Table 1– Patient characteristics, history of cancer and history of antineoplastic treatments
Patient number
Age at diagnosis of malignancy years1443424205
Neoplastic conditionAcute T-lymphoblastic leukaemiaAcute lymphoblastic leukaemiaHodgkin’s disease (stage IIB)Oligodendroglioma (grade III)Acute myelogenous leukaemiaPylocytic astrocytoma
Chemotherapy regimen#14 years of age: cyclophosphamide, vincristine, prednisone, daunorubicin, l-asparaginase, intrathecal methotrexate, aracytine; consolidation: cyclophosphamide, vincristine, methotrexate. 17 years of age: maintenance: cyclophosphamide, vincristine, aracytine, l-asparaginase, prednisone, methotrexate, 6-mercaptopurine.4 years of age: vincristine, rubidomycin, prednisone, intrathecal methotrexate, cytosine-thioguanine. 7 years of age: cyclophosphamide, thioguanine, rubidomycin, hydroxyurea, methotrexate, BCNU, acracytine, vincristine. 10 years of age: vincristine, rubidomycin, l-asparaginase, prednisone, cyclophosphamide, vincristine, adriamycin, aracytine.34 years of age: doxorubicin, ablastin, bleomycin, dacarbazine, etoposide, adriblastine, ifosfamide; followed by: doxorubicin, cyclophosphamide, vindesine, bleomycin, prednisone, dexamethasone-aracytine, cisplatin. 35 years of age: busulfan, aracytine, melphalan, autologous stem cell Tx.24 years of age: ACNU, BCNU; second line: carboplatin, etoposide; third line: oral procarbazine.20 years of age: aracytin, idarubicin, familial donor stem cell Tx, cyclophosphamide, etoposide. 23 years of age: intrathecal depomedrol, aracytine, methotrexate, second stem cell Tx, cyclophosphamide, busulfan, aracytine.5 years of age: carboplatin, etoposide, cyclophosphamide, procarbazine, cisplatin, vincristine. 11 years of age: vinblastine. 12 years of age: thioguanine, procarbazine, CCNU, vincristine.
Irradiation type and doseNoneCraniospinal irradiation 18 Gy (10 years of age) Testicular 18 Gy (10 years of age)NoneBrainstem 55 Gy (31 years of age)TBI (20 years of age) Craniospinal (23 years of age)None
Cycophosphamide/BCNU total dose>3.6 gUnknown3.4 g1090 g·m-2UnknownUnknown
Age at treatment termination years171035252312
Age at respiratory symptom onset years222637263013
Age at PPFE diagnosis years322838273015
Time from chemotherapy end to chest symptom onset5 years16 years2 years1 year7 years6 months
Pleuritic chest painYesYesYesNoYesNo
Chest deformity/platythorax+/-+++++-
Spontaneous pneumothoraxBilateralYesBilateralBilateralBilateralNo
Smoking statusCurrent smoker (20 pack-years)Never-smokerNever-smokerNever-smokerNever-smokerNever-smoker
TLC % pred455949545430
FEV1/FVC %909798709168
KCO mL·min−1·mmHg−1·L−1 (% pred)4.63 (94)5.71 (109)3.94 (73)NDNDDLCO 44% pred
Clinical course(year after PPFE diagnosis)Deterioration (9)Death (2)Death (3)Death (15)Death (7)Deterioration (2)
  • BCNU: 1,3-bis-(2-chloroethyl)-1-nitrosourea; Tx: transplantation; ACNU: N'-[(4-amino-2-methylpyrimidin-5-yl)methyl]-N-(2-chloroethyl)-N-nitrosourea; CCNU: N-(2-chloroethyl)-N'-cyclohexyl-N-nitrosourea; TBI: total body irradiation; PPFE: pleuroparenchymal fibroelastosis; NYHA: New York Heart Association; BMI: body mass index; TLC: total lung capacity; FEV1: forced expiratory volume in 1 s; FVC: forced vital capacity; KCO: transfer coefficient of the lung for carbon monoxide; ND: no data; DLCO: diffusing capacity of the lung for carbon monoxide. #: age at beginning of treatment. -: absent; +: present; ++: marked.