Sabnani Indu, Tsang Patricia
Department of Hematology and Oncology, Newark Beth Israel Medical Center, Newark, NJ 07112, USA.
Br J Haematol. 2007 Jan;136(1):30-7. doi: 10.1111/j.1365-2141.2006.06374.x. Epub 2006 Nov 8.
Abnormalities diagnosed on routine blood work, such as mild neutropenia, anaemia, thrombocytopenia and relative lymphocytosis, often have obscure aetiologies. A series of 30 patients were evaluated for various unexplained haematological abnormalities between 1997 and 2005, and found to have circulating monoclonal T-cell large granular lymphocytes (T-LGL). These patients fit the diagnosis of T-cell clonopathy of unknown significance (TCUS), which may represent a clinical spectrum of clonal T-LGL proliferation. Our patients were characterised by a complete absence of severe neutropenia (<0.5 x 10(9)/l), absence of recurrent neutropenic infection (0%), negative rheumatoid serology (0%) and a low incidence of constitutional symptoms (20%). This overall asymptomatic clinical presentation appeared to be different from other previously reported series of TCUS or T-LGL leukaemia who typically had symptomatology and required treatment. Our series of 30 patients represented the benign end of the spectrum of clonal T-LGL proliferation, and might reflect diagnosis at earlier stages of the condition relative to other reported series. TCUS may be a heterogeneous and under-diagnosed condition. This study further broadens our understanding of the clinical and laboratory manifestations of indolent clonal T-cell proliferation, and raises our awareness of this condition. We suggest that TCUS should be considered in the diagnostic evaluation of unexplained haematological problems.
常规血液检查诊断出的异常情况,如轻度中性粒细胞减少、贫血、血小板减少和相对淋巴细胞增多,其病因往往不明。1997年至2005年间,对30例有各种不明原因血液学异常的患者进行了评估,发现他们存在循环单克隆T细胞大颗粒淋巴细胞(T-LGL)。这些患者符合意义未明的T细胞克隆病(TCUS)的诊断,这可能代表了克隆性T-LGL增殖的一种临床谱。我们的患者具有以下特征:完全没有严重中性粒细胞减少(<0.5×10⁹/L)、没有复发性中性粒细胞减少感染(0%)、类风湿血清学阴性(0%)以及全身症状发生率低(20%)。这种总体无症状的临床表现似乎与先前报道的其他TCUS或T-LGL白血病系列不同,后者通常有症状且需要治疗。我们这组30例患者代表了克隆性T-LGL增殖谱的良性末端,可能反映了相对于其他报道系列而言该病处于更早阶段的诊断情况。TCUS可能是一种异质性且诊断不足的疾病。这项研究进一步拓宽了我们对惰性克隆性T细胞增殖的临床和实验室表现的理解,并提高了我们对这种疾病的认识。我们建议在不明原因血液学问题的诊断评估中应考虑TCUS。