Giant Follicular Lymphoblastoma (Brill-Symmers' Disease)

DiSEASE processes affecting the reticuloendothelial system fall into three main groups: 1. I?eactive.-'The changes found in the marrow, spleen, and lymphoid tissue are produce(d in response to infection, acute or chronic. 2. Hyperplastic.-The causative factor is unknown. 'I'his group includes the leukemias, Hodgkin's disease, and giant follicular lymphoblastoma. 3. Neoplastic.-The commonest are reticulum cell sarcoma and lymphosarcoma. Around some of these conditions, in particular Hodgkin's dlisease and lymphosarcoma, there has grown a voluminous literature. Giant follicular lymphoblastoma, although not uncommon, has received scant attention. 'I'he condition is of relatively recent recognition. Brill (1925) describe(l two cases. Symmers (19:38) in an exhaustive account recorded twenty-five cases. Between these years some forty-three cases appeared in the literature. Giant follicular lymphoblastoma is almost invariablv mistaken by the clinician for Hodgkin's (lisease, with which it has many features in common. Both are characterised by localised or generalised enlargement of the lymph glands. Splenomegaly may occur in either. Systemic manifestations such as fever and wasting, however, point to Hodgkin's (lisease rather than giant follicular lymphoblastoma. Pathologically the diseases are easily distinguished. In Hodgkin's disease the histological picture is complex, while in Brill-Symmers' disease the picture is comparatively simple, consisting, as it does, of hyperplasia of the lymph follicles, and of an increase in their number. The (listinction is of importance from the prognostic and therapeutic aspects, since giant follicular lvmphoblastoma in its early stages is a reversible hyperplasia and readily amenable to treatment by (leep X-ray therapy in comparatively small (loses. Examination of the biopsy andl post-mortem material in the Institute of Pathology reveals that seven cases of this disease occurred during the ten-year period 1937-46. The pathological features of these cases will be described and the clinical history will be given in such detail as the records permit.