Terstappen L W, Nguyen M, Huang S, Lazarus H M, Medof M E
Becton Dickinson Immunocytometry Systems, San Jose, CA 95131.
Br J Haematol. 1993 Jul;84(3):504-14. doi: 10.1111/j.1365-2141.1993.tb03108.x.
The expression of decay-accelerating factor (DAF or CD55) and CD59 during haematopoietic cell development in bone marrow aspirates of two patients with paroxysmal nocturnal haemoglobinuria (PNH) was compared with that in normal bone marrow by five-dimensional flow cytometry. In contrast to early uncommitted haematopoietic progenitor cells (CD34+, CD38-) in normal bone marrow which uniformly express DAF and CD59, the majority of CD34+, CD38- cells in both patients' marrow exhibited the absence of the two proteins. In both specimens, however, subpopulations of CD34+, CD38- cells expressing DAF and CD59 were detectable, indicative of the presence of two lines of haematopoiesis, one abnormal and the other normal. Concurrent abnormal and normal haematopoietic development was further evident by the presence of subpopulations of DAF-, CD59- and DAF+, CD59+ cells along the differentiation and maturation pathways of the myeloid (CD33+, CD15(-)-->CD33+-->++, CD15+), the erythroid (CD45dim, CD71dim-->CD45-, CD71++), and the B-lymphoid cell lineages (CD10++, CD20(-)-->CD10-, CD20++). While the majority of cells differentiating into and maturing along each cell lineage lacked DAF and CD59, the majority of mature B (CD20++, CD10-) and T-lymphocytes lymphocytes (CD3+) expressed both proteins suggestive of the presence of lymphocytes with a long life span which were generated from normal haematopoietic progenitors before the onset of the disease. The detection of distinct sets of CD34+, CD38(-)--> + progenitor cells which are DAF+, CD59+ or DAF-, CD59- in marrow of PNH patients has relevance for the treatment of PNH. Cells with the phenotype CD34+, CD38-, DAF+, CD59+ are capable of self renewal and represent potential candidates for autologous bone marrow transplantation following depletion of CD34+, CD38-, DAF-, CD59- cells.
通过五维流式细胞术，比较了两名阵发性夜间血红蛋白尿（PNH）患者骨髓穿刺物中造血细胞发育过程中衰变加速因子（DAF或CD55）和CD59的表达与正常骨髓中的表达情况。与正常骨髓中均匀表达DAF和CD59的早期未定向造血祖细胞（CD34 +，CD38 -）不同，两名患者骨髓中的大多数CD34 +，CD38 -细胞均未表达这两种蛋白。然而，在两个标本中，均可检测到表达DAF和CD59的CD34 +，CD38 -细胞亚群，这表明存在两条造血系，一条异常，另一条正常。在髓系（CD33 +，CD15（-）--> CD33 +--> ++，CD15 +）、红系（CD45dim，CD71dim--> CD45 -，CD71 ++）和B淋巴细胞系（CD10 ++，CD20（-）--> CD10 -，CD20 ++）的分化和成熟途径中，同时存在DAF -、CD59 -和DAF +、CD59 +细胞亚群，进一步证明了造血发育的异常与正常并存。虽然沿每个细胞系分化和成熟的大多数细胞缺乏DAF和CD59，但大多数成熟B（CD20 ++，CD10 -）和T淋巴细胞（CD3 +）均表达这两种蛋白，这表明存在寿命较长的淋巴细胞，它们是在疾病发作前由正常造血祖细胞产生的。在PNH患者骨髓中检测到不同组的CD34 +，CD38（-）--> +祖细胞，它们分别为DAF +，CD59 +或DAF -，CD59 -，这与PNH的治疗相关。具有CD34 +，CD38 -，DAF +，CD59 +表型的细胞能够自我更新，是CD34 +，CD38 -，DAF -，CD59 -细胞耗竭后自体骨髓移植的潜在候选细胞。
