Dhodapkar M V, Li C Y, Lust J A, Tefferi A, Phyliky R L
Division of Hematology, Mayo Clinic and Foundation, Rochester, MN 55905.
Blood. 1994 Sep 1;84(5):1620-7.
We identified 68 patients with clonal T-large granular lymphocyte (T-LGL) proliferations who were seen at the Mayo Clinic between 1984 and 1992. Nineteen (28%) were asymptomatic at diagnosis, while the rest experienced fatigue (60%), B-symptoms (12%), and recurrent infections (15%). Associated comorbid conditions included rheumatoid arthritis (RA) in 26%. Severe anemia (hemoglobin [Hb] < 8g/dL) and neutopenia (absolute neutrophil count [ANC] < 500/microL) were seen in 19% and 40% of patients, respectively. Immunophenotypic studies showed CD3+, CD8+ phenotype in the majority (72%). Twenty-one patients (31%) have required no therapy, and remain relatively stable with a median follow-up period of 50 months. Treatment was required at either diagnosis (36 patients) or at subsequent follow-up (11 patients). Initial response rates were similar in patients treated with cyclophosphamide (CTX) with or without prednisone (69%), or prednisone alone (73%). Overall, 61 patients (90%) are alive with a median follow-up of 44 months. Actuarial median survival of this entire cohort is 161 months. The presence of anemia or symptoms does not appear to correlate with the tumor burden. In patients requiring therapy, a lower ANC and the presence of B-symptoms/infection were independently associated with a significantly lower probability of achieving a molecular or hematologic complete remission (H-CR). Intermittent immunosuppressive therapy is effective in achieving durable responses in a number of patients. T-LGL proliferations are associated with a favorable prognosis and response to therapy. However, significant heterogeneity exists in clinical presentation and associated comorbid conditions. These disorders should be included in the differential diagnosis of patients with unexplained cytopenias, particularly in the setting of RA and other autoimmune disorders. Analogous to the situation with monoclonal gammopathies, a term such as T-cell clonopathy of undetermined significance (TCUS) may be more appropriate to describe these patients.
我们确定了1984年至1992年间在梅奥诊所就诊的68例克隆性T大颗粒淋巴细胞（T-LGL）增殖患者。19例（28%）在诊断时无症状，其余患者有疲劳（60%）、B症状（12%）和反复感染（15%）。相关合并症包括26%的类风湿关节炎（RA）。分别有19%和40%的患者出现严重贫血（血红蛋白[Hb]<8g/dL）和中性粒细胞减少（绝对中性粒细胞计数[ANC]<500/μL）。免疫表型研究显示大多数（72%）为CD3+、CD8+表型。21例（31%）患者无需治疗，在中位随访期50个月时仍相对稳定。36例患者在诊断时或11例患者在随后的随访中需要治疗。接受环磷酰胺（CTX）联合或不联合泼尼松治疗的患者与单独接受泼尼松治疗的患者初始缓解率相似（分别为69%和73%）。总体而言，61例（90%）患者存活，中位随访时间为44个月。整个队列的精算中位生存期为161个月。贫血或症状的存在似乎与肿瘤负荷无关。在需要治疗的患者中，较低的ANC以及B症状/感染的存在与实现分子或血液学完全缓解（H-CR）的概率显著降低独立相关。间歇性免疫抑制治疗在许多患者中能有效实现持久缓解。T-LGL增殖与良好的预后和对治疗的反应相关。然而，临床表现和相关合并症存在显著异质性。这些疾病应纳入不明原因血细胞减少患者的鉴别诊断，特别是在RA和其他自身免疫性疾病的背景下。类似于单克隆丙种球蛋白病的情况，使用如意义未明的T细胞克隆病（TCUS）这样的术语来描述这些患者可能更合适。
