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Histiocytosis X: natural history and management in childhood
Author(s) -
PRITCHARD JON
Publication year - 1979
Publication title -
clinical and experimental dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.587
H-Index - 78
eISSN - 1365-2230
pISSN - 0307-6938
DOI - 10.1111/j.1365-2230.1979.tb01637.x
Subject(s) - medicine , histiocytosis , malignancy , natural history , chemotherapy , bone marrow , intensive care medicine , pediatrics , immunology , disease , surgery
Summary 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.