J. Gogusev et C. Nezelof, MALIGNANT HISTIOCYTOSIS - HISTOLOGIC, CYTOCHEMICAL, CHROMOSOMAL, AND MOLECULAR-DATA WITH A NOSOLOGIC DISCUSSION, Hematology/oncology clinics of North America, 12(2), 1998, pp. 445
The histologic, chromosomal, and molecular features of malignant histi
ocytosis (MH) as an entity belonging to the malignant histiocytic diso
rders are reported in this article. The contributions pros vided by th
e MH-derived permanent cell lines are described more specifically. The
se in vitro studies have demonstrated that the MH proliferation is cha
racterized by a unique chromosomal abnormality, the 5q35 usually assoc
iated with a t(2;5) translocation generating a fusion gene NPM/ALK and
the subsequent expression of p80 protein. In addition, these in vitro
studies have shown that 5q35 proliferative cells are glass adherent,
can develop an immunodependent phagocytosis, and are able to reduce NE
T and produce TNF-alpha. Remarkably, they express constitutively c-fms
, the receptor of the macrophage growth factor, and under phorbol dies
ter stimulation, are able to modulate the expression of this receptor
and its ligand as well as TNF-alpha and IL-1. An important part of the
article is devoted to the nosological discussion concerning the conce
ptual controversy about the T-lymphoid or histiocytic origin of the pr
oliferative cells.