Horny H P, Sillaber C, Menke D, Kaiserling E, Wehrmann M, Stehberger B, Chott A, Lechner K, Lennert K, Valent P
Institute of Pathology, University of Tübingen, Germany.
Am J Surg Pathol. 1998 Sep;22(9):1132-40. doi: 10.1097/00000478-199809000-00013.
The term "mastocytosis" is used to describe a heterogeneous group of disorders characterized by abnormal growth and accumulation of mast cells (MCs). Cutaneous and systemic variants exist. Systemic mastocytosis may show an indolent or malignant clinical course. In malignant mastocytosis (MM), the diagnosis often is missed because the MCs are morphologically abnormal and lack metachromatic granules or the underlying histologic picture is complex. The cytoplasmic serine protease tryptase is produced by MCs and is thought to be expressed at all stages of MC maturation. To assess the diagnostic value of tryptase staining in mastocytosis, tissue sections from 93 patients with mastocytosis, including MM (n = 37), systemic indolent mastocytosis (n = 47), urticaria pigmentosa (n = 5), MC leukemia (n = 2), and solitary skin mastocytoma (n = 2) were stained with the antitryptase antibody G3. The results were compared with those of Giemsa and chloroacetate esterase (CAE) staining. Using antitryptase antibody G3, MC infiltrates were identified in all patients examined, including those with MM (37 of 37), and virtually all the neoplastic MCs (> 95%) appeared to react with G3. In MM, significantly fewer MCs were positive in Giemsa (54.5%; p < 0.05) and CAE (78.8%; p < 0.05). Moreover, G3 produced clear diagnostic staining in all cases of MM, but the proportion of cases with clear diagnostic results (> 10% of neoplastic cells positive) was considerably lower with Giemsa (48.6%; p < 0.05) and CAE (75.7%; p < 0.05) staining. By contrast, tryptase, Giemsa, and CAE produced diagnostic staining of MCs in virtually all cases of systemic indolent mastocytosis, urticaria pigmentosa, and solitary skin mastocytoma. In systemic mastocytosis, survival was significantly reduced in cases with Giemsa-/tryptase+ or CAE-/tryptase+ tumor cells compared to those cases with Giemsa+ or CAE+ MC infiltrates (p < 0.001).
“肥大细胞增多症”一词用于描述一组异质性疾病，其特征为肥大细胞（MC）异常生长和积聚。存在皮肤型和系统性型。系统性肥大细胞增多症可能表现为惰性或恶性临床病程。在恶性肥大细胞增多症（MM）中，由于MC形态异常且缺乏异染颗粒或潜在组织学表现复杂，诊断常常被漏诊。细胞质丝氨酸蛋白酶类胰蛋白酶由MC产生，被认为在MC成熟的所有阶段均有表达。为评估类胰蛋白酶染色在肥大细胞增多症中的诊断价值，对93例肥大细胞增多症患者的组织切片进行抗类胰蛋白酶抗体G3染色，这些患者包括MM（n = 37）、系统性惰性肥大细胞增多症（n = 47）、色素性荨麻疹（n = 5）、MC白血病（n = 2）和孤立性皮肤肥大细胞瘤（n = 2）。将结果与吉姆萨染色和氯乙酸酯酶（CAE）染色结果进行比较。使用抗类胰蛋白酶抗体G3，在所有检查的患者中均识别出MC浸润，包括MM患者（37例中的37例），并且几乎所有肿瘤性MC（> 95%）似乎都与G3发生反应。在MM中，吉姆萨染色（54.5%；p < 0.05）和CAE染色（78.8%；p < 0.05）显示阳性的MC明显较少。此外，G3在所有MM病例中均产生清晰的诊断性染色，但吉姆萨染色（48.6%；p < 0.05）和CAE染色（75.7%；p < 0.05）显示清晰诊断结果（> 10%的肿瘤细胞阳性）的病例比例要低得多。相比之下，类胰蛋白酶、吉姆萨和CAE在几乎所有系统性惰性肥大细胞增多症、色素性荨麻疹和孤立性皮肤肥大细胞瘤病例中均产生MC的诊断性染色。在系统性肥大细胞增多症中，与吉姆萨阳性或CAE阳性的MC浸润病例相比，吉姆萨阴性/类胰蛋白酶阳性或CAE阴性/类胰蛋白酶阳性肿瘤细胞的病例生存率显著降低（p < 0.001）。
