INTRODUCTION:Primary pulmonary lymphomas represent only 4 percent of all Non Hodgkin’s lymphomas. Most of the T-cell derived lymphomas arise in the thymus, skin or nasal cavity and primary T-cell lymphoma of the lung is rare. We report a case of primary pulmonary T-cell lymphoma.
CASE PRESENTATION:A 74 year old male with history of chronic obstructive pulmonary disease (COPD) and coronary artery disease (CAD) presented with progressive cough and dyspnea. Initial Chest Computer Tomography (CT) scan showed bilateral pulmonary nodules in the lower lobes (5 to 15 mm in diameter). Positron Emission Tomography (PET) scan showed increased uptake in the lung nodules consistent with metastatic disease. Serologies for histoplasmosis, blastomycosis and coccidioidomycosis were negative. Sputum grew Mycobacterium avium intracellulare (MAI) and aspergillus. CT guided needle biopsy of a 1.5 cm nodule was nondiagnostic. He was treated for MAI and aspergillus for ∼3 months without benefit and he was hospitalized. On admission he complained of worsening cough with minimal sputum, increasing breathlessness, weight loss and lassitude without fevers or night sweats. On physical examination he appeared cachexic. Examination of chest, abdomen and lymph nodes was unremarkable. Laboratory analysis showed a hemoglobin of 13.1 g/dl, white blood cell count of 6,500/L (neutrophil 62%, eosinophil 2%,basophils 1%, monocytes 14%, lymphocytes 22%), platelets 217 K/cumm, total protein 5.8 g/dl and lactate dehydrogenase (LDH) was 219 IU/L. Epstein Barr Virus (EBV) titers were low. Human immunodeficiency virus (HIV) antibodies and purified protein derivative for tuberculosis (PPD) were non-reactive. Repeat Chest CT scan showed an increase in the size and number of the lung nodules. A surgical lung biopsy was done. Histology showed pulmonary nodules with dense lympho-histiocytic infiltrate with extension to the blood vessel walls. Insitu hybridization for EBV was negative. Immunohistochemical staining showed that the majority of the lymphocytes were CD4+ T-cells. The diagnosis was pulmonary T-cell lymphoma.
DISCUSSIONS:T-cell lymphomas are rare and the majority occur in HIV positive patients. Only a hand-ful of pulmonary T-cell lymphomas in non-HIV patients have been reported. The male to female ratio is 2:1. Patients typically presented with progressive cough, dyspnea, weight loss and fatigue. Three cases were diagnosed incidentally as a result of an abnormal chest x-ray. The most common radiologic finding is bilateral pulmonary nodules but masses, lobar consolidation, adenopathy and pleural effusions have been reported. Surgical lung biopsy is required for diagnosis. Immunophenotyping shows a monoclonal population of T-cells. The primary differential diagnosis is a reactive T-cell infiltrate. There are no characteristic genetic abnormalities associated with pulmonary T-cell lymphomas. Treatment with CHOP (cyclophosphamide, adriamycin, vincristine and prednisone) has shown variable success. The overall 5 year survival is < 25%. Clinical and radiographic response to high dose systemic steroids has been described. In one case, steroids improved a patient’s clinical status so that an open lung biopsy could be performed. Surgical resection has been reported in two patients with focal lung involvement.
CONCLUSION:Primary pulmonary T-cell lymphoma is rare, the diagnosis requires a surgical lung biopsy, immunophenotyping shows a monoclonal population of T-cells, treatment options are limited and the prognosis is poor. Pulmonary T-cell lymphoma should be included in the differential diagnosis of bilateral pulmonary nodules.
DISCLOSURE:Nureain Mirza, No Financial Disclosure Information; No Product/Research Disclosure Information