[Intravascular lymphomatosis presenting in the lung].
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.
Feng
Rui-E
RE
Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing 100730, China. feng.ruie@yahoo.com.cn
Liu
Hong-Rui
HR
Zhou
Wei-Xun
WX
Shi
Ju-Hong
JH
Tian
Xin-Lun
XL
Wang
Huan-Ling
HL
Ren
Xin-Yu
XY
Ling
Qing
Q
Cui
Quan-Cai
QC
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*