Immunodeficiency, pulmonary lymphoreticular infiltration, paraproteinemia, and terminal lymphoma

Abstract

A 64-year-old woman was found to have pulmonary lymphoreticular infiltration associated with a cryoprecipitate monoclonal paraprotein. History and investigation suggested that these features may have complicated long-standing immunodeficiency. Subsequent postmortem revealed a lymphocytic lymphoma of the lung and stomach. It is postulated that pulmonary lymphoreticular infiltration and monoclonal parapruteinemia might be a consequence of defective immunoregulation with excessive B cell proliferation. The eventual lymphoma is thought to represent malignant transformation. Case HeportThe patient, aged 64. was admitted to Sir Charles Gairdner Hospital in July 1975 for investigation of increasing respiratory difficulty. Symptoms had progressed over a period of at least six years and had been paralleled by radiologic evidence of increasing pulmonary infiltration predominantly in the upper zone (Figs. IA-C). In the year before admission a left pleural effusion had developed. Aspiration and culture were negative for tuberculosis, and treatment with prednisolone, 10 mg daily, was begun.There was a past history of recurrent rehpiratory infections and of four attacks of measles in childhood. Subsequently. frequent episodes of chest infection with purulent sputum developed, including pleurisy at age 25 and lobar pneumonia at age 42. Recent attacks were accompanied by wheezing. She was a nonsmoker, and there was no history of allergy. chest pain. fever, weight loss, or night sweats.Two brothers, both smokers. died of lung cancer, and the mother also died of cancer, type unknown. Rheumatoid arthritis and asthma were present in other family members.Examination revealed the absence of fever. a pulse rate of 84 beatshinute and blood pressure of 160190. Dyspnea was evident on minimal exertion. There were signs of a moderate left pleural effusion. and coarse inspiratory crepitations were present in both upper zones. The spleen was just palpable below the left costal margin. There was no significant lymphadenopathy or salivary gland enlargement.Initial investigations included a hemoglobin value of 115 &I, a leukocyte count of 5600/mm3. and erythrocyte sedimentation rate of 42 mm (Westergren). The chest x-ray on admission showed striking changes in both lung fields (