Department of Dermatology, Hospital Nacional de Pediatría "Prof. Dr. Juan P. Garrahan", Buenos Aires, Argentina.
Department of Hemato-Oncology, Hospital Nacional de Pediatría "Prof. Dr. Juan P. Garrahan", Buenos Aires, Argentina.
Pediatr Dermatol. 2022 Jan;39(1):115-118. doi: 10.1111/pde.14887. Epub 2021 Dec 21.
Progressive nodular histiocytosis is a rare variant of non-Langerhans cell histiocytosis that affects the skin and mucous membranes and displays a progressive clinical course and poor response to treatment. We describe a case of severe progressive nodular histiocytosis harboring a KRAS p.G12S mutation in a 9-year-old boy, refractory to chemotherapy, who was successfully treated with the MEK inhibitor cobimetinib. This is the first report of the use of MEK inhibition for this histiocytosis subtype in a pediatric patient.
进行性结节性组织细胞增生症是一种罕见的非朗格汉斯细胞组织细胞增生症变异型，影响皮肤和黏膜，表现为进行性临床病程和对治疗反应不佳。我们描述了一例 9 岁男孩患有严重进行性结节性组织细胞增生症，该患者携带有 KRAS p.G12S 突变，对化疗耐药，使用 MEK 抑制剂 cobimetinib 成功治疗。这是首例报道在儿科患者中使用 MEK 抑制治疗这种组织细胞增生症亚型的病例。
