Ventura Filipa, Pereira Teresa, da Luz Duarte Maria, Marques Herlander, Pardal Fernando, Brito Celeste
Dermatology and Venereology Department, Braga Hospital, Apartado 2242, 4701-965 Braga, Portugal.
Dermatol Res Pract. 2010;2010:569345. doi: 10.1155/2010/569345. Epub 2010 Jun 21.
Indeterminate cell histiocytosis (ICH) is a rare proliferative disorder, in which the predominant cells share morphologic and immunophenotypic features from both Langerhans and non-Langerhans cell histiocytosis. We describe a 62-year-old man presenting a 2-month history of firm nodular lesions on the upper lip. Histopathology, immunohistochemical, and ultrastructural analysis showed typical findings of ICH. The patient was treated with thalidomide and almost complete regression of the lesions was reached within 7 months. Nevertheless, one month after remission, he developed an acute myeloid leukemia of the subtype monocytic leukemia (M5). The patient's condition rapidly worsened and he died due to a respiratory failure four weeks later. We present this case because apart of being rare it joins the effectiveness of thalidomide and the association with an acute monocytic leukemia. A review of the literature is made.
未定型细胞组织细胞增多症（ICH）是一种罕见的增殖性疾病，其中主要细胞具有朗格汉斯细胞组织细胞增多症和非朗格汉斯细胞组织细胞增多症的形态学和免疫表型特征。我们描述了一名62岁男性，其在上唇出现了2个月的坚实结节性病变病史。组织病理学、免疫组织化学和超微结构分析显示了ICH的典型表现。该患者接受了沙利度胺治疗，7个月内病变几乎完全消退。然而，缓解1个月后，他发展为单核细胞白血病（M5）亚型的急性髓系白血病。患者病情迅速恶化，四周后因呼吸衰竭死亡。我们呈现此病例是因为它除了罕见之外，还体现了沙利度胺的有效性以及与急性单核细胞白血病的关联。并对文献进行了综述。
