We present a case of primary plasma cell leukemia with Bence Jones pro
teinuria. After combination chemotherapy, leukemic cells and the urina
ry levels Bence Jones protein were decreased. Small lytic bone lesions
were detected only in the skull. Typical plasma cells were rarely see
n in peripheral blood on the hyperleukocytic phase, however they were
increased in the advanced stages. The most important diagnostic sign w
as persistent expression of CD38 antigen on leukemic cells throughout
the entire course of the illness and these leukemic cells expressed ve
ry late antigen-4 (VLA-4) but not VLA-5.