Emanuel P D, Barton J C, Gualtieri R J, Sams W M
Department of Medicine, University of Alabama, Birmingham.
J Am Acad Dermatol. 1991 May;24(5 Pt 2):893-7. doi: 10.1016/0190-9622(91)70142-o.
A 64-year-old man had urticaria pigmentosa and myelodysplasia (refractory anemia with excess blast cells; partial trisomy 8 syndrome) without increased numbers of marrow mast cells. Clonal marrow assays in agar demonstrated normal to increased colony-forming units of granulocytes/macrophages. In long-term liquid cultures containing mast cell growth factor (interleukin 3), his marrow cells proliferated after 3 weeks to produce abnormal myeloid precursors similar to those in the corresponding marrow aspirate specimen. Cells with basophilic-staining granules were less abundant in comparison with normal marrow specimens cultured similarly. These results suggest that the mast cells in this patient are not of the same clone as the preleukemic marrow cells, although the possible marrow-cell origin of urticaria pigmentosa mast cells cannot be excluded. Previous reports suggest that urticaria pigmentosa without systemic mastocytosis occurs as a nonspecific abnormality in a variety of myeloid, lymphoid, and nonhematologic malignancies. Our data also support this hypothesis that urticaria pigmentosa is a reactive process rather than a manifestation of clonal proliferation of the primary malignancy.
一名64岁男性患有色素性荨麻疹和骨髓发育异常（伴原始细胞增多的难治性贫血；8号染色体部分三体综合征），骨髓肥大细胞数量未增加。琼脂中的克隆骨髓检测显示粒细胞/巨噬细胞集落形成单位正常或增加。在含有肥大细胞生长因子（白细胞介素3）的长期液体培养中，他的骨髓细胞在3周后增殖，产生了与相应骨髓抽吸标本中相似的异常髓系前体细胞。与同样培养的正常骨髓标本相比，具有嗜碱性染色颗粒的细胞较少。这些结果表明，该患者的肥大细胞与白血病前期骨髓细胞不是同一克隆，尽管不能排除色素性荨麻疹肥大细胞可能的骨髓细胞起源。先前的报告表明，无系统性肥大细胞增多症的色素性荨麻疹是多种髓系、淋巴系和非血液系统恶性肿瘤中的一种非特异性异常。我们的数据也支持这一假设，即色素性荨麻疹是一种反应性过程，而非原发性恶性肿瘤克隆增殖的表现。
