Lymphocyte-depleted hodgkin's disease. Clinicopathologic review of 25 patients (original) (raw)

1986, The American Journal of Medicine

Clinicopathologic material from 25 patients with lymphocyte-depleted Hodgkin's disease was reviewed. The median age of the patients was 57 years. The patients had no prior diagnosis of Hodgkin's disease and were divided according to pathologic subtype of lymphocyte-depleted Hodgkin's disease: 11 diffuse fibrosis, 10 reticular, and four not otherwise specified. The clinical presentation included B symptoms of fever, weight loss, or night sweats (92 percent), subdiaphragmatic disease (88 percent), frequent marrow involvement (58 percent), and advanced-stage disease (100 percent). Four of 11 patients with diffuse fibrosis had peripheral adenopathy as compared with seven of 10 patients with the reticular subtype (p = 0.3); 10 of 11 patients with diffuse fibrosis had marrow involvement compared with two of nine patients with the reticular subtype (p = 0.008). Among patients who received chemotherapy, median survival was longer in the dtffuse fibrosis subtype (nine patients, 39 months) than in the reticular subtype (IO patients, 10 months), p = 0.005. of the 17 patients who received more than one cycle of combination chemotherapy with mechlorethiamine, vincristine, procarbazine, and prednisone, the median survival was 38 months with 11 (85 percent) complete remissions. In eight patients, disease remains in remission (12 to 127 months) with five patients surviving beyond five years. These results indicate that lymphocyte-depleted Hodgkin's disease has at least two clinicopathologk subtypes and is curable if adequate therapy can be given. Lymphocyte-depleted Hodgkin's disease has long been recognized as a distinct, albeit rare, entity in the major subclassifications of Hodgkin's disease [l-3]. Jackson and Parker [l] identified three subtypes based on clinicopathologic features and on the theory that they may evolve into another subtype. The sarcoma form, which was the forerunner of lymphocyte-depleted Hodgkin's disease, occurred in older patients and had a more aggressive course than the paragranuloma or granuloma subtype. Lukes et al [2] subdivided Hodgkin's disease into six subtypes that included two variants of lymphocyte-depleted Hodgkin's disease: diffuse fibrosis and reticular subtypes. The diffuse fibrosis subtype was characterized by lymphocyte depletion with a background of nonbirefringent proteinaceous stroma and infrequent Reed-Sternberg cells. The reticular variant was characterized by numerous Reed-Sternberg cells with lymphocyte depletion and without fibrosis. The Rye modification, which was introduced for clinical simplification and for grouping of prognostically similar groups, combined the two variants into one subtype [3]. In most series, lymphocyte-depleted Hodgkin's disease has been associated with a worse prognosis than the other subtypes [4,5]; how