Department of Internal Medicine, Mayo Clinic Florida, Jacksonville, FL.
Department of Pathology, Mayo Clinic Florida, Jacksonville, FL.
Clin Lymphoma Myeloma Leuk. 2021 Feb;21(2):106-112.e1. doi: 10.1016/j.clml.2020.09.013. Epub 2020 Oct 5.
Hepatosplenic T-cell lymphoma (HSTCL) is a rare subtype of peripheral T-cell lymphoma accounting for less than 1% of non-Hodgkin lymphomas. It is generally associated with poor prognosis.
We performed a cohort study of patients with HSTCL treated at the Mayo Clinic between 1996 and 2020 exploring the clinical characteristics and therapeutic outcomes.
Twenty-two cases of HSTCL were identified with a median (range) age at diagnosis of 45.5 (15.5-80.6) years and a male predominance (15/22, 68.2%). Clinical characteristics include massive splenomegaly in 16 patients (73%), hepatic involvement in 13 (59%), and chronic immunosuppressed state in 8 (36%). Phenotypically, lymphoma cells had gamma/delta T-cell receptor expression in 18 (82%) and alpha/beta in 4 patients. Cytogenetic abnormalities included isochromosome 7q (i7q) in 8 (62%) of 13 and trisomy 8 in 4 (44%) of 9. The median (range) follow-up of surviving patients was 33 (2.5-137) months. The median progression-free and overall survival were 9.5 months (95% CI, 1.8, 16.3) and 12.4 months (95% CI, 4.9, 18.5), respectively. Long-term survival was seen in 4 (18%) of 22 patients, with survival of 55, 74, 95, and 137 months. Moreover, 3 of 4 long-term survivors had splenectomy as part of initial treatment, and 2 of 4 long-term survivors received an allogeneic hematopoietic cell transplant (allo-HCT).
Liver involvement and chronic immunosuppression were associated with shorter survival. Although splenectomy and allo-HCT have anecdotal benefit in the literature, our data do not show a statistically significant benefit of splenectomy and/or allo-HCT, likely as a result of our small sample size.
肝脾 T 细胞淋巴瘤（HSTCL）是一种罕见的外周 T 细胞淋巴瘤亚型，占非霍奇金淋巴瘤的比例不到 1%。它通常与预后不良相关。
我们对 1996 年至 2020 年在 Mayo 诊所接受治疗的 HSTCL 患者进行了队列研究，探讨了其临床特征和治疗结果。
确定了 22 例 HSTCL 病例，中位（范围）诊断年龄为 45.5（15.5-80.6）岁，男性居多（15/22，68.2%）。临床特征包括 16 例（73%）患者巨脾肿大、13 例（59%）肝受累和 8 例（36%）慢性免疫抑制状态。表型上，淋巴瘤细胞中有 18 例（82%）表达γ/δ T 细胞受体，4 例表达α/β。细胞遗传学异常包括 13 例中的 8 例（62%）存在 7q 等臂染色体（i7q）和 9 例中的 4 例（44%）存在 8 三体。存活患者的中位（范围）随访时间为 33（2.5-137）个月。中位无进展生存期和总生存期分别为 9.5 个月（95%CI，1.8，16.3）和 12.4 个月（95%CI，4.9，18.5）。22 例患者中有 4 例（18%）长期存活，存活时间分别为 55、74、95 和 137 个月。此外，4 例长期幸存者中有 3 例在初始治疗中接受了脾切除术，有 2 例长期幸存者接受了异基因造血细胞移植（allo-HCT）。
肝受累和慢性免疫抑制与生存期更短相关。尽管脾切除术和 allo-HCT 在文献中有轶事获益，但我们的数据并未显示脾切除术和/或 allo-HCT 的统计学获益，可能是由于我们的样本量较小。
