Greenberger Shoshana, Landov Hagai, Confino Yitzhak, Vaknine Hananya, Avivi Camila, Baum Sharon, Barzilai Aviv
Department of Dermatology, The Chaim Sheba Medical Center, Ramat Gan, Israel.
Sackler Faculty of Medicine, Tel-Aviv University, Tel Aviv, Israel.
Pediatr Dermatol. 2019 Jul;36(4):477-481. doi: 10.1111/pde.13850. Epub 2019 May 23.
Pediatric mastocytosis differs from adult mastocytosis in its presentation and clinical course. However, the data regarding the immunophenotypic characterization of mast cells in children are limited. Our objective was to evaluate the immunophenotype of mast cells in pediatric mastocytosis and correlate it with the clinical course.
Biopsy specimens of children with cutaneous mastocytosis were retrieved from the institutions of pathology and were stained for CD25, CD2, and CD30. The percentage of mast cells and the staining intensity were correlated with the clinical data.
Twenty-five biopsy specimens were included in the study. Patients' average age was 15.4 at presentation and 37.5 months at biopsy performance. Clinical presentations included maculopapular cutaneous mastocytosis in 79% and mastocytoma in 21% of cases. CD25, CD2, and CD30 were positive in 60%, 44%, and 84% of the biopsy specimens, respectively. The staining score was significantly higher for CD30 as compared to those for CD25 and CD2 (P = 0.02). No correlation was found between the immunophenotype and the clinical form or course of disease.
Our results confirm that CD30 is a sensitive marker for pediatric-onset mastocytosis. Nevertheless, its expression does not correlate with clinical subtype or clinical course. The sensitivity of CD25 is higher than that of CD2 in skin lesions.
儿童肥大细胞增多症在临床表现和临床病程方面与成人肥大细胞增多症有所不同。然而,关于儿童肥大细胞免疫表型特征的数据有限。我们的目的是评估儿童肥大细胞增多症中肥大细胞的免疫表型,并将其与临床病程相关联。
从病理机构获取皮肤肥大细胞增多症患儿的活检标本,进行CD25、CD2和CD30染色。肥大细胞百分比和染色强度与临床数据相关联。
本研究纳入25份活检标本。患者就诊时平均年龄为15.4岁,活检时平均年龄为37.5个月。临床表现包括79%的病例为斑丘疹性皮肤肥大细胞增多症,21%的病例为肥大细胞瘤。活检标本中CD25、CD2和CD30阳性率分别为60%、44%和84%。与CD25和CD2相比,CD30的染色评分显著更高(P = 0.02)。未发现免疫表型与临床类型或疾病病程之间存在相关性。
我们的结果证实CD30是儿童期肥大细胞增多症的敏感标志物。然而,其表达与临床亚型或临床病程无关。在皮肤病变中,CD25的敏感性高于CD2。