastname: Ones
authors.forename: Sema
authors.initials: S
authors.lastname: Anak
authors.forename: Nermin
authors.initials: N
authors.lastname: Güler
authors.forename: Bilge
authors.initials: B
authors.lastname: Bilgic
authors.forename: Dilek
authors.initials: D
authors.lastname: Yilmazbayhan
authors.forename: Feryal
authors.initials: F
authors.lastname: Gun
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000627:therapeutic use; D002648:Child; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000008:administration & dosage; D003609:Dactinomycin / Q000008:administration & dosage; D004211:Disseminated Intravascular Coagulation / Q000209:etiology*; D004417:Dyspnea / Q000188:drug therapy / Q000209:etiology; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008202:Lymphangioma / Q000097:blood / Q000150:complications* / Q000188:drug therapy / Q000532:radiotherapy; D008482:Mediastinum / Q000473:pathology; D010166:Palliative Care; D018714:Radiotherapy, Adjuvant; D013629:Tamoxifen / Q000008:administration & dosage; D013899:Thoracic Neoplasms / Q000097:blood / Q000150:complications* / Q000188:drug therapy / Q000532:radiotherapy; D013921:Thrombocytopenia / Q000209:etiology; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D000305:Adrenal Cortex Hormones; D013629:Tamoxifen; D003609:Dactinomycin; D014750:Vincristine; D003520:Cyclophosphamide