title: [Intravascular lymphomatosis presenting in the lung].
abstract: OBJECTIVE
  To investigate the clinical, radiographic and pathological characteristics of intravascular lymphomatosis primarily manifested in the lung, without skin and central nervous system involvements.
  
  
  METHODS
  A case of T cell intravascular lymphomatosis presenting with fever and multiple pulmonary shadows on chest radiograph was described and 14 similar cases reported in the English literature were reviewed.
  
  
  RESULTS
  We described a case of T cell intravascular lymphomatosis, who was a 36 year old man, complained of fever and multiple pulmonary shadows on chest radiograph and admitted to Peking Union Medical College Hospital in march, 2008. Open lung biopsy showed features characteristic of intravascular lymphomatosis. He received CHOP chemotherapy, but died 20 days after diagnosis. Most cases of intravascular lymphomatosis primarily manifested in the lung occurred in older patients, ranging from 36 to 79 years of age (mean 59 years), with a male predominance (M : F = 11 : 4). The chief complaints were dyspnoea (10/15), cough (9/15), fever (5/15) and weight loss (5/15). Pulmonary function tests usually revealed some degree of decreased diffusing capacity. Eight cases had high serum lactate dehydrogenase levels. Chest CT scan showed multiple reticular or/and nodular density. Immunophenotypically, 10 cases were B cell lineage, 3 cases were T cell lineage. Six cases of B cell intravascular lymphomatosis were followed, of whom 4 and were alive, and 2 died of respiratory failure. Three cases of T cell intravascular lymphomatosis showed poor prognosis, both of whom died of respiratory failure.
  
  
  CONCLUSIONS
  Intravascular lymphomatosis primarily manifested in the lung is a rare malignant disease. Its pulmonary symptoms were nonspecific, and pulmonary function tests and chest CT scan manifested as those of interstitial pneumonia or pulmonary infection. It can be pathologically diagnosed through transbronchial lung biopsy or surgical lung biopsy.
authors.affiliation: Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing 100730, China. feng.ruie@yahoo.com.cn
authors.forename: Rui-E
authors.initials: RE
authors.lastname: Feng
authors.forename: Hong-Rui
authors.initials: HR
authors.lastname: Liu
authors.forename: Wei-Xun
authors.initials: WX
authors.lastname: Zhou
authors.forename: Ju-Hong
authors.initials: JH
authors.lastname: Shi
authors.forename: Xin-Lun
authors.initials: XL
authors.lastname: Tian
authors.forename: Huan-Ling
authors.initials: HL
authors.lastname: Wang
authors.forename: Xin-Yu
authors.initials: XY
authors.lastname: Ren
authors.forename: Qing
authors.initials: Q
authors.lastname: Ling
authors.forename: Quan-Cai
authors.initials: QC
authors.lastname: Cui
mesh_terms: D000328:Adult; D006801:Humans; D008168:Lung / Q000473:pathology*; D008175:Lung Neoplasms / Q000378:metabolism* / Q000473:pathology; D008228:Lymphoma, Non-Hodgkin / Q000473:pathology*; D008297:Male; D019043:Vascular Neoplasms / Q000473:pathology*