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Case Presentation. Weijun Cao Department of Respiratory Medicine Shanghai Pulmonary Hospital March 16, 2012. 60-year-old woman presented with…. Chronic cough productive of purulent sputum , intermittent fever for one year
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Case Presentation Weijun Cao Department of Respiratory Medicine Shanghai Pulmonary Hospital March 16, 2012
60-year-old woman presented with… • Chronic cough productive of purulent sputum, intermittent fever for one year • Without chest pain, hemoptysis, joint pain, night sweats, or weight loss • Chest CT scan showed bilateral poorly defined nodules and infiltrates, mildly enlarged mediastinal lymph nodes • No response to empirical antibiotic treatment
Medical History • Tuberculosis 20 years ago. Treated with anti-tuberculosis for a peroid of 6 months • Neutropenia • No exposure to chemicals, organic dust, toxic gas, bird feed or smoking
Physical Examination • T：37℃ P：80bpm R：20bpm BP：120/78mmHg • Moderate inspiratory rales at both lung bases • Rashes on her body skin • No clubbing or peripheral edema • Cardiac, abdomen, central nervous system and superficial lymph nodes examinations were unremarkable
Laboratory Tests • Elevated erythrocyte sedimentation rate of 38mm/hour • The ratio of CD4 to CD8 was 0.11 (lower than normal range: 1.45±0.59) • Rheumatoid factor, anti-DNA, anti-nuclear antigen and anti-neutrophil cytoplasmic antibody were negative • Sputum culture was negative. PPD skin test was negative • Pulmonary function tests (PFT) revealed mild restrictive dysfunction
Differential Diagnosis • Infectious • Bacterial • Miliary TB • Fungal: invasive aspergillosis • Parasitic • Malignant • Primary: lung, lymphoma • Metastatic disease • Granulomatosis • Wegener's granulomatosis • Rheumatoid Arthritis, Sarcoidosis, Lymphomatoid Granulomatosis
Is there anything else to do? • The tracheobronchial airways appeared entirely normal by bronchoscopy. There was no bleeding tendency, spontaneously or during the transbronchial lung biopsies • The examination of bronchoalveolar lavage is negative • CT-guard needle aspiration was performed. Some lymphocytes in the lung tissue.
Next Step? • Open Lung Biopsy
Labeling in situ hybridization for EB virus (+), T cells rearrangement (-), B cells heavy chain rearrangement (+).
Final Diagnosis • Pulmonary Lymphomatoid Granulomatosis
Treatment • Treated with chemotherapy of 4 period CHOP regime
Follow up 2011.5.30 2011.7.2 2011.11.3
Follow up 2011.5.30 2011.7.2 2011.11.3
Definition • Pulmonary Lymphomatoid Granulomatosis is related to EBV positive diffuse large B-cell lymphoma
Etiology • An EBV-driven B lymphoproliferative disorder, probably arising in a background of immunodeficiency in most case
Localization • Masses or nodules can involve a variety of organs, most often lung and central nervous system, and kidney; skin may be involved
Clinical features • Up to 70% of patients show bilateral, usually peripheral, lung nodules • Cavitation may or may not be present • Other radiographic patterns include diffuse reticulonodular or alveolar infiltrates, localized infiltrates or a solitary mass.
1，2: Infiltrates 3，4: Solitary mass 5，6: Nodules 7：Infiltrates，solitary mass， nodules，consolidation
Histopathology • Less than 30% of transbronchial biopsies are diagnostic, so a surgical lung biopsy will be necessary in most cases to achieve a diagnosis.
Histopathology • Grade 1 lesions: few or no EBV-infected cells (less than 5 per high-power field), usually lack necrosis • Grade 2 lesions: scattered EBV-infected cells (5-20 per high-power field) and foci of necrosis • Grade 3 lesions: Sheets of EBV-infected cells, necrosis, and are considered a subtype of diffuse large B-cell lymphoma
Immunophenotype • LYG is a T-cell-rich, B-cell lymphoproliferative process, as show by a number of studies, both in lung and in other sites, such as skin.
Treatment • Grade 1， Grade 2：Interferon α-2b • Grade 3：Chemotherapy
