HISTIOCYTOSIS-X - NATURAL-HISTORY AND MANAGEMENT IN CHILDHOOD

被引：26

作者：

PRITCHARD, J

机构：

[1] Department of Haematology and Oncology, Hospital for Sick Children, London, WCIN 3JH, Great Ormond Street

来源：

CLINICAL AND EXPERIMENTAL DERMATOLOGY | 1979年 / 4卷 / 04期

关键词：

D O I：

10.1111/j.1365-2230.1979.tb01637.x

中图分类号：

R75 [皮肤病学与性病学];

学科分类号：

100206 ;

摘要：

Multi‐system histiocytosis X is still a potentially fatal disorder. Death occurs from pulmonary, hepatic, small bowel or bone marrow failure often with severe intercurrent infection as the terminal illness. Chemotherapy has had some impact on mortality but morbidity in survivors is still a real problem. Around 50% of survivors will have a significant handicap. Complete response can be achieved with a variety of chemotherapeutic agents but the introduction of combination therapy has led to unacceptable toxicity especially in the very young. There is preliminary evidence that treatment with calf ‘thymic humoral factor’ may benefit a proportion of patients. This recent work supports the notion that histiocytosis X is not a true malignancy, but rather a subtle form of immune deficiency possibly involving a soluble thymic or T‐lym‐phocyte product. Copyright © 1979, Wiley Blackwell. All rights reserved

引用

页码：421 / 433

页数：13

共 16 条

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[10]

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