Clinical Profile

The largest series (39 cases) addressing RS emerged from a retrospective analysis of the M.D. Anderson Cancer Center computerized database of1374 CLL cases diagnosed between 1972 and 1992 (21). The authors included in their definition of RS cases of immunoblastic non-Hodgkin's lymphomas. The median interval between the initial diagnosis and the onset of RS was approximately 4 yr. Salient presenting features included male predominance (62%), progressive lym-phadenopathy (64%), systemic symptoms (59%), and extranodal involvement (41%), mainly pleura and central nervous system, and increased levels of lactic dehydrogenase (82%). Several observations made by the authors merit emphasis, as they have major clinical and pathogenic relevance: whereas nearly half of the patients had stage III/IV CLL at the onset of RS, 25% were in a complete remission induced by a fludarabine-based regimen. Likewise, whereas 82% of patients had been treated for CLL prior to the onset of RS, 8% had not. Thus, neither disease stage nor successful treatment, including that with fludarabine monophosphate, seemed to affect the risk for CLL patients to develop RS. Alternatively, three or more cytogenetic abnormalities were detected in 9 of the 11 RS patients who exhibited abnormal karyotypes, confirming previous reports that complex clonal chromosomal abnormalities increase the risk for RS (22). Finally, of the 33 patients (85%) who were treated for RS (32 with systemic chemotherapy, including 9 who received adjuvant radiation therapy, and 1 with radiotherapy alone), 10 achieved a complete remission. However, only three survived longer than 1 yr, and the group's median survival was 5 mo from the onset of RS, exposing the inability of our current therapeutic modalities to reverse or control this disorder's relentless progression and rapid demise.

An earlier series from Paris (19) had reported 25 cases of RS, identified by the authors as "histiocytic lymphoma in 23 cases and Hodgkin's disease in 2 cases": 19 arose from CLL, 4 from Waldenstrom's macroglobulinemia, and 2 from small cell lymphoma (SCL). The time interval to the diagnosis of RS was 49 mo and the median survival was 4 mo even though complete remissions were achieved in six, four of whom had localized disease. An additional relatively large series of nine RS patients (23) mirrored the M.D. Anderson Cancer Center and Paris reports with respect to clinical features, time interval to RS, and a dismal median survival.

As described above, most cases of RS occur some time after the primary clonal disease has been recognized and usually treated. However, in addition to this "classic" presentation, a "variant" form of RS is said to occur when RS is diagnosed concomitantly with a previously unrecognized low-grade lymphoproliferative disease (19,24). From a clinical standpoint, these cases appear indistinguishable from the classic type, except for a higher response rate (50% complete remission) reported by one group (24). Hodgkin's disease supervening in patients with CLL has been referred to as HD variant of RS, whereas it should more appropriately be viewed as a second malignancy. According to the M.D. Anderson Cancer Center CLL group (25), these patients present with a histological profile indistinguishable from de novo HD, with a median time to HD diagnosis of 45 mo. However, unlike patients with de novo HD, they exhibit a poor response to chemotherapy (25%), and a short median survival (12 mo).

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