Kettelhut B V, Parker R I, Travis W D, Metcalfe D D
Mast Cell Physiology Section, National Institute of Allergy and Infectious Diseases, Bethesda, Maryland 20892.
Am J Clin Pathol. 1989 May;91(5):558-62. doi: 10.1093/ajcp/91.5.558.
Sixteen bone marrow aspirates and 15 trephine core biopsies from 17 children with cutaneous mastocytosis, of which 15 exhibited urticaria pigmentosa and 2 exhibited diffuse cutaneous mastocytosis, were evaluated for the presence of bone marrow-associated pathologic conditions. Eight bone marrow aspirates from 8 children and 23 trephine core bone marrow biopsies from 16 children who had had evaluation for hematologic abnormalities not associated with cutaneous mastocytosis served as a control population. Eosinophilia was a prominent finding in bone marrows of 9 of 17 patients who had cutaneous mastocytosis. Increased mast cell numbers in bone marrow aspirates were observed in 5 children with cutaneous mastocytosis (5 of 16) and in 2 of the control children (2 of 8). Examination of the trephine core bone marrow biopsies obtained from patients with cutaneous mastocytosis demonstrated focal perivascular and paratrabecular aggregates consisting of mast cells, eosinophils, and early myeloid cells in 10 of 15 individuals. Similar lesions were observed in trephine core bone marrow biopsies of 3 of 16 control patients. The focal mast cell lesions characteristic of adult systemic mastocytosis were not observed. The authors conclude that cutaneous mastocytosis in the pediatric age group is rarely associated with definitive bone marrow findings suggestive of systemic mast cell disease and that this observation is consistent with previous reports that cutaneous mastocytosis in the majority of pediatric cases resolves by adulthood.
对17例皮肤肥大细胞增多症患儿的16份骨髓穿刺物和15份骨髓活检组织进行评估，以确定是否存在与骨髓相关的病理状况。其中15例表现为色素性荨麻疹，2例表现为弥漫性皮肤肥大细胞增多症。另外，选取8例因血液学异常接受评估但与皮肤肥大细胞增多症无关的患儿的8份骨髓穿刺物，以及16例患儿的23份骨髓活检组织作为对照。嗜酸性粒细胞增多是17例皮肤肥大细胞增多症患儿中9例骨髓的显著特征。16例皮肤肥大细胞增多症患儿中有5例骨髓穿刺物中的肥大细胞数量增加，8例对照患儿中有2例出现同样情况。对皮肤肥大细胞增多症患儿的骨髓活检组织检查显示，15例中有10例出现由肥大细胞、嗜酸性粒细胞和早期髓系细胞组成的局灶性血管周围和小梁旁聚集。16例对照患者中有3例的骨髓活检组织也观察到类似病变。未观察到成人系统性肥大细胞增多症特有的局灶性肥大细胞病变。作者得出结论，儿童期皮肤肥大细胞增多症很少与提示系统性肥大细胞疾病的明确骨髓表现相关，这一观察结果与之前的报道一致，即大多数儿童病例的皮肤肥大细胞增多症在成年期会消退。
