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不明原因血液学异常应归咎于克隆性T细胞大颗粒淋巴细胞吗?

Are clonal T-cell large granular lymphocytes to blame for unexplained haematological abnormalities?

作者信息

Sabnani Indu, Tsang Patricia

机构信息

Department of Hematology and Oncology, Newark Beth Israel Medical Center, Newark, NJ 07112, USA.

出版信息

Br J Haematol. 2007 Jan;136(1):30-7. doi: 10.1111/j.1365-2141.2006.06374.x. Epub 2006 Nov 8.

Abstract

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.

摘要

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。

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