慢性淋巴细胞白血病合并ALK+间变性大细胞淋巴瘤1例分析

IF 1.6 Q2 MEDICINE, GENERAL & INTERNAL Intractable & rare diseases research Pub Date : 2022-11-01 DOI:10.5582/irdr.2022.01086
Wuqiang Lin, Xiuli Chen, Zhenjie Cai, Heyong Zheng, Hanxing Huang, Huanxing Yang, Jianda Hu, Jing Zheng, Tetsuya Asakawa
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引用次数: 0

摘要

慢性淋巴细胞白血病(CLL)转化为更具侵袭性的淋巴瘤被称为Richter综合征(RS)。CLL合并t细胞瘤很少报道;ALK+间变性大细胞淋巴瘤(ALCL)也极少报道。中国南方一名63岁妇女,表现为全身淋巴结肿大和发热;她早在9年前就被诊断出患有慢性淋巴细胞白血病。根据她目前的诊断,是CLL + ALK+ ALCL。双淋巴结和骨髓活检显示两种类型的细胞群:i)左侧颈部淋巴结活检提示CLL (Ki67: 10%),骨髓活检显示小淋巴细胞增强(30%),胞质较少,细胞核圆形或不规则,大量染色质。可见表达CD30和ALK的大细胞(< 1%);免疫组化结果如下:CD20(弱阳性);PAX5(正面);CD23和CD5(弱阳性);CD3、CD10和CyclinD1(阴性);ii)左侧锁骨上淋巴结活检提示ALK+ ALCL (Ki67: 70%)。最终诊断为CLL合并ALCL。这种情况的机制尚不完全清楚,可能与CLL细胞对T细胞的慢性刺激以及免疫功能障碍有关。
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Insights from a patient with chronic lymphocytic leukemia complicating ALK+ anaplastic large cell lymphoma.

Chronic lymphocytic leukemia (CLL) that transforms into a more aggressive lymphoma has been termed Richter syndrome (RS). CLL with T-cell neoplasia is rarely reported; those with ALK+ anaplastic large cell lymphoma (ALCL) are also exceedingly rarely reported. A 63-year-old woman from the south of China presented with generalized lymphadenectasis and fever; she already had a prior diagnosis of CLL 9 years ago. As per her current diagnosis, it was CLL with ALK+ ALCL. The two-lymph node and bone marrow biopsies presented two types of cellular groups: i) left cervical lymph node biopsy suggested CLL (Ki67: 10%), along with bone marrow biopsy exhibited enhancement of the small lymphocytes (30%) with scant cytoplasm, round or irregular cell nuclei, and massive amounts of chromatin. Large cells (< 1%) that expressed CD30 and ALK were visible; The results of immunohistochemistry were as follows: CD20 (weak positive); PAX5 (positive); CD23 and CD5 (weak positive); and CD3, CD10, and CyclinD1 (negative); ii) left supraclavicular lymph node biopsy suggested ALK+ ALCL (Ki67: 70%). The final diagnosis was CLL with ALCL. The mechanisms of this condition are not fully understood, which might be associated with chronic stimulation of T cells by CLL cells along with immune dysfunction.

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Intractable & rare diseases research
Intractable & rare diseases research MEDICINE, GENERAL & INTERNAL-
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29
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