Torrey E, Simpson K, Wilbur S, Munoz P, Skikne B
Division of Hematology, Kansas University Medical Center, Kansas City 66103.
Am J Hematol. 1990 Aug;34(4):283-6. doi: 10.1002/ajh.2830340409.
A case of malignant mastocytosis with peripheral blood involvement is presented. The course of the patient's illness was complicated by recurrent hypotensive episodes, presumed to have been caused by mast cell degranulation. Treatment with hydroxyurea was associated with persistent hypotension which resulted in death. It has been proposed that the diagnosis of mast cell leukemia be given to patients presenting with greater than 10% atypical mast cells in the blood. However, review of 16 published cases of malignant mastocytosis with circulating mast cells reveals that the clinical manifestations, complications, and survival do not vary significantly with the percentage of peripheral blood mast cells. Patients with malignant mastocytosis with significant involvement by atypical mast cells in the bone marrow and peripheral blood should be considered as having an aggressive disease, regardless of the percentage of circulating mast cells.
本文报告一例伴有外周血受累的恶性肥大细胞增多症病例。患者病程因反复出现低血压发作而复杂化，推测是由肥大细胞脱颗粒引起的。羟基脲治疗导致持续低血压，最终患者死亡。有人提出，血液中异常肥大细胞大于10%的患者应诊断为肥大细胞白血病。然而，回顾16例已发表的伴有循环肥大细胞的恶性肥大细胞增多症病例发现，临床表现、并发症和生存率与外周血肥大细胞百分比并无显著差异。骨髓和外周血中存在大量异常肥大细胞的恶性肥大细胞增多症患者，无论循环肥大细胞的百分比如何，均应被视为患有侵袭性疾病。
