The presented case is a boy with T-cell acute lymphoblastic leukemia (ALL)
with hairy cell (HC) features and monoclonal gammopathy, The disease proces
s had an acute onset and followed a rapid, progressive course. The patient
had minimal splenomegaly and bicytopenia, but the bone marrow displayed inc
reased numbers of reticulin fibers. The blasts were positive for tartrate-r
esistant acid phosphatase (TRAP) and CD11c, Molecular analysis revealed rea
rrangement of immunoglobulin heavy chain genes and a rearranged T-cell rece
ptor (TcR beta) beta gene. The patient responded to conventional ALL therap
y. Acute T-cell ALL with HC features in childhood has not been reported pre
viously, either alone or in association with monoclonal gammopathy, We prop
ose "T-ALL with hairy cell features" to describe this case. (C) 2000 Wiley-
Liss, Inc.