Lymphomatoid granulomatosis

Abstract

Lympliomatoid granulomatosis, defined on the basis of study of the first 40 patients, is an angiocentric and angiodestructive lymphoreticular proliferative and granulomatous disease involving predominantly the lungs. The infiltrate is polymorphous Nrith varying numbers of cells in mitosis and may contAm atypical, especially plasmacytoid, cells of reticuloendothelial origin, but, remarkably, usually tends to spare lymph nodes, spleen, and bone marrow. Rarely, however, generalized atypical lymphoid hyperplasia may precede the onset of pulmonary lesions, sometimes by many years. The question usually raised is whether the process represents lymphoma or Wegener's granulomatosis. Men predominate in a ratio of two to one, and most patients are in early middle age. Most complAm of cough, often productive, fever, and dyspnea, in some combination. Radiographically, pulmonary lesions predominate in the lower lung fields peripherally and bilaterally and resemble metastases, but tend rapidly to wax and wane, and there is no hilar adenopathy. Cutaneous involvement occurs in 45 per cent of the patients and may call attention to silent pulmonary lesions. Nodular renal lesions similar to those of the lungs, but not glomerulonephritis, occur likewise in 45 per cent and there may be scattered lesions in other organs. Remarkably, central nervous system involvement occurs in at least 20 per cent of the patients and peripheral neuritis almost as frequently. Although many patients respond well to treatment, 26 of the original 40 have died, in 14 instances in consequence of involvement of the lung. Massive hemorrhage from cavitative lesions was the mechanism of death in three. In six other patients, however, pulmonary lesions were healed or healing and death occurred from central nervous system or other involvement. In 25 per cent of the ultimate fatalities the patients had survived two years or more, one for as long as seven years. Progress to atypical lytnpltoma with involvement of lymph nodes and other reticuloendothelial tissues occurred in five patients (13 per cent). Six among the 14 survivors have lived more than four years after the diagnosis was made, and four are apparently free of disease. Although this has been credited to steroid therapy in three patients, another, with no evidence of disease after eight years, received no therapy whatever.