Fayad L, Robertson L E, O'Brien S, Manning J T, Wright S, Hagemeister F, Cabanillas F, Keating M J
Department of Hematology, University of Texas M.D. Anderson Cancer Center, Houston, USA.
Leuk Lymphoma. 1996 Oct;23(3-4):333-7. doi: 10.3109/10428199609054836.
Patients developing Hodgkin's disease (HD) after a diagnosis of chronic lymphocytic leukemia (CLL), are frequently included in a series of patients with Richter's syndrome (RS). We sought to determine the natural history of the association of CLL and HD. Over a 21 year period, 1374 patients with CLL have been registered in our computer data base. Seven cases of CLL and HD have been documented and confirmed. The median age of these patients was 71 years (range 44-77) and clinical features included male gender (86%), B symptomatology (86%), rapidly progressive lymphadenopathy (71%), prior CLL therapy (71%), advanced Ann Arbor stage (86%), marrow involvement with HD (43%), and autoimmune hemolytic anemia (29%). HD was documented by excisional lymph node biopsy in six cases and splenectomy in one. Mixed cellularity HD was shown in six and nodular sclerosis in one. Five of the biopsies revealed intervening areas consistent with small lymphocytic lymphoma. The Sternberg-Reed (SR) cells were CD15+ in 6/7 cases, and Ki-1+ in the 6 patients tested. CD45 and CD20 staining of the SR cells was nonreactive. The median time to development of HD was 45 months (range 0 to 96). The overall responses to different chemotherapy regimens was approximately 25% with only one CR. Six patients have died at 3, 9, 10, 13, 15 and 36 months and one patient is alive with progressive disease at 11 months. Our data suggests that CLL patients have a heightened risk for HD, features of advanced HD on presentation, and a poor response rate with short survival.
在诊断为慢性淋巴细胞白血病（CLL）后发生霍奇金淋巴瘤（HD）的患者，常被纳入里氏综合征（RS）患者系列中。我们试图确定CLL与HD关联的自然病程。在21年期间，1374例CLL患者已登记在我们的计算机数据库中。已记录并确诊7例CLL合并HD。这些患者的中位年龄为71岁（范围44 - 77岁），临床特征包括男性（86%）、B症状（86%）、快速进展的淋巴结病（71%）、既往CLL治疗史（71%）、Ann Arbor分期晚期（86%）、HD累及骨髓（43%）和自身免疫性溶血性贫血（29%）。6例通过切除性淋巴结活检确诊HD，1例通过脾切除术确诊。6例显示为混合细胞型HD，1例为结节硬化型。5例活检显示存在与小淋巴细胞淋巴瘤一致的中间区域。7例中有6例的施特恩伯格 - 里德（SR）细胞CD15阳性，6例检测的患者中SR细胞Ki - 1阳性。SR细胞的CD45和CD20染色无反应。发生HD的中位时间为45个月（范围0至96个月）。对不同化疗方案的总体缓解率约为25%，仅1例完全缓解（CR）。6例患者分别在3、9、10、13、15和36个月死亡，1例患者在11个月时病情进展仍存活。我们的数据表明，CLL患者发生HD的风险增加，就诊时具有晚期HD的特征，缓解率低且生存期短。
