Clinical features of nodular paragranuloma (Hodgkin's disease,lymphocyte predominance type,nodular)

Summary Clinical aspects of 145 cases of nodular paragranuloma (nodular subtype of lymphocyte predominance type of Hodgkin's disease) were investigated. There was a marked male predominance, and the age curve showed a peak in the 4th decade. In a majority of cases lymphadenopathy developed within 1 year. General (B) symptoms were observed in only 15 patients. The most frequent sites of primary involvement were cervical, axillary, and inguinal lymph nodes. Other organs were rarely involved. At the time of diagnosis 50% of patients were in stage I, 21% in stage II, 22% in stage III, and 7% in stage IV. The prognosis was usually favorable or very favorable and depended on the stage of disease at diagnosis and on the age of the patient. Patients with stage I or III disease without splenic involvement had about the same probability of survival as the normal population. Stage III patients with splenic involvement had a lower probability of survival. The prognosis for stage II was also less favorable. Patients in stage IV had the lowest probability of survival. Closer analysis of the ten stage IV cases revealed two groups with different outcomes. Four cases showed progressive disease that did not respond to treatment and led to death within 12 months. The second, more favorable form (6 patients) responded well to chemotherapy. Nine patients in stage I who were not treated after lymph node biopsy were free of disease even after periods of up to 14 years. A total of 52 patients had one or more relapses. The recurrent tumors developed locally in a majority of cases. There was transformation of nodular paragranuloma into another subtype of Hodgkin's disease in only four cases. Five cases showed transformation into large-cell tumors that resembled immunoblastic lymphoma and require further immunological study.