1. Pulmonary complications in a child with AML
CHILDREN’S HOSPITAL & RESEARCH CENTER OAKLAND
Hazel Villa, MD

2. Background LC,11 y/o girl AMLM1 at 20 months old
1st transplant (BMT) at 2 y/o–HLA-matched
sibling donor
Recurrent cutaneous disease at 3 y/o
2nd transplant peripheral stem cell at 3 y/o
-same sibling donor 2

3. Background First transplant: BMT
1.Induction chemotherapy: Idarubicin, Ara-C, Etoposide, 6-thioguanine, dexamethasone
2. Preparation for transplant: myeloablation with :
Busulfan, CyclophosphamideCytoxan
3. Prophylaxis for GVHD: Methotrexate 3

4. Background 2nd transplant : peripheral stem cell transplant
( She had cutaneous relapse)
1.Preparation for SCT: total body irradiation
chemotherapy with: Etoposide, cyclophosphamide
2.GVHD prevention with Methotrexate 4

5. Background 10/2003-1/2004 (5 months post SCT) Chronic GVHD!!!
Oral lesionsbudesonide topical
Crackles- chest CT: mosaic perfusion
Flovent 44 2 puffs BID
Cyclosporine 5

6. 2-4 years after 2nd transplant ( Patient is 4-6 years of age)
Asymptomatic
PFT
FVC 94
pre FEV1 68
post FEV1 74
FEV1/FVC 62
TLC 142
RV 259
DLCO- normal
Flovent BID /Albuterol MDI prn 6

7. 7

8. What do you see? 8

9. Disease Progression 7 years post 2nd transplant ( patient was 10 y/o)
Admitted from the ED for respiratory distress
Treated for community acquired pneumonia 9

10. % predicted 10

11. 11

12. Patient was re-admitted * CXR –increased infiltrates on the right
* Chest CT : 12

13. What do you think of the CT?13

14. * Flexible bronchoscopy: normal anatomy
* BAL: AFB result was pending, NURF
Treatment intensified
* Plan to start azithromycin for BO, if TB negative

15. 15

16. BAL : Mycobacterium kansasii
Quantiferon Gold –negative
INH, RIF, EMB
* Airway clearance therapy was continued 16

17. 17

18. 18

19. 19

20. What is your next step?

21. ? BOS or BOOP/COP INFECTION BOS/BOOP PROGRESSION
REMOVE THE CYST OR NOT ? 21

22. Patient came back… 22

23. Pulmonary Plan: * Agree with immunosuppression if (-) pneumothorax,
(-) chest tube
* Resection of the enlarging cyst. (Blebectomy preferred,
pt has low lung reserve)
* NO pleurodesis for recurrent pneumothorax,
if lung transplant is an option
* Favor Azithromycin (BOS/ NTB)
Prednisone (BOS/Immunosuppresion) 23

24. Course: * Underwent blebectomy- lung tissue sent for histopathology
* No recurrence of pneumothorax post-blebectomy
* Started on cyclosporine and prednisone
* Now 4-drug treatment for M. kansasii (+ Azithromycin)
Outpatient follow up: 10/4/10
* Pt doing well.
per Heme- Oncology

25. Histopathological Report
No evidence of recurrent AML
Areas of obliterated bronchioles show mature collagenous fibrosis
No interstitial scarring in most of the damaged airways.
No features of cryptogenic organizing pneumonia (COP). 25

26. ORGANIZING FIBRINOUS PLEURITIS CONSISTENT WITH PNEUMOTHORAX
OBLITERATIVE BRONCHIOLITIS CONSISTENT WITH PULMONARY GRAFT VERSUS HOST DISEASE 26

27. Any other thoughts?

28. Thank you
very much!!!