Blood, 1 October 2002, Vol. 100, No. 7, pp. 2677-2677
CORRESPONDENCE
To the editor:
Is hemophagocytic lymphohistiocytosis an autoimmune disease?
Arkwright et al recently reviewed the association of
autoimmunity with several inherited immunodeficiency
diseases.1 It has been suggested that most if not all
autoimmune diseases are initiated by response to a single
self-antigen.2 Mackay and Rosen3 defined an
autoimmune disease as a clinical syndrome caused by the activation of T
and/or B cells in the absence of an ongoing infection or other
discernible cause. In many cases of autoimmune diseases, autoantibodies
are produced and may serve as markers of the antigen-specific B- and
T-cell response.2
In the context of X-linked lymphoproliferative disease (XLP) and
other inherited immunodeficiency diseases, Arkwright et al for
the first time included also the immunologic disorder "hemophagocytic lymphohistiocytosis (HLH)" as an autoimmune phenomenon.1
HLH is characterized by uncontrolled T-lymphocyte and macrophage
activation. Unrestricted release of inflammatory cytokines, such as
interferon and tumor necrosis factor, is a prominent feature of primary
and secondary HLH, including the Epstein-Barr virus-related
form.4,5
According to common classifications, HLH does not fulfill the criteria
of an autoimmune disease (ie, an immune reaction to a more or less
defined self-antigen).
We wonder, that Arkwright et al defines autoimmunity only as a
bystander tissue damage due to suboptimal, chronic immune response to
persisting opportunistic infection.1 Due to this
definition, all chronic infectious disease in immune-deficient subjects
would be classified as autoimmune phenomena.
Volker Schuster, Friedemann Horn, and Michael Borte
Correspondence: Volker Schuster, Department of Pediatrics,
Division of Immunology, University of Leipzig, Leipzig, Germany;
e-mail: schv{at}medizin.uni-leipzig.de
References
1.
Arkwright PD, Abinun M, Cant AJ.
Autoimmunity in human primary immunodeficiency diseases.
Blood.
2002;99:2694-2702[Abstract/Free Full Text].
2.
Marrack P, Kappler J, Kotzin BL.
Autoimmune disease: why and where it occurs.
Nat Med.
2001;7:899-905[CrossRef][Medline]
[Order article via Infotrieve].
3.
Mackay IR, Rosen FS.
Autoimmune diseases.
N Engl J Med.
2001;345:340-350[Free Full Text].
4.
Osugi Y, Hara J, Tagawa S, et al.
Cytokine production regulating Th1 and Th2 cytokines in hemophagocytic lymphohistiocytosis.
Blood.
1997;89:4100-4103[Abstract/Free Full Text].
5.
Lay JD, Tsao CJ, Chen JY, Kadin ME, Su IJ.
Upregulation of tumor necrosis factor-alpha gene by Epstein-Barr virus and activation of macrophages in Epstein-Barr virus-infected T cells in the pathogenesis of hemophagocytic syndrome.
J Clin Invest.
1997;100:1969-1979[Medline]
[Order article via Infotrieve].
