Baghestanian M, Bankl H c, Sillaber C, Beil W J, Radaszkiewicz T, Füreder W, Preiser J, Vesely M, Schernthaner G, Lechner K, Valent P
Department of Internal Medicine I, University of Vienna, Austria.
Leukemia. 1996 Jan;10(1):159-66.
The phenotypic and biologic properties of malignant cells in a case of aggressive mastocytosis with multi-organ involvement, circulating mast cell precursors and absence of skin infiltrates were analyzed. Circulating mast cell precursors were detected by immunostaining using antibodies against mast cell tryptase as well as by electron microscopy. These progenitors were tryptase+/chymase- (MCT) and accounted for 10 to 20% of nucleated mononuclear blood cells (MNC). A subset of them contained metachromatic granules. As assessed by combined toluidine blue/immunofluorescence staining, the granulated mast cell precursors were found to express CD9 (P24), CD33 (gp67) and CD44 (Pgp-1), but not basophil-related markers (CD11b (C3biR), CDw17 (lactosylceramide), CD123 (il-3R alpha))or monocyte-related antigens (CD14, CD15). Expression of the mast cell growth factor (MGF) receptor, c-kit(CD117), was also demonstrable, whereas the skin mast cell marker C5aR (CD88) could not be detected on mast cell precursors. The ligand of c-kit, recombinant human (rh) stem cell factor (SCF = MGF), induced histamine release from circulating mast cell progenitors, whereas rhC5a, a potent skin mast cell-/basophil-agonist, was ineffective over the dose-range (10(-9) to 10(-7(M)) tested. Analysis of mast cell antigens in malignant mastocytosis or mast cell leukemias may be helpful to establish a diagnosis and to determine the phenotype of the clone.
对一例侵袭性肥大细胞增多症患者的恶性细胞的表型和生物学特性进行了分析，该患者多器官受累，有循环中的肥大细胞前体且无皮肤浸润。通过使用抗肥大细胞类胰蛋白酶抗体的免疫染色以及电子显微镜检测循环中的肥大细胞前体。这些祖细胞为类胰蛋白酶阳性/糜蛋白酶阴性（MCT），占核单核血细胞（MNC）的10%至20%。其中一部分含有异染性颗粒。通过甲苯胺蓝/免疫荧光联合染色评估，发现有颗粒的肥大细胞前体表达CD9（P24）、CD33（gp67）和CD44（Pgp-1），但不表达嗜碱性粒细胞相关标志物（CD11b（C3biR）、CDw17（乳糖神经酰胺）、CD123（IL-3Rα））或单核细胞相关抗原（CD14、CD15）。肥大细胞生长因子（MGF）受体c-kit（CD117）的表达也可检测到，而在肥大细胞前体上未检测到皮肤肥大细胞标志物C5aR（CD88）。c-kit的配体重组人（rh）干细胞因子（SCF = MGF）可诱导循环中的肥大细胞祖细胞释放组胺，而rhC5a，一种强效的皮肤肥大细胞/嗜碱性粒细胞激动剂，在测试的剂量范围（10^(-9)至10^(-7)M）内无效。分析恶性肥大细胞增多症或肥大细胞白血病中的肥大细胞抗原可能有助于建立诊断并确定克隆的表型。
