Distinguishing adult T-cell leukemia/lymphoma (ATLL) from peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) is pathologically difficult in the absence of clinical findings of monoclonal cells infected with human T-cell lymphotropic virus type-1 (HTLV-1). However, if the immunophenotypic difference between ATLL and PTCL-NOS is clearly identified, the pretest probability of ATLL will increase even without clinical information of HTLV-1, when distinguishing ATLL from PTCL-NOS. We clinicopathologically studied paraffin-embedded tissues of 37 patients who were diagnosed with ATLL or PTCL-NOS according to the WHO classification, 4th edition, at Showa University Hospital from November 1983 to September 2009. We analyzed the immunophenotypic differences between ATLL and PTCL-NOS by using immunohistochemistry. Significant differences between ATLL and PTCL-NOS were observed for CD7, CD25, CD56, CCR4, and TIA-1. Loss of CD7 was observed in all ATLL cases. CD25-positive cases represented 72% of the ATLL cases, i.e., more than in PTCL-NOS cases (P = 0.005). Similarly, CCR4-positive cases constituted 72% of the ATLL cases, i.e., more than in PTCL-NOS cases (P < 0.001). CD56- and TIA-1-positive cases were significantly more frequent in patients with PTCL-NOS than in patients with ATLL (CD56, P = 0.01; TIA-1, P = 0.03). Immunophenotypic evaluation for CD7, CD25, CD56, CCR4, and TIA-1 using immunohistochemistry is useful for distinguishing ATLL from PTCL-NOS. Therapies with anti-CD25 and anti-CCR4 antibodies are expected to be effective in ATLL treatment, because of high CD25- and CCR4-positive rates in ATLL patients.
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