Harousseau J L, Flandrin G, Tricot G, Brouet J C, Seligmann M, Bernard J
Cancer. 1981 Sep 15;48(6):1302-8. doi: 10.1002/1097-0142(19810915)48:6<1302::aid-cncr2820480609>3.0.co;2-q.
Richter's syndrome (RS) has been defined as "histiocytic" lymphoma (HL) or Hodgkin's disease (HD) supervening in the course of chronic lymphocytic leukemia (CLL) and related disorders. The clinical, histologic, and immunologic findings in 25 cases (11 women, 14 men) of RS are presented. The initial diagnosis was CLL in 19 cases, diffuse well-differentiated lymphocytic lymphoma in 2 cases, and Waldenstrom's macroglobulinemia in 4 cases. The interval between the initial diagnosis and that of RS ranged from 0 (two cases) to 120 months (median 49 months). At the time of diagnosis of RS, the initial lymphoproliferative disorder was in apparent complete remission in only two cases. The lymphoma was disseminated in at least 18 cases. The overall median survival was four months, but complete remission was achieved in six cases and has been maintained for 15 to 77 months. In four of these six cases, the RS was localized. The histologic diagnosis of HD was made in only two cases. In the other 23 cases, the diagnosis was HL, but in five of these cases, the proliferation was heterogeneous and was considered as an early aspect of HL. Immunologic studies of lymph node cell suspensions were performed in seven cases. In all cases, the B-lymphocytic origin of the lymphoma cells could be ascertained. Detailed studies in four cases showed that lymphoma cells carried SIg of the same isotype and light chain type as that of SIg detected on CLL cells or of monoclonal serum Ig. In these cases, the lymphoma was actually related to the initial B-cell chronic lymphoid disease.
里氏综合征（RS）被定义为在慢性淋巴细胞白血病（CLL）及相关疾病病程中出现的“组织细胞性”淋巴瘤（HL）或霍奇金病（HD）。本文报告了25例（11例女性，14例男性）RS的临床、组织学和免疫学表现。初始诊断为CLL的有19例，弥漫性高分化淋巴细胞淋巴瘤2例，瓦尔登斯特伦巨球蛋白血症4例。初始诊断至RS诊断的间隔时间为0（2例）至120个月（中位49个月）。在诊断RS时，仅2例初始淋巴细胞增殖性疾病处于明显完全缓解状态。至少18例淋巴瘤已播散。总体中位生存期为4个月，但6例实现了完全缓解并维持了15至77个月。在这6例中的4例中，RS为局限性。仅2例作出HD的组织学诊断。在其他23例中，诊断为HL，但其中5例增殖具有异质性，被认为是HL的早期表现。对7例进行了淋巴结细胞悬液的免疫学研究。在所有病例中，均可确定淋巴瘤细胞的B淋巴细胞起源。对4例的详细研究表明，淋巴瘤细胞携带的表面免疫球蛋白（SIg）与在CLL细胞上检测到的SIg或单克隆血清Ig具有相同的同种型和轻链类型。在这些病例中，淋巴瘤实际上与初始B细胞慢性淋巴细胞疾病相关。
