Table 2.
Requirements to establish the diagnosis of T-PLL
| Major criteria | Minor criteria (at least 1 required) |
|---|---|
| • >5 × 109/L cells of T-PLL phenotype in peripheral blood or bone marrow | • Abnormalities involving chromosome 11 (11q22.3; ATM) |
| • T-cell clonality (by PCR for TRB/TRG, or by flow cytometry) | • Abnormalities in chromosome 8: idic(8)(p11), t(8;8), trisomy 8q |
| • Abnormalities of 14q32 or Xq28 OR expression of TCL1A/B, or MTCP1* | • Abnormalities in chromosome 5, 12, 13, 22, or complex karyotype |
| • Involvement of T-PLL specific site (eg, splenomegaly, effusions) |
*
Cases without TCL1A, TCL1B, or MTCP1 rearrangement or their respective overexpression are collected as TCL1-family negative T-PLL.