Table 1.

Patients and BLPD characteristics

Patient/sexAge (y)DiseaseTransplantAffected sitesGeneral manifestationsPathologic morphology of BLPD lesionsPhenotype of infiltrating cells in BLPD lesionsClonality of BLPD lesionsEBV in BLPD lesionsDetection of EBV genome by PCR out of tumor siteCirculating B cells (CD19 or CD20/μl)
1/F 41 Lymphoangiomatosis Unipulmonary Endobronchial None Polymorphic necrosis CD20+ Polyclonal LMP1+
EBER+ 
ND 80 
2/M 61 OLD Bipulmonary Bone, bone marrow, pleura, mediastinal nodes Fever,
high CRP level 
Monomorphic, plasmacytoid IgAκ+ Polyclonal EBER+ ND ND* 
3/M 33 Renal artery trauma Kidney Lymph nodes, liver Fever,
high CRP level 
Polymorphic CD20+
CD79+
CD30+ 
ND LMP1+ Blood 
4/F 27 Pulmonary hypertension Bipulmonary Oral None Monomorphic necrosis CD30+ Monoclonal LMP1+
EBER+ 
ND 20 
5/M 38 Cystic fibrosis Bipulmonary Lungs None Polymorphic necrosis CD20+
CD79+ 
ND LMP1+
EBER+ 
Blood bone marrow CSF 92  
6/M 62 Liver cirrhosis Liver Liver hilum High CRP level Polymorphic necrosis CD20+
CD79+ 
Polyclonal LMP1+ Blood 14 
7/M 49 NOCM Heart Perinephritis, cutaneous High CRP level Monomorphic plasmacytoid κ chain+ Monoclonal Southern+
PCR+ 
ND ND* 
8/M Biliary atresia Liver Liver, spleen, lymph nodes pleura Fever,
high CRP level 
Polymorphic CD20+ Monoclonal PCR+ Blood, CSF, 40  
9/M 12 α1Anti-trypsine deficiency Liver Liver hilum, graft (liver), bone marrow Fever,
high CRP level 
Polymorphic necrosis CD19+
CD20+
CD22+ 
Monoclonal EBER+
PCR+ 
Pleursy blood, bone marrow 45  
10/M 31 Interstitial nephritis Kidney Liver, abdomen, lymph nodes, subcutaneous High CRP level Polymorphic CD20+
CD79+ 
ND LMP1+ Blood ND 
11/M 32 Kidney hypoplasia Kidney Graft (kidney), kidney hilum, lymph nodes Fever,
high CRP level 
Polymorphic CD20+ ND LMP1+
EBER+ 
ND 
12/M 20 Cystic fibrosis Bipulmonary Lungs, mediastinal nodes High CRP level Polymorphic necrosis CD19+
CD20+
CD79+ 
Monoclonal LMP1+
EBER+ 
Blood None 
Patient/sexAge (y)DiseaseTransplantAffected sitesGeneral manifestationsPathologic morphology of BLPD lesionsPhenotype of infiltrating cells in BLPD lesionsClonality of BLPD lesionsEBV in BLPD lesionsDetection of EBV genome by PCR out of tumor siteCirculating B cells (CD19 or CD20/μl)
1/F 41 Lymphoangiomatosis Unipulmonary Endobronchial None Polymorphic necrosis CD20+ Polyclonal LMP1+
EBER+ 
ND 80 
2/M 61 OLD Bipulmonary Bone, bone marrow, pleura, mediastinal nodes Fever,
high CRP level 
Monomorphic, plasmacytoid IgAκ+ Polyclonal EBER+ ND ND* 
3/M 33 Renal artery trauma Kidney Lymph nodes, liver Fever,
high CRP level 
Polymorphic CD20+
CD79+
CD30+ 
ND LMP1+ Blood 
4/F 27 Pulmonary hypertension Bipulmonary Oral None Monomorphic necrosis CD30+ Monoclonal LMP1+
EBER+ 
ND 20 
5/M 38 Cystic fibrosis Bipulmonary Lungs None Polymorphic necrosis CD20+
CD79+ 
ND LMP1+
EBER+ 
Blood bone marrow CSF 92  
6/M 62 Liver cirrhosis Liver Liver hilum High CRP level Polymorphic necrosis CD20+
CD79+ 
Polyclonal LMP1+ Blood 14 
7/M 49 NOCM Heart Perinephritis, cutaneous High CRP level Monomorphic plasmacytoid κ chain+ Monoclonal Southern+
PCR+ 
ND ND* 
8/M Biliary atresia Liver Liver, spleen, lymph nodes pleura Fever,
high CRP level 
Polymorphic CD20+ Monoclonal PCR+ Blood, CSF, 40  
9/M 12 α1Anti-trypsine deficiency Liver Liver hilum, graft (liver), bone marrow Fever,
high CRP level 
Polymorphic necrosis CD19+
CD20+
CD22+ 
Monoclonal EBER+
PCR+ 
Pleursy blood, bone marrow 45  
10/M 31 Interstitial nephritis Kidney Liver, abdomen, lymph nodes, subcutaneous High CRP level Polymorphic CD20+
CD79+ 
ND LMP1+ Blood ND 
11/M 32 Kidney hypoplasia Kidney Graft (kidney), kidney hilum, lymph nodes Fever,
high CRP level 
Polymorphic CD20+ ND LMP1+
EBER+ 
ND 
12/M 20 Cystic fibrosis Bipulmonary Lungs, mediastinal nodes High CRP level Polymorphic necrosis CD19+
CD20+
CD79+ 
Monoclonal LMP1+
EBER+ 
Blood None 

OLD, obstructive lung disease; NOCM, nonobstructive cardiomyopathy; CSF, cerebrospinal fluid.

*

In 2 patients, hyperbasophilic cells could be detected in blood.

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