Travis W D, Li C Y, Hoagland H C, Travis L B, Banks P M
Mayo Clin Proc. 1986 Dec;61(12):957-66. doi: 10.1016/s0025-6196(12)62636-6.
We report the clinical and pathologic findings in one case of mast cell leukemia observed in a series of 60 patients with systemic mast cell disease. The leukemic variant of systemic mast cell disease is rapidly fatal (mean duration of survival, less than 6 months) in contrast to most nonleukemic cases, which follow an indolent clinical course. On the basis of our case and eight previously reported cases, mast cell leukemia is characterized by a substantial increase in atypical mast cells in the peripheral blood, diffuse infiltration with atypical mast cells in the bone marrow, a strong association with peptic ulcer disease, prominent constitutional symptoms, and hepatosplenomegaly. These cases should be distinguished from malignant mastocytosis without a substantial number of circulating atypical mast cells and also cases of acute nonlymphocytic leukemia that arise in the background of systemic mast cell disease.
我们报告了在60例系统性肥大细胞病患者中观察到的1例肥大细胞白血病的临床和病理结果。与大多数非白血病病例的惰性临床病程不同，系统性肥大细胞病的白血病变体迅速致命（平均生存期少于6个月）。根据我们的病例及之前报道的8例病例，肥大细胞白血病的特征为外周血中非典型肥大细胞大量增加、骨髓中出现非典型肥大细胞弥漫浸润、与消化性溃疡病密切相关、明显的全身症状以及肝脾肿大。这些病例应与不存在大量循环非典型肥大细胞的恶性肥大细胞增多症以及发生在系统性肥大细胞病背景下的急性非淋巴细胞白血病相鉴别。
