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一例犬 T 细胞慢性淋巴细胞白血病进展为伴有中枢神经系统累及的里希特综合征。

A case of T-cell chronic lymphocytic leukemia progressing to Richter syndrome with central nervous system involvement in a dog.

机构信息

School of Veterinary Medicine, Small Animal Hospital, College of Medical, Veterinary, and Life Sciences, University of Glasgow, Glasgow, UK.

Veterinary Diagnostic Services, School of Veterinary Medicine, College of Medical, Veterinary, and Life Sciences, University of Glasgow, Glasgow, UK.

出版信息

Vet Clin Pathol. 2020 Mar;49(1):147-152. doi: 10.1111/vcp.12839. Epub 2020 Mar 25.

Abstract

An 8-year-old neutered Beagle dog was presented with polyuria and polydipsia. Routine clinicopathologic testing showed a significant lymphocytosis and proteinuria. Lymphocytes were of small to intermediate in size with a mature morphology. Infectious disease screening was negative. PCR for antigen receptor gene rearrangements showed a clonal T-cell receptor (TCR) rearrangement consistent with T-cell chronic lymphocytic leukemia (CLL). Bone marrow cytology showed <30% lymphocytes, while the proportion in splenic fine-needle aspirate cytology was considered increased. The dog was initially monitored but started on prednisolone and chlorambucil therapy 2 months later due to worsening clinical signs and progressive lymphocytosis. After an additional 2 weeks, the dog developed multifocal spinal pain and single-node lymphadenomegaly. Cytology of the lymph node showed a monomorphic population of large lymphoblasts consistent with lymphoma. Cytology of a cerebrospinal fluid sample also showed large lymphoblasts. PCR for antigen receptor gene rearrangement at both sites showed a clonal TCR rearrangement of the same molecular size as in the initial leukemic cells. The dog was diagnosed with a transformation of the CLL to Richter syndrome (RS) with involvement of the central nervous system (CNS). Therapy was started with L-asparaginase and an increased dose of prednisolone; however, the dog was euthanized due to progressive clinical signs. To our knowledge, this is the first report of canine RS with direct involvement of the CNS.

摘要

一只 8 岁已绝育的雄性比格犬出现多尿和多饮的症状。常规临床病理检查显示明显的淋巴细胞增多和蛋白尿。淋巴细胞大小为小到中等,形态成熟。传染病筛查为阴性。抗原受体基因重排 PCR 显示克隆性 T 细胞受体 (TCR) 重排,符合 T 细胞慢性淋巴细胞白血病 (CLL)。骨髓细胞学显示 <30%的淋巴细胞,而脾脏细针抽吸细胞学检查的比例被认为增加。这只狗最初接受了监测,但由于临床症状恶化和淋巴细胞增多,在 2 个月后开始接受泼尼松龙和苯丁酸氮芥治疗。又过了两周,狗出现多发性脊柱疼痛和单个淋巴结肿大。淋巴结细胞学检查显示为与淋巴瘤一致的大型淋巴母细胞的单形性群体。脑脊液样本的细胞学检查也显示有大型淋巴母细胞。这两个部位的抗原受体基因重排 PCR 均显示出与初始白血病细胞相同分子大小的克隆 TCR 重排。这只狗被诊断为 CLL 转化为 Richter 综合征 (RS),涉及中枢神经系统 (CNS)。开始用 L-天冬酰胺酶和增加剂量的泼尼松龙进行治疗;然而,由于临床症状的逐渐恶化,这只狗被安乐死。据我们所知,这是首例直接涉及中枢神经系统的犬类 RS 报告。

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