Stone R M
Department of Medicine, Dana-Farber Cancer Institute, Boston, Massachusetts.
Hematol Oncol Clin North Am. 1990 Apr;4(2):457-71.
PLL is an unusual clinical and morphologic variant of CLL which, in the more common B cell version, represents malignant transformation of a B lymphocyte at an intermediate stage of development. The immunophenotype of PLL cells, characterized by heavy cell surface staining for IgM and/or IgD and loss of mouse red blood cell receptors, suggests derivation from a slightly more mature cell than the one that gives rise to typical CLL. In patients with PLL prolymphocytic invasion accounts for massive splenomegaly and white counts of well over 100,000 per mm3 with minimal lymphadenopathy. Prolymphocytes are large cells with relatively open chromatin and prominent nucleoli. Extra material on the long arm of chromosome 14 is the most common cytogenetic abnormality. The clinical course of patients with PLL is aggressive, with median survivals usually of all stages. Combination chemotherapy regimens typically reserved for those with an unfavorable prognosis for non-Hodgkin's lymphomas probably are more effective in the treatment of patients with PLL than are the less myelosuppressive oral regimens used in CLL.
幼淋巴细胞白血病（PLL）是慢性淋巴细胞白血病（CLL）一种不常见的临床和形态学变异型，在更常见的B细胞型中，它代表B淋巴细胞在发育中间阶段的恶性转化。PLL细胞的免疫表型特征为细胞表面IgM和/或IgD重度染色以及小鼠红细胞受体缺失，提示其来源于比产生典型CLL的细胞稍成熟的细胞。在PLL患者中，幼淋巴细胞浸润导致脾肿大明显，白细胞计数超过每立方毫米100,000，淋巴结病轻微。幼淋巴细胞是大细胞，染色质相对疏松，核仁突出。14号染色体长臂上的额外物质是最常见的细胞遗传学异常。PLL患者的临床病程具有侵袭性，各阶段的中位生存期通常较短。通常用于预后不良的非霍奇金淋巴瘤患者的联合化疗方案，可能比CLL中使用的骨髓抑制作用较小的口服方案在治疗PLL患者方面更有效。
