Cannizzo Elisa, Carulli Giovanni, Del Vecchio Luigi, Azzarà Antonio, Galimberti Sara, Ottaviano Virginia, Preffer Frederic, Petrini Mario
Division of Hematology, Department of Oncology, Transplants and Advances in Medicine, University of Pisa, 56126 Pisa, Italy.
Case Rep Hematol. 2011;2011:230568. doi: 10.1155/2011/230568. Epub 2011 Jul 28.
Acute undiffentiated leukemia (AUL) is an acute leukemia with no more than one membrane marker of any given lineage. Blasts often express HLA-DR, CD34, and/or CD38 and may be positive for terminal deoxynucleotidyl transferase (TdT). The expression of CD34, HLA-DR, and CD38 has been shown in pro-T-ALL, although in this case, blasts should also express CD7 and cyCD3. However, some cases of T-ALL without CD3 in the cytoplasm and all TCR chain genes in germ line configuration are reported, features that fit well with a very early hematopoietic cell. We report a case of acute leukemia CD34+/-HLADR+CD7+CD38+cyCD3- in which a diagnosis of AUL was considered. However the blasts were also positive for CD99 and TCR delta gene rearrangement which was found on molecular studies. Therefore a differential diagnosis between AUL and an early cyCD3 negative T-ALL was debated.
急性未分化白血病（AUL）是一种急性白血病，任何特定谱系的膜标志物均不超过一种。原始细胞通常表达HLA-DR、CD34和/或CD38，并且可能对末端脱氧核苷酸转移酶（TdT）呈阳性。CD34、HLA-DR和CD38的表达已在原T-ALL中得到证实，不过在这种情况下，原始细胞还应表达CD7和胞质环CD3。然而，有报道称一些T-ALL病例胞质中无CD3且所有TCR链基因处于种系构型，这些特征与非常早期的造血细胞相符。我们报告一例急性白血病，其表现为CD34+/-、HLA-DR+、CD7+、CD38+、胞质环CD3-，最初考虑诊断为AUL。然而，分子研究发现原始细胞对CD99也呈阳性且存在TCRδ基因重排。因此，对AUL与早期胞质环CD3阴性T-ALL之间的鉴别诊断展开了讨论。
