GIANT-CELL GRANULOMATOUS PULMONARY AND MYOCARDIAL LESIONS IN NECROBIOTIC XANTHOGRANULOMA WITH PARAPROTEINEMIA

Objective: To report three cases of pulmonary or myocardial disease (o r both) and necrobiotic xanthogranuloma, Material nod Methods: Giant c ell granulomas of the lung and myocardium were demonstrated in three p atients who had pulmonary and myocardial lesions of necrobiotic xantho granuloma in conjunction with skin lesions, leukopenia, paraproteinemi a, and complement deficiencies, The patients were two men who were 47 and 64 years of age and a 39-year-old woman. Results: Biopsies of skin and visceral lesions showed asteroid and cytoplasmic inclusions, B-ce ll lymphoid nodules were found, In one of the male patients, a major c lonal T-cell receptor gene rearrangement was detected in the periphera l blood, Prednisone was ineffective in two of the patients. The other patient experienced regression of skin lesions and diminishment of a c hest nodule after receiving alkylating agent therapy. Conclusion: Esta blishing the correct diagnosis is important, and apparently it is poss ible to establish the nature of the myocardial and pulmonary lesions w ith use of appropriate scans and by biopsy, Successful treatment of ne crobiotic xanthogranuloma skin lesions with corticosteroids or alkylat ing agents (or both) implies that evolution of serious disease that co mpromises the heart and lungs could he controlled.