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 1 st transplant (BMT) at 2 y/o–HLA-matched sibling donor Recurrent cutaneous disease at 3 y/o 2 nd transplant peripheral stem cell at 3 y/o -same sibling donor

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

4. Background 2 nd 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

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

6. 2-4 years after 2 nd 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

8. What do you see?

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

10. % predicted

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

13. What do you think of the CT?

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

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

20. What is your next step?

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

22. Patient came back…

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)

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. * Started on cyclosporine and prednisone per Heme- Oncology

25. 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). Histopathological Report

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

27. Any other thoughts?

28. Thank you very much!!!