Secker-Walker L M, Craig J M
Department of Haematology, Royal Free Hospital and School of Medicine, London, UK.
Leukemia. 1993 Feb;7(2):147-51.
Philadelphia-positive (Ph) acute lymphoblastic leukemia (ALL) is heterogeneous both in terms of the BCR gene breakpoints (M-bcr and m-bcr) and in the number of cell lineages carrying the Ph chromosome. The impact of these observations on the controversy surrounding Ph ALL and Ph+ chronic granulocytic leukemia (CGL) is unclear. Twenty cases of Ph ALL (four newly investigated and 16 previously published) were classified into nine stem-cell (the Ph in myeloid and lymphoid cells) and 11 lymphoid-restricted cases. Lymphoid cases had a lower leucocyte count (56 x 10(9)/l) than stem-cell cases (151 x 10(9)/l) (p < 0.01). The M-bcr and m-bcr breakpoints (in 14 cases) were found in lymphoid (four cases of each) and stem-cell (five and one cases respectively) cases. Lymphoid cases had significantly shorter event-free survival (median 4 months) than stem-cell cases (median 35 months) (p < 0.01). M-bcr cases were older (mean 41 years) than m-bcr cases (mean 36 years)(p = NS); m-bcr cases had higher leukocyte counts (mean 85 x 10(9)/l) than M-bcr cases (36 x 10(9)/l)(p < 0.01) but breakpoint had no impact on prognosis. Lineage involvement, but not breakpoint, appears to distinguish prognostically important sub-groups of Ph ALL. Paradoxically, lymphoid-restricted cases have a worse prognosis than cases arising in a pluripotent stem-cell.
费城染色体阳性（Ph）急性淋巴细胞白血病（ALL）在BCR基因断点（M-bcr和m-bcr）以及携带Ph染色体的细胞系数量方面均具有异质性。这些观察结果对围绕Ph ALL和Ph+慢性粒细胞白血病（CGL）的争议的影响尚不清楚。20例Ph ALL（4例为新研究病例，16例为先前发表病例）被分为9例干细胞型（髓系和淋巴细胞中均有Ph染色体）和11例淋巴细胞受限型病例。淋巴细胞型病例的白细胞计数（56×10⁹/L）低于干细胞型病例（151×10⁹/L）（p<0.01）。在淋巴细胞型（各4例）和干细胞型（分别为5例和1例）病例中发现了M-bcr和m-bcr断点（共14例）。淋巴细胞型病例的无事件生存期（中位数4个月）明显短于干细胞型病例（中位数35个月）（p<0.01）。M-bcr病例的年龄（平均41岁）大于m-bcr病例（平均36岁）（p=无显著性差异）；m-bcr病例的白细胞计数（平均85×10⁹/L）高于M-bcr病例（36×10⁹/L）（p<0.01），但断点对预后无影响。谱系受累而非断点似乎可区分Ph ALL预后重要的亚组。矛盾的是，淋巴细胞受限型病例的预后比多能干细胞来源的病例更差。
