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British Medical Bulletin 52:818-825 (1996)
© 1996 The British Council

research-article

Histiocyte disorders

D K H Webb

Department of child Health, Llandough Hospital & Community NHS Trust Cardiff, UK

D K H Webb, Department of Haemafology and Oncology, Great Ormond Street Children's Hospital, London WC1N 3JH, UK.

Abstract

Histiocyte disorders are characterised by tissue infiltration with cells of monocyte/macrophage lineage, with two disorders, Langerhans' cell histicytosis (LCH) and haemophagocytic lymphohistiocytosis (HLH) accounting for the overwhelming majority of cases in childhood and, apart from monocytic variants of acute myeloid leukaemia, histiocytic malignancy is very rare. Although both LCH and HLH are considered reactive disorders, the prognosis of these conditions differs greatly. LCH is usually self limiting, with a mortality of 10%, but HLH is usually fatal, with a mortality of over 80% in the absence of bone marrow transplatation. Increased levels of cytokines have been demostrated in these disorders, and may be responsible for many of the clinical features; it is unclear whether histiocyted themselves, or other immune cells, particularly Tlymphocytes, are the abnormal cell population. Due to the rarity of histiocyte disorders, collaborative studies are essential to improve understanding and advance treatment.


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