[朗格汉斯细胞组织细胞增多症与骨髓单核细胞白血病：一种非偶然的关联]
[Langerhans cell histiocytosis and myelomonocytic leukemia: a non-fortuitous association].
作者信息
Baikian B, Descamps V, Grossin M, Marinho E, Picard C, Aitken G, Sigal M, Crickx B, Belaich S
机构信息
Service de Dermatologie, Hôpital Bichat-Claude Bernard, Paris.
出版信息
Ann Dermatol Venereol. 1999 May;126(5):409-11.
BACKGROUND
Langerhans cell histiocytosis is an uncommon clonal disorder. Its reactional or genetic nature is debated.
CASE REPORT
Three patients aged 71, 75 and 73 years with Langerhans cell histiocytosis also developed myelomoncytic leukemia (AML4, AML5, and chronic myelomonocytic leukemia). In two cases, there was no prior treatment which could potentially induce leukemia. In the third case, AML4 occurred shortly (10 months) after initiation of a vinblastin treatment.
DISCUSSION
This pathogenic association suggests a common origin for these two conditions, corresponding to an anomalous pluripotent stem cell line. These cases provide further evidence favoring the hypothesis of a genetic origin rather than a reactional origin for Langerhans cell histiocytosis.
背景
朗格汉斯细胞组织细胞增多症是一种罕见的克隆性疾病。其反应性或遗传性本质存在争议。
病例报告
三名年龄分别为71岁、75岁和73岁的朗格汉斯细胞组织细胞增多症患者同时发生了骨髓单核细胞白血病（AML4、AML5和慢性骨髓单核细胞白血病）。在两例中，之前没有可能诱发白血病的治疗。在第三例中，AML4在长春花碱治疗开始后不久（10个月）发生。
讨论
这种致病关联提示这两种疾病有共同起源，对应于异常的多能干细胞系。这些病例为支持朗格汉斯细胞组织细胞增多症的遗传起源而非反应性起源假说提供了进一步证据。
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