Case Reports: Sunday, October 23, 2011 |

Pulmonary Lymphomatoid Granulomatosis : An Uncommon Cause of Multiple Pulmonary Nodules FREE TO VIEW

Aanchal Kapoor, MD
Chest. 2011;140(4_MeetingAbstracts):38A. doi:10.1378/chest.1118768
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INTRODUCTION: Multiple pulmonary nodules are commonly secondary to metastatic malignancy or are infectious in etiology. We describe an uncommon pulmonary disorder characterized by multiple pulmonary nodular lesions with lymphocytic invasion of vascular walls on biopsy called pulmonary lymphomatoid granulomatosis (PLG).

CASE PRESENTATION: 72 year old male with history of COPD presented with sudden onset of progressively worsening weakness since laminectomy done three weeks prior to the presentation. One week after the surgery he developed cough and low grade fever for which he was treated with antibiotics by his family physician for presumable bronchitis. Six days later he was admitted to the hospital with worsening fever and shortness of breath. He denied any headache, visual disturbances, back pain, seizure or neck stiffness. On examination, he was tachycardic, tachypneic and was febrile. Chest X-ray revealed bibasilar atelectasis. He was started on levofloxacin and a chest CT was done to evaluate for pulmonary embolism. CT showed numerous nodules scattered throughout the left lung. Bronchial washings were sent for routine cultures and cytology. While awaiting the report, he developed pancytopenia, worsening shortness of breath, abdominal pain and a repeat CT of the chest along with abdomen and pelvis was done. Results were consistent with bilateral worsening nodules both in size and in number and nodular lesions on the left adrenal gland. Pancytopenia worsened progressively. He developed 0.5 cm macular oval erythematous lesions on his left lower extremity with raised border, largest at left ankle. Given its rapidity in progression, we were highly concerned about infectious cause - fungal, particularly disseminated histoplasmosis, being a significant concern given lack of response to antibacterials, negative bacterial cultures, pancytopenia, rash and pulmonary nodules. Noninfectious causes were also entertained such as vasculitis and autoimmune phenomenon. He was started on amphotericin, vancomycin, ciprofloxacin and aztreonam empirically. Fungal serology was sent along with PCR for CMV, EBV and work up for vasculitis. Skin biopsy was performed which was nondiagnostic. CMV titre came back >600 K, EBV titre was > 400 K, though unlikely to cause pulmonary nodules, he was started on ganciclovir. Biopsy of adrenal nodule was done which revealed coagulative necrosis. Cytology from bronchial washings came back for atypical cells with hyperchromatic nuclei suspicious for malignancy. Finally Video assisted thoracoscopic surgery (VATS) gave away the diagnosis of lymphomatoid granulomatosis grade 3. Patient was started on solucortef and rituximab. Initially he did show response but soon deteriorated, developed tumor lysis syndrome, worsening renal function and family withdrew care.

DISCUSSION: PLG likely represents a lymphoproliferative disorder in the family of Epstein-Barr virus (EBV)-associated B cell lymphomas. It usually presents between the ages of 30 and 50. PLG can be seen in patients with an underlying immunodeficiency (eg, Wiskott-Aldrich, X-linked immunodeficiency, immunosuppression, organ transplant). These immune defects may lead to an abnormal host response to EBV infection, resulting in lymphomatoid granulomatosis. The lung is the most commonly involved organ; the skin and neurologic system may be affected separately or concurrently. The most common presenting symptoms and signs include cough, fever, rash/nodules, malaise, weight loss, neurologic abnormalities, dyspnea, and chest pain. Chest imaging studies typically show multiple ill-defined nodular opacities. Nodules are preferentially located along the bronchovascular structures or interlobular septa. Thin walled cystic lesions may also be present. The histopathologic diagnosis of PLG requires a triad of polymorphic lymphoid infiltrates, transmural infiltration of arteries and veins by lymphoid cells ("angiitis"), and focal areas of necrosis within the lymphoid infiltrates. The clinical course of lymphomatoid granulomatosis is variable, ranging from remission without treatment to death within 2 years from malignant lymphoma.

CONCLUSIONS: Pulmonary lymphomatoid granulomatosis (PLG) should be considered in the differential diagnosis of multiple pulmonary nodules.

Reference #1 Liebow AA, Carrington CR, Friedman PJ. Lymphomatoid granulomatosis. Hum Pathol 1972; 3:457.

Reference #2 Jaffe ES, Wilson WH. Lymphomatoid granulomatosis: pathogenesis, pathology and clinical implications. Cancer Surv 1997; 30:233.

Reference #3 Pisani RJ, DeRemee RA. Clinical implications of the histopathologic diagnosis of pulmonary lymphomatoid granulomatosis. Mayo Clin Proc 1990; 65:151.

DISCLOSURE: The following authors have nothing to disclose: Aanchal Kapoor

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