Come S E, Jaffe E S, Andersen J C, Mann R B, Johnson B L, DeVita V T, Young R C
Am J Med. 1980 Nov;69(5):667-74. doi: 10.1016/0002-9343(80)90416-7.
A leukemic phase occurred in 30 (14 percent) of 214 patients with non-Hodgkin's lymphoma. To determine the significance of peripheral blood involvement in each type of NHL, patients were subdivided according to a modified Rappaport classification. Each histologic subtype presented a homogeneous clinical picture which differed from that seen in other histologic subtypes. Of particular note was the recognition of two distinctive cytologic and clinical subtypes within the category of nodular lymphoma, poorly differentiated lymphocytic lymphoma (NPDL). In one subtype, the predominant cells had cytologic features akin to those of lymphoblasts. In these cases, although the interval to peripheral blood involvement was variable, the median leukemic survival was only two months. In contrast in conventional NPDL the median leukemic survival was 43+ months, and peripheral blood involvement did not appear to exert an independent effect on prognosis. In diffuse large cell lymphomas the median leukemic survival was 0.5 months, with peripheral blood involvement appearing as a terminal event associated with unresponsive disease in multiple sites. The recognition of adult lymphoblastic lymphoma as a clinicopathologic entity with a high risk of leukemic conversion, 100 percent in this study, is also confirmed.
