Cervantes F, Villamor N, Esteve J, Montoto S, Rives S, Rozman C, Montserrat E
Department of Medicine, Hospital Clínic, University of Barcelona, Spain.
Br J Haematol. 1998 Jan;100(1):123-8. doi: 10.1046/j.1365-2141.1998.00542.x.
It has been suggested that in blast crisis (BC) of chronic myeloid leukaemia (CML) the clinical and laboratory features of patients with 'lymphoid' phenotype differ from those of patients with non-lymphoid BC. In order to assess any differences, 97 patients consecutively diagnosed with BC that followed a known chronic phase of CML were analysed. 19 patients had 'lymphoid' BC: in 17 the blasts expressed a B-lineage phenotype: in the remaining two they corresponded to T lymphoblasts. Four cases of B-lineage phenotype BC were considered as biphenotypic, due to the co-expression of myeloperoxidase and one or two other myeloid markers (CD33, CD13 and CD68) on the blast cells; in the other six cases of B-lineage BC the blasts expressed one or both of the myeloid markers CD33 (n = 4) and CD13 (n = 3). Patients with 'lymphoid' BC seldom had an accelerated phase prior to BC (1/19 v 36/78 with non-lymphoid BC, P = 0.002), had less frequent splenomegaly (9/19 v 59/78, P = 0.03) and hepatomegaly (5/19 v 45/78, P = 0.02) and showed a higher degree of marrow blast infiltration (mean value 74 +/- 24% v 38 +/- 23%, P < 0.0001), lesser blood basophilia (2.2 +/- 2.5% v 8.2 +/- 7.8%, P < 0.0001), and higher serum albumin levels (P = 0.001) than those with non-lymphoid BC. 13 patients with 'lymphoid' BC (68.4%) showed a favourable response to chemotherapy regimens including vincristine and prednisone and, overall, 'lymphoid' BC patients survived significantly longer than the remainder (median survival 12 months v 4.7 months, P = 0.006). These results indicate that 'lymphoid' BC of CML has a distinct clinicohaematological profile and confirm the better prognosis of such patients.
有人提出，在慢性髓性白血病（CML）的急变期（BC），具有“淋巴样”表型的患者的临床和实验室特征与非淋巴样BC患者不同。为了评估差异，对97例连续诊断为BC且之前有已知CML慢性期的患者进行了分析。19例患者为“淋巴样”BC：17例中原始细胞表达B系表型；其余2例为T淋巴母细胞。4例B系表型BC被认为是双表型，因为原始细胞同时表达髓过氧化物酶和一种或两种其他髓系标志物（CD33、CD13和CD68）；在其他6例B系BC中，原始细胞表达髓系标志物CD33（n = 4）和CD13（n = 3）中的一种或两种。“淋巴样”BC患者在BC之前很少有加速期（1/19对比非淋巴样BC的36/78，P = 0.002），脾肿大（9/19对比59/78，P = 0.03）和肝肿大（5/19对比45/78，P = 0.02）的发生率较低，骨髓原始细胞浸润程度较高（平均值74±24%对比38±23%，P < 0.0001），血嗜碱性粒细胞较少（2.2±2.5%对比8.2±7.8%，P < 0.0001），血清白蛋白水平较高（P = 0.001），均优于非淋巴样BC患者。13例“淋巴样”BC患者（68.4%）对包括长春新碱和泼尼松的化疗方案显示出良好反应，总体而言，“淋巴样”BC患者的生存期明显长于其余患者（中位生存期12个月对比4.7个月，P = 0.006）。这些结果表明，CML的“淋巴样”BC具有独特的临床血液学特征，并证实了此类患者预后较好。
