Hodgkin lymphoma

Introduction: Primary Non Hodgkin lymphomas rarely affects the lungs accounting for [is less than] 1% of all of the lymphoma cases. The cases reported until now have all been B cell lymphomas considered to originate from mucosa associated lymphoid tissue. The majority of the patients with low grade lymphoma are asymptomatic and the pulmonary lesions are usually discovered during a routine chest roentgenogram. Most patients with high grade lymphoma presents with fever, weight loss, cough, dyspnea and hemoptysis. The case here described is that of a 68 year old woman with an aggressive 2 week course of malaise, fatigue and weight loss associated with pulmonary nodular infiltrates. An open lung biopsy and special cell markers confirmed the diagnosis of primary pulmonary T cell lymphoma. In contrast to the slower course of B cell lymphoma, the rapid progression of the disease resulted in the patient death 3 weeks after diagnosis.

Case Report: A 68 year old woman visiting from Guatemala was admitted with history of syncopal episodes, weakness, fatigue and weight loss. The patient was seen two days before admission for sinusitis and was treated with antibiotics. On physical exam the patient was tachycardic, afebrile, 02 sat 96% on RA with congested and purulent nose. The rest of the exam was normal. No nodes or visceromegaly were noted. Chest roentgenogram showed patchy bilateral nodular densities, without pleural effusions. A subsequent chest CT showed multiple lung nodules bilaterally without mediastinal or hilar nodes. A head, abdomen and pelvis CT were normal. A CBC showed mild pancytopenia with normal differential. LFT's were elevated with AST 223 and ALT 170. A hepatitis profile was negative. Urinalysis showed 3+ protein, trace blood, 3-5 granular casts. In her 3rd hospital day the patient developed fever. Blood, sputum and urine cultures were negative for common pathogens. A 5 IU PPD was negative. Sputum for AFB were negative. She developed refractory hypoxemia. Urine and serum electrophoresis did not show monoclonal spike. CANCA was also negative. ANA + 1:640 Antinative DNA -. A bone marrow biopsy showed hemophagocytosis. A bronchoscopy showed normal anatomy. BAL, brushings and transbronchial biopsy were negative for malignancy and infection. An open lung biopsy was performed. The findings were compatible with Lymphoplasmocytoid Lymphoma usually a B cell lymphoma. Typification of the cells showed that it was a T cell Lymphoma with moderate grade of malignancy. After surgery the patient remained intubated due to respiratory failure. She was treated with chemotherapy (Cytoxan, Adriamycin, Vincristine and Prednisone). Subsequently the patient developed a neutropenic syndrome, became septic and despite aggressive supportive therapy ed. A postmortem examination was denied by the family members.

Discussion: Primary Non Hodgkin lymphoma rarely affects the lungs ([is less than] 1%).[1,2] They are usually low grade B cell lymphoma considered to originate from mucosa associated lymphoid tissue of the bronchus.[1] Treatment for low grade localized lymphomas is surgery plus XRT.[1] High grade lymphoma are usually treated with chemotherapy.[1] There has been no case report of primary pulmonary lymphoma of the T cell type in the literature. This type of lymphoma is also called Immunocytoma, an immunoglobulin-producing neoplasia of low grade malignancy.[2,3] Our patient was atypical because the tumor was of the T cell type with moderate grade of malignancy. The absence of monoclonal spikes on serum and urine PEP, which was reported to occur in approximately 30% of positive in B cell primary pulmonary lymphomas,[1] could be explained by the fact that it was T cell type. The progression of this patient's disease suggests a more aggressive and malignant course.

Conclusion: We conclude that primary pulmonary lymphomas can present as bilateral pulmonary nodules. The diagnosis is made by open lung biopsy. The tissue type will determine the treatment.

References

[1] Primary pulmonary lymphomas. Chest 1993; 103:201-208

[2] Primary non-hodgkin's lymphoma and pseudolymphomas of lung: a study of 161 patients. Human Pathology 1983; 14:1024-1038

[3] Updated Kiel classification for lymphomas. Lancet 1988; 1:293-293

[4] Primary Inmunocytoma of the lung: the diagnostic value of BAL. Thorax 1985; 40:542-543

Juan Pablo de Torres, MD, L. Kenney, MD, M. Girish, MD, B. Celli, MD--St Elizabeth's Medical Center, Boston, MA

COPYRIGHT 1999 American College of Chest Physicians
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