Giant cell granulomatosis of lymph nodes with a fatal course

We report two examples of an unusual expression of fatal granulomatosis with primary lymph node location, characterized histologically by numerous giant cells of the Langhans' type, effacement of the nodal structure, mixed lymphohistiocytic infiltrate, and marked histiocyte cell maturity, all features which initially did not exclude the possibility of a granulomatous process of infectious origin. The clinical features of these cases include cellular or combined immunodeficiency, a possible family history and a rapidly fatal course. The present article emphasizes the great similarity which can exist between histiocytic and granulomatous disorders, the great variability of histiocytic diseases from the pathological view-point, the presence of borderline examples, and the several overlapping features of different entities. Specifically, the most remarkable finding of these two cases is the existence of numerous mature giant cells of the Langerhans' type within a massive obliteration of the nodal structure. Furthermore, these giant cells characterized by a strikingly orderly disposition of peripheral nuclei in a wreath-like pattern, frequently suggesting the structure of Touton's giant cells.