No | Sex | Start pulm. dis. (y) | Course pulm. dis. | Chest CT scan | Treatments | Age at last follow up (y) | Out-come | Final diagnosis and likely cause of PAP |
---|---|---|---|---|---|---|---|---|
1 | F | 64 | Chronic respiratory insufficiency, PAP, inactive TBC, ARDS | Diffuse ground glass with clear interstitial septal thickening (crazy paving pattern), bilateral alveolar consolidations | Oxygen, WLL (2) | 66 | dead | CML, karyotype 46XX t(9,22) (q34,q11) 25, CD 2+, renal failure |
2 | M | 34 | Respiratory insufficiency, recurrent interstitial pneumonia (HSV); bron-chiolitis obliterans; PAP | Interstitial thickening and destructions in both lungs, crazy-paving pattern | Prednisolone, azathioprine, oxygen, WLL (3) | 37 | dead | Monocyte defect, M. avium intracellulare infection, GATA2 mutation (p.Y377D) |
3 | M | 0.6 | Infection, tachydyspnea (7 months old), hypoxia, PAP (1.7 y) | Diffuse ground glass, interstitial markings, emphysema | Oxygen, immune-suppressive drugs, methylprednisolone, WLL (3) | 3, lost on follow up | sick-same | Juvenile myelo-monocytic leukemia, Monosomy 7, 2x SCT, intestinal, hepatic, cutaneous GVHD |
4 | F | 38 | Dyspnea, clubbing, respiratory insufficiency, cholesterol pneumonia, PAP | Diffuse ground glass, interstitial markings, scattered alveolar opacities | Oxygen, no WLL | 43, lost on follow up | sick-same | Monocyte defect, cholesterol pneumonia, GATA2 mutation (p.R398W) |
5 | F | 6 | PAP | Ground glass, interstitial pneumonia | Oxygen, no WLL | 7 | sick-better | C-acute lymphoblastic leukemia |
6 | F | 59 | Dyspnea, respiratory insufficiency, PAP | Diffuse ground glass, interstitial septal thickening (crazy paving), markedly basal, with traction bronchiectasis | Oxygen, WLL (1) | 59 | dead | MDS |
7 | F | 4 | Recurrent airway infections; chronic hypoxic failure, PAP | Alveolar opacities in almost all lung areas, see Fig. 2 | Oxygen, WLL (14) | 7.3 | healthy | MDS, DiGeorge Syndrome Type 2, Monosomy 7, Trisomy 8 (SCT) |
8 | M | 0.33 | CMV infection, respiratory failure, PAP | Ground glass opacities with interstitial septal thickening (crazy paving pattern), alveolar consolidations bilaterally in the lower parts | Oxygen, WLL (11) | 2 | sick-better | Sideroblastic anemia |