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当组织细胞病X(HX)累及单一器官系统,预后极好,而在多个系统受侵时则预后不良。据报告,2岁以下的儿童累及肺、肝及骨髓(Letterer-Siwe病)时,死亡率为50～70％。因怀疑HX不是一种真性恶性疾患,和用多种化疗似无益,因而近来趋向于应用包括胸腺素这类较为温和的治疗。本文报告2例多系统被HX受侵婴儿的自发性缓解。例1在出生后24小时内于颊部便发生皮疹,以后渐扩大范围,此儿受侵部位有皮肤、肺与齿龈;例
When histiocytosis X (HX) involves a single organ system, the prognosis is excellent, whereas in the case of multiple systems, the prognosis is poor. It has been reported that the mortality rate of children under 2 years of age involving lung, liver and bone marrow (Letterer-Siwe disease) is 50 to 70%. Because of the suspicion that HX is not a true malignancy and is not beneficial with multiple chemotherapies, there has recently been a trend towards the use of milder treatments, including thymosin. This article reports the spontaneous remission of 2 cases of multi-system HX infants. Example 1 A rash occurs in the buccal area within 24 hours of birth and then expands to a wider area where there is skin, lungs and gums at the site of invasion