2. Primary Pulmonary Lymphoma

3. Case I  73 y male Xsmoker 20 y with 30p  Transferred from Dryden to Int.Medicine  Was supposed to see thoracic Sx OPD  Unwell last 6 months

4. History  Dry cough & SOB II – III  Fever, night sweating  Wt loss 3o Ibs  Abd. Pain, No GI bleeding  No urinary symptoms

5. History  Received 2 courses of Abx  no response  GI scope  ulceration ? Celiac disease  PMH : DM, HTN, CVA complete recovery 2000  Rx : ASA, Metoprolol, Atrovastatin, Glyburide Cefuroxime,Azithromycine Cefuroxime,Azithromycine  High school teacher, No travel, contact, pets

6. Examinations  Unwell mild respiratory distress  Temp 38.5 RR 18 Sat 88% RA 96 5l O2 BP 120/70 HR 70 JVP 5 ASA BP 120/70 HR 70 JVP 5 ASA  Chest : decreased BS Bronchial Lt Bronchial Lt  CVS : S1+S2+S3 ESM II/VI LSB ESM II/VI LSB  LL edema

7. Investigation  WBC 16 Poly 13 Hb 120 MCV N Hb 120 MCV N Plt 400 INR 10 PTT 55 Plt 400 INR 10 PTT 55  Creat 120 BUN 13  ABG PH 7.40 PCO 35 PO2 150 on 6l O2  LFT Mild cholestasis  CXR & CT Chest

8. Investigation  Sputum GS, AFB, & Fungal  -ve  ANA, ANCA, RF –ve Complement N  UA & microscopy N  Bronch  the most difficult i have seen Conscious sedation & 1 hour of topical Rx Conscious sedation & 1 hour of topical Rx No Bx No Bx BAL –ve micro & cytology BAL –ve micro & cytology

9. Investigation  CT guided lung Bx  Lymphoma  Lymphoma Primary Pulmonary Lymphoma Primary Pulmonary Lymphoma

10. Primary Pulmonary Lymphoma  Lymphomatous proliferation can involve the lung in three ways: Hematogenous spread of HD or NHL Hematogenous spread of HD or NHL Contiguous invasion from LN Contiguous invasion from LN Primary pulmonary involvement PPL Primary pulmonary involvement PPL Eur Resp Jr 2002 Eur Resp Jr 2002

11. Primary Pulmonary Lymphoma  Definition: Clonal lymphoid proliferation Clonal lymphoid proliferation affecting lung (parenchyma or airway) affecting lung (parenchyma or airway) without extrapulmonary involvement without extrapulmonary involvement at Dx or during subsequent 3 months at Dx or during subsequent 3 months Eur Resp Jr 2002 Eur Resp Jr 2002

12. Primary Pulmonary Lymphoma  PPL includes: MALT NHL  Multifocal mucosa associated MALT NHL  Multifocal mucosa associated lymphoid tissue lymphoid tissue Satellite nodes Satellite nodes Lymphamatoid granulomatosis Lymphamatoid granulomatosis  Very rare 0.5-1% of 1ry pulmonary malignancy 0.5-1% of 1ry pulmonary malignancy 3-4 % of extranodal NHL (25-50% of NHL) 3-4 % of extranodal NHL (25-50% of NHL) Eur Resp Jr 2002 Eur Resp Jr 2002

13. Primary Pulmonary Lymphoma  Classification: Bronchial MALT most frequent  Pesudolymphoma Slowly progressive & relative benign histology 90% correspond to MALT NHL No documented triggering antigen for bronchial MALT ( H.pylori equivalent )  ?Autoimmune diseases  ?Autoimmune diseases Eur Resp Jr 2002 Eur Resp Jr 2002

14. Primary Pulmonary Lymphoma Bronchial MALT 58-87 % of PPL Age of onset 50-60 y Age of onset 50-60 y 50 % of pts are asymptomatic  radiological B symptoms in 25% Alveolar opacity with air bronchogram 50% Bilateral 60-70% BAL  B lymphocytes > 10% Eur Resp Jr 2002 Eur Resp Jr 2002

15. Primary Pulmonary Lymphoma Bronchial MALT Bronchoscopy  N, mucosal inflammation or stenosis or stenosis Bx shows 1) lymphoid proliferation 2) lymphoepithelial lesion 2) lymphoepithelial lesion 3) Blastic changes 3) Blastic changes Peribronchovascular distribution Concomitant other mucosal MALT 25-35% BM involvement < 20% Eur Resp Jr 2002 Eur Resp Jr 2002

16. Primary Pulmonary Lymphoma Bronchial MALT Other work up  ENT, GI scopes SPE {High B2 microglobulin poor prognosis} SPE {High B2 microglobulin poor prognosis} 5y survival > 80% Progression faster than GI MALT No difference in prognosis with TNM,histology or bilateral disease Ann Thoracic Sx 2000 Ann Thoracic Sx 2000

17. Primary Pulmonary Lymphoma Bronchial MALT No consensus on Rx Surgery for localized disease Chemo for bilateral, extrapulmonary disease Combination Vs single agent  no difference Radiotherapy is not used Heme Onc 1983 Cancer 1985 Heme Onc 1983 Cancer 1985

18. Primary Pulmonary Lymphoma  High Grade PPL 11-19% of PPL Age 60y Solid organ Tx, HIV, Sjogren Syndrome Pts symptomatic  fever,wt loss Single pulmonary mass Multiple cavitatory lesions in HIV Eur Resp Jr 2002 Eur Resp Jr 2002

19. Primary Pulmonary Lymphoma  High Grade PPL Higher yield from transbronchial Bx  higher mitotic activity & endoluminal invasion Prognosis poor Rx combined surgical & chemo Eur Resp Jr 2002 Eur Resp Jr 2002

20. Primary Pulmonary Lymphoma  Lymphomatoid Granulomatosis 500 – 600 reported cases Age 30-50 y males > females Most Pts symptomatic Bilateral lower lobe predominance Peribronchovascular tend to cavitate Eur Resp Jr 2002 Eur Resp Jr 2002

21. Primary Pulmonary Lymphoma  Lymphomatoid Granulomatosis Extrapulmonary involvement  skin, renal, joints, peripheral & central nervous system Bx  nodular, polymorphous no granuloma formation no granuloma formation vascular tropism vascular tropism 3-4 y survival Rx Cyclophosphamide + predinsone Eur Resp Jr 2002 Eur Resp Jr 2002