Transformation of T‐cell large granular lymphocyte leukaemia into a high‐grade large T‐cell lymphoma
Transformation of T‐cell large granular lymphocyte leukaemia into a high‐grade large T‐cell lymphoma
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T细胞大颗粒淋巴细胞白血病转化为高级别大T细胞淋巴瘤
DOI：
10.1046/j.1365-2141.2001.03220.x
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发表时间：
2001
影响因子：
6.5
通讯作者：
D. Catovsky
中科院分区：
文献类型：
--
作者：
E. Matutes;A. Wotherspoon;N. Parker;N. Osuji;P. Isaacson;D. Catovsky
我们描述了一例T细胞大颗粒淋巴细胞（LGL）白血病，在诊断后11年转化为大细胞T细胞淋巴瘤。 1例29岁无症状女性患者于1989年出现淋巴细胞增多症、中性粒细胞减少症和轻度骨髓浸润。循环细胞为具有CD 2+、CD 3+、CD 8+、CD 4-、CD 16+、CD 56+、CD 57-表型的LGL。2000年8月，她出现发热、下颌下大肿块和肝脾肿大。生化检查显示肝功能异常，乳酸脱氢酶（LDH）水平升高。EB病毒、巨细胞病毒、人嗜T淋巴细胞病毒-I、人疱疹病毒（HHV）-6和HHV-7的血清学筛查结果为阴性。肿块的组织学与由大细胞组成的外周T细胞淋巴瘤的诊断一致，免疫组织化学显示淋巴瘤细胞具有与成熟LGL相同的表型。聚合酶链反应（PCR）分子分析表明，骨髓成熟LGL和淋巴结细胞中T细胞受体（TCR）γ链基因重排，具有相同大小的条带。患者接受CHOP（环磷酰胺、长春新碱、多柔比星和泼尼松龙）治疗，导致肿块消失，肝脾肿大、LDH和肝脏异常改善。她接受了脾切除术，脾组织学显示T细胞LGL白血病受累，无转化证据。该病例说明，转化或Richter综合征可能发生在少数T细胞LGL白血病患者中，这种疾病在大多数情况下具有良性临床病程。这是第一个通过分子方法记录的预先存在的克隆转化的病例。
We describe a case of T‐cell large granular lymphocyte (LGL) leukaemia that transformed into a large‐cell T‐cell lymphoma 11 years from diagnosis. A 29‐year‐old asymptomatic female presented in 1989 with lymphocytosis, neutropenia and mild bone marrow infiltration. The circulating cells were LGL with a CD2+, CD3+, CD8+, CD4–, CD16+, CD56+, CD57– phenotype. In August 2000, she developed fever, a large submandibular mass and hepatosplenomegaly. Biochemistry showed abnormal liver function tests and raised lactate dehydrogenase (LDH) levels. A serological screen for Epstein–Barr virus, cytomegalovirus, human T‐lymphotropic virus‐I, human herpes virus (HHV)‐6 and HHV‐7 was negative. Histology of the mass was consistent with the diagnosis of peripheral T‐cell lymphoma composed of large cells, and immunohistochemistry showed that the lymphoma cells had a phenotype identical to the mature LGL. Molecular analysis with the polymerase chain reaction (PCR) demonstrated rearrangement of the T‐cell receptor (TCR) γ‐chain gene with a band of identical size in both bone marrow mature LGL and lymph node cells. The patient was treated with CHOP (cyclophosphamide, vincristine, doxorubicin and prednisolone), resulting in the disappearance of the mass and improvement of the hepatosplenomegaly, LDH and liver abnormalities. She underwent splenectomy, and spleen histology showed involvement by T‐cell LGL leukaemia with no evidence of transformation. This case illustrates that transformation or Richter syndrome may occur in a minority of patients with T‐cell LGL leukaemia, a disease that has a benign clinical course in most cases. This is the first case documented by molecular methods of the transformation of the pre‐existing clone.
影响因子：
20.3
作者：
T. Loughran
通讯作者：
T. Loughran
CD3、CD56 大颗粒淋巴细胞白血病的侵袭性变体。
影响因子：
20.3
作者：
Gentile,TC;Uner,AH;Hutchison,RE;Wright,J;Ben-Ezra,J;Russell,EC;LoughranJr,TP
通讯作者：
LoughranJr,TP
自然杀伤样 T 细胞淋巴瘤：T 大颗粒淋巴细胞的侵袭性淋巴瘤。
影响因子：
20.3
作者：
Macon,WR;Williams,ME;Greer,JP;Hammer,RD;Glick,AD;Collins,RD;Cousar,JB
通讯作者：
Cousar,JB