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Blood, 15 August 2000, Vol. 96, No. 4, pp. 1267-1273
Soluble stem cell factor receptor (CD117) and IL-2 receptor alpha
chain (CD25) levels in the plasma of patients with mastocytosis:
relationships to disease severity and bone marrow pathology
Cem Akin,
Lawrence B. Schwartz,
Takashi Kitoh,
Hirokazu Obayashi,
Alexandra S. Worobec,
Linda M. Scott, and
Dean D. Metcalfe
From the Laboratory of Allergic Diseases, National
Institute of Allergy and Infectious Diseases, National Institutes of
Health, Bethesda, MD; Virginia Commonwealth University, Richmond, VA;
and Nichirei Corporation, Tokyo, Japan.
Systemic mastocytosis is a disease of mast cell proliferation that
may be associated with hematologic disorders. There are no features on
examination that allow the diagnosis of systemic disease, and mast
cell-derived mediators, which may be elevated in urine or blood, may
also be elevated in individuals with severe allergic disorders. Thus,
the diagnosis usually depends on results of bone marrow biopsy. To
facilitate evaluation, surrogate markers of the extent and severity of
the disease are needed. Because of the association of mastocytosis with
hematologic disease, plasma levels were measured for soluble KIT (sKIT)
and soluble interleukin-2 receptor alpha chain (sCD25), which are known
to be cleaved in part from the mast cell surface and are elevated in
some hematologic malignancies. Results revealed that levels of
both soluble receptors are increased in systemic mastocytosis. Median
plasma sKIT concentrations as expressed by AU/mL (1 AU = 1.4
ng/mL) were as follows: controls, 176 (n = 60); urticaria pigmentosa
without systemic involvement, 194 (n = 8); systemic indolent
mastocytosis, 511 (n = 30); systemic mastocytosis with an associated
hematologic disorder, 1320 (n = 7); aggressive mastocytosis, 3390 (n = 3). Plasma sCD25 levels were elevated in systemic mastocytosis;
the highest levels were associated with extensive bone marrow
involvement. Levels of sKIT correlated with total tryptase levels,
sCD25 levels, and bone marrow pathology. These results demonstrate that
sKIT and sCD25 are useful surrogate markers of disease severity in
patients with mastocytosis and should aid in diagnosis, in the
selection of those needing a bone marrow biopsy, and in the
documentation of disease progression.
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