Table 1 Patient characteristics

From: Pleuroparenchymal fibroelastosis in patients with pulmonary disease secondary to bone marrow transplantation

  

Case 1

Case 2

Case 3

Case 4

 

Sex

Female

Male

Male

Female

Clinical presentation

Age (years)

13

23

32

55

 

Underlying disease

Aplastic anaemia

Acute myeloid leukaemia

Acute lymphoblastic leukaemia

Acute myeloid leukaemia

 

Bone marrow transplantation conditioning procedure

Cyclophosphamide

Total body irradiation

Total body irradiation

Unknown

 

Transplant type

Allogeneic, matched sibling

Allogeneic, sibling

Allogeneic, sibling

Autologous

 

Time from bone marrow transplantation to presentation (in years)

2 years 9 months

12

16

2

 

Symptoms

Dyspnoea, chest pain

Dyspnoea, phlegm, weight loss

Dyspnoea, bronchitis

Dyspnoea, pyrexia

 

Pneumothorax

Bilateral

Right

Bilateral

Left

 

Extra-pulmonary graft-vs host disease

No

Yes

No

No

CT

 

Patchy visceral pleural thickening

Visceral pleural thickening

Non-uniform visceral pleural thickening

Pleuroparenchymal irregularity consistent with fibrosis

  

Subpleural blebs

Pleuroparenchymal tags

Subpleural blebs

Fine interstitial fibrosis

  

Mosaicism

Mosaicism

Mosaicism

Ground-glass opacification

Pathology

Method of sampling

Video-assisted thoracoscopic biopsy

Postmortem

Video-assisted thoracoscopic biopsy

Postmortem

 

Pleuroparen chymal fibroelastosis

Yes

Yes

Yes

Yes

 

Obliterative bronchiolitis

Yes

Yes

Yes

Yes

Follow-up

 

Progressive disease

Died 12 years after bone marrow transplantation

Stable disease

Died 5 years after bone marrow transplantation