Parker R I
Department of Pediatrics, State University of New York, Stony Brook School of Medicine, USA.
Hematol Oncol Clin North Am. 2000 Jun;14(3):557-68. doi: 10.1016/s0889-8588(05)70296-3.
Systemic mast cell disease is characterized by bone marrow involvement by mast cells and frequently by peripheral blood cytopenias. The coexistence of hematologic disorders, such as myeloproliferative or myelodysplastic syndromes, or of lymphoreticular malignancies with SMCD is common. Overt mast cell leukemia is rare. In general, patients who coexhibit a severe hematologic disorder tend to have a more compressed clinical course and a worse prognosis. Hemorrhage can be a result of heparin release from stimulated mast cells.
系统性肥大细胞疾病的特征是骨髓有肥大细胞浸润,且常伴有外周血细胞减少。血液系统疾病,如骨髓增殖性或骨髓增生异常综合征,或淋巴网状恶性肿瘤与系统性肥大细胞疾病共存很常见。明显的肥大细胞白血病很少见。一般来说,同时患有严重血液系统疾病的患者往往临床病程更紧凑,预后更差。出血可能是受刺激的肥大细胞释放肝素所致。