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Primary Pulmonary Lymphoma

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

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Primary Pulmonary Lymphoma

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  1. Primary Pulmonary Lymphoma

  2. 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

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

  4. 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 • High school teacher , No travel , contact , pets

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

  6. Investigation • WBC 16 Poly 13 Hb 120 MCV N 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

  7. 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 No Bx BAL –ve micro & cytology

  8. Investigation • CT guided lung Bx  Lymphoma Primary Pulmonary Lymphoma

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

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

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

  12. 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 Eur Resp Jr 2002

  13. Primary Pulmonary Lymphoma Bronchial MALT 58-87 % of PPL 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

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

  15. Primary Pulmonary Lymphoma Bronchial MALT Other work up  ENT , GI scopes 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

  16. 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

  17. 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

  18. 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

  19. 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

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

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