Primary Pulmonary AIDS-Related Lymphoma (PPL)

A 39yrs.-old transgender male- female patient presented with unexplained weight loss and oropharyngeal candida infection and was diagnosed with HIV with a CD4- count of 11/µl. Further workup included an abdominal ultrasound which revealed a pleural-based, hypervascularized pulmonary mass of 36 × 21 mm in the left dorsal lobe. This had initially been missed on chest x-ray but was detected there as well on reevaluation of the exam and on the subsequently performed CT-scan.

A transthoracic needle biopsy of this lesion was performed showing infiltration of a diffuse-high grade large B-cell non-Hodgkin lymphoma (NHL). An EBV-proliferation could not be proven. There were no suspicious lymph nodes or infiltrations of bone marrow and the abdominal organs, a bronchoscopy was also negative.

Extranodal involvement is common in HIV associated lymphomas, being the second most malignancy in this group. The most frequent location is in the CNS, GI system, liver, spleen and bone marrow. A primary pulmonary AIDS-related NHL is very rare and occurs in < 10% of cases with a variety of clinical forms including pleural effusion, interstitial and alveolar lunge disease and peripheral nodules as in our case. Clinical presentation is nonspecific, with “B” and respiratory symptoms. This lymphoma is generally a high-grade B-cell non-Hodgkin lymphoma and contrary to our case Epstein-Barr virus is strongly associated with the pathogenesis of these tumors.

Median survival in primary pulmonary lymphoma is reported to be only four months due to tumor growth and opportunistic infections. Therapy in our patient consisted of 6 cycles of Rituximab- CHOP (Cyclophosphamide, Doxorubicine, Vincristine, Prednisone). It was complicated only by a reactivation of a salmonella enteritis and the lymphoma size appears stable now, more than 7 months after the last chemotherapy course.