Maryniak R K, Konecki R
Samodzielnej Pracowni Patomorfologii, Instytutu Hematologii, Warszawie.
Acta Haematol Pol. 1991;22(1):165-9.
Two cases of Richter's syndrome are reported (in a 62 and 64 years old man) consistent with the appearance of B cell lymphoma of high malignancy in the course of CLL (low malignancy B cell lymphoma). In one patient, after 8-, and in the other one--after 53 months since the diagnosis of CLL, there was rapid clinical deterioration with lymphadenopathy, hepato- splenomegaly, fever and progressive cachexia, anemia and thrombocytopenia and leukopenia, unrelated to treatment. Both patients died, 4 and 3 months respectively, since the appearance of these symptoms. In the first cases Richter's syndrome was diagnosed histopathologically from the autopsy material. In the liver, spleen, adrenals and bone marrow, in addition to the characteristic infiltrates of CLL (small lymphocytes) there were areas of large cell proliferation consistent with high malignancy lymphoma. In the other case, the infiltrates of large cell lymphoma were found in the gall bladder removed because of acute cholecystitis, and in the lymph node from the hepatic hilar area. Immunocytochemical studies performed on the biopsy material indicated that the neoplastic cells had markers of B lymphocytes and cytoplasmic IgM kappa, as lymphocytes of CLL. In patients with CLL, who display rapid clinical deterioration and general symptoms with cachexia, the possibility of Richter's syndrome should be considered, and appropriate morphological studies performed.
报告了2例里氏综合征病例（分别为一名62岁和一名64岁男性），其表现符合慢性淋巴细胞白血病（低恶性B细胞淋巴瘤）病程中出现的高恶性B细胞淋巴瘤。在一名患者中，自慢性淋巴细胞白血病诊断后的8个月，而在另一名患者中——自诊断后的53个月，出现了快速的临床恶化，伴有淋巴结病、肝脾肿大、发热和进行性恶病质、贫血、血小板减少和白细胞减少，与治疗无关。两名患者分别在出现这些症状后的4个月和3个月死亡。在第一例病例中，从尸检材料中经组织病理学诊断为里氏综合征。在肝脏、脾脏、肾上腺和骨髓中，除了慢性淋巴细胞白血病的特征性浸润（小淋巴细胞）外，还有与高恶性淋巴瘤一致的大细胞增殖区域。在另一例病例中，在因急性胆囊炎切除的胆囊以及肝门区淋巴结中发现了大细胞淋巴瘤浸润。对活检材料进行的免疫细胞化学研究表明，肿瘤细胞具有B淋巴细胞标志物和细胞质IgM κ，与慢性淋巴细胞白血病的淋巴细胞相同。对于出现快速临床恶化和伴有恶病质的全身症状的慢性淋巴细胞白血病患者，应考虑里氏综合征的可能性，并进行适当的形态学研究。
