Associates' Presentations
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Primary serosal lymphoma.
Leah L. Dietrich, M.D. (Associate) Jordan Berlin, M.D.. University of Wisconsin Hospital and Clinics, Madison, WI
A 68 year old female with chronic coccidiomycoses suppressed with fluconazole presented with symptoms of chest pain and dyspnea. Examination revealed increased jugular venous pressure and no pulsus paradoxus or lymphadenopathy. Lungs were clear with decreased breath sounds and dullness to percussion at bilateral bases. A pericardial friction rub was present. Laboratory studies were normal except: protein 2.6, alkaline phosphatase 120, GGT 103, and LDH 544. Her HIV status was negative. Echocardiogram revealed a large pericardial effusion. Chest X-ray showed bilateral pleural effusions, an enlarged cardiac silhouette, and no infiltrates or pulmonary vascular congestion. Thoracentesis yielded clear yellow fluid with LDH 3144, and 52,250 white cells with 34% neutrophils and 64% lymphocytes. Pleural fluid Gram's stain, bacterial cultures, and tests for AFB, coccidiomycosis and other fungi were negative. Large cells with an increased N:C ratio and prominent nucleoli were present on cytology. Immunohis tochemistry classified these cells as monoclonal B-cells indicating large B-cell lymphoma. CT scans of the chest and abdomen revealed pleural effusions, a large pericardial effusion, enhancement of the pericardium, and no significant adenopathy or additional sites of lymphoma. A diagnosis of primary serosal lymphoma (PSL) was made and chemotherapy was initiated.
Discussion - We report a case of primary serosal lymphoma in a patient with chronic coccidiomycoses. Lymphoma involving the heart, pericardium, or pleural spaces is not a rare finding, however, primary serosal lymphoma is quite rare. PSL is defined as pericardial, peritoneal or pleural lymphomatous effusions without an identifiable mass. It has been reported in several patients with AIDS and in two immunocompetent patients. An association with human herpes virus-8 (HHV-8), Kaposi's sarcoma and EBV has been noted. An etiologic association with HHV-8 has been suggested. The most common presenting symptoms are chest pain, dyspnea and fever. Histology is most often large cell lymphomas of B-cell lineage. All PSLs tested for cell surface markers express CD 45. Cytology and flow cytometry may be the only diagnostic tools as nodal and organ involvement is absent. CHOP is the treatment of choice but prognosis is poor. Our patient fits the above definition, description of symptoms and histologic features but has no known immunodeficiency. Interestingly, it has recently been proposed that disseminated or chronic coccidiodomycosis may indicate an as yet undiscovered underlying immunodefienciency.
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