Diffuse thoracic lymphangiomatosis with disseminated intravascular coagulation in a child.
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.
Tamay
Zeynep
Z
Division of Pediatric Allergy and Chest Diseases, Department of Pediatrics, Istanbul University, Istanbul Medical School, Turkey. eztamay@yahoo.com
Saribeyoglu
Ebru
E
Ones
Ulker
U
Anak
Sema
S
Güler
Nermin
N
Bilgic
Bilge
B
Yilmazbayhan
Dilek
D
Gun
Feryal
F
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
D000305:Adrenal Cortex Hormones; D013629:Tamoxifen; D003609:Dactinomycin; D014750:Vincristine; D003520:Cyclophosphamide