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Histiocytes and histiocytosis [see comments]
MJ Cline
Department of Medicine, University of California Los Angeles.
The term histiocyte refers to cells of either the macrophage or Langerhans
cell lineages. The histiocytic disorders are characterized by the
proliferation of cells of these lineages. With recent advances in knowledge
of the developmental biology of histiocytic cells, it is now possible to
formulate a reasonable catalogue of histiocytic diseases based on
ultra-structural and phenotypic markers of cellular origins and molecular
or chromosomal markers of malignancy. The catalogue includes the following
groups of diseases. Nonmalignant reactive macrophage disorders include (1)
macrophage storage diseases, (2) several benign proliferative macrophage
disorders that predominantly involve skin and bone, and (3) several
hemophagocytic syndromes that vary from indolent and benign to fulminant
and fatal. In some of the latter disorders, viruses have been identified as
the inciting stimulus. The malignant macrophage disorders include (1) acute
monocytic leukemia and (2) chronic myelomonocytic leukemia. A rare disorder
that gave rise to a permanent cell line with an anomaly of chromosomal
segment 5q35 may also be an example of a histiocytic malignancy. The
existence of a separate category of true histiocytic lymphoma of macrophage
type is uncertain. Reactive Langerhans cell disorders include (1)
congenital self-healing histiocytosis, (2) the many variants of
eosinophilic granuloma, and (3) a related disorder designated as relapsing
Langerhans cell histiocytosis that is characterized by a relapsing course
and infiltration of bone and soft tissues by Langerhans cells.
Presumptively neoplastic diseases of Langerhans and dendritic cells include
(1) progressive Langerhans cell histiocytosis, a disease with prominent
involvement of blood and BM as well as skin and viscera; (2) Langerhans
cell lymphoma, and (3) dendritic cell lymphoma. However, clonality as a
marker of malignancy has not been proven in these disorders.
Volume 84,
Issue 9,
pp. 2840-2853,
11/01/1994
Copyright © 1994 by The American Society of Hematology
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