B-慢性淋巴细胞白血病在ofatumumab治疗期间显示三重转化,弥漫性大B细胞、cd20阴性和t细胞肿瘤:1例报告。

Q2 Medicine
BMC Clinical Pathology Pub Date : 2018-05-22 eCollection Date: 2018-01-01 DOI:10.1186/s12907-018-0072-5
Osamu Imataki, Makiko Uemura
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引用次数: 3

摘要

背景:慢性淋巴细胞白血病(CLL)是一种成熟的淋巴样肿瘤,目前被归类为惰性型恶性淋巴瘤。慢性淋巴细胞白血病进展缓慢,但最终会转变为更具侵袭性的淋巴瘤,如弥漫性大b细胞(DLBCL)型,也称为Richter综合征。病例介绍:我们治疗了一位69岁的日本男性,他在6年的慢性淋巴细胞白血病后被组织学诊断为里希特综合征。淋巴结病变全系统进展多年,淋巴细胞计数小于1万个/μL, Rai分级I期和Binet分级b期,Richter转化后出现高热、肝脾肿大。患者接受了ofatumumab治疗难治性CLL,缓解了他的发热性淋巴结病。他总共接受了11个ofatumumab疗程,并获得部分缓解。在第12个疗程的当天,他的疾病复发并伴有发热性淋巴结病。计算机断层扫描显示多发性肝脏肿块和全身性淋巴结病,而肝脏活检证实t细胞淋巴瘤。同时,外周血和骨髓中检测到cd20缺失的CLL细胞,左颈淋巴结活检病理检查显示cd20阳性DLBCL。最终诊断为三种不同类型的淋巴瘤病理:(1)淋巴结cd20阳性的DLBCL,(2)外周血和骨髓缺乏cd20的CLL,(3)肝脏外周t细胞淋巴瘤(PTCL)。他接受静脉和口服地塞米松治疗作为姑息治疗。他在诊断为三转化CLL后2个月因腹部肿块迅速发展而死亡。尸检显示侵袭性PTCL伴侵袭性全身累及肝脏、脾脏、胆囊、心包、骨髓和纵隔-腹主动脉旁-腹腔内淋巴结。尸检标本的t细胞受体研究支持PTCL扩散到腹腔内器官和淋巴结的诊断。我们的结论是,由于双重恶性转化,其致病性发展为三重淋巴瘤的混合物,包括CLL的PTCL, cd20阴性CLL和cd20阳性DLBCL的Richter转化。结论:我们的病例提供了CLL从低度化疗敏感状态转变为恶性化疗耐药状态的生物学信息。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

B-chronic lymphocytic leukemia showed triple transformation, to diffuse large B cell, CD20-negative, and T-cell neoplasm during ofatumumab treatment: a case report.

B-chronic lymphocytic leukemia showed triple transformation, to diffuse large B cell, CD20-negative, and T-cell neoplasm during ofatumumab treatment: a case report.

B-chronic lymphocytic leukemia showed triple transformation, to diffuse large B cell, CD20-negative, and T-cell neoplasm during ofatumumab treatment: a case report.

B-chronic lymphocytic leukemia showed triple transformation, to diffuse large B cell, CD20-negative, and T-cell neoplasm during ofatumumab treatment: a case report.

Background: Chronic lymphocytic leukemia (CLL) is a mature lymphoid neoplasm currently categorized as an indolent type of malignant lymphoma. CLL progresses slowly over years, but it eventually transforms to a more aggressive lymphoma such as the diffuse large B-cell (DLBCL) type, also known as Richter's syndrome.

Case presentation: We treated a 69-year-old Japanese male who was histologically diagnosed with Richter's syndrome after 6 years of CLL. His lymphadenopathy had systemically progressed for years, with lymphocyte counts of less than 10,000 cells/μL and a disease status of Rai classification stage I and Binet classification B. He had high fever and hepatosplenomegaly upon Richter's transformation. The patient was treated with ofatumumab for refractory CLL, which relieved his febrile lymphadenopathy. He received a total of 11 ofatumumab courses and achieved partial remission. On the day of the 12th course of ofatumumab, his disease relapsed with febrile lymphadenopathy. Computed tomography revealed multiple liver masses and systemic lymphadenopathy, while a liver biopsy confirmed T-cell lymphoma. Concomitantly, CD20-lacking CLL cells were detected in his peripheral blood and bone marrow, and pathological examination of his left cervical lymph node biopsy showed CD20-positive DLBCL. The final diagnosis was three different types of lymphoma pathologies: (1) CD20-positive DLBCL of the lymph nodes, (2) CD20-lacking CLL of the peripheral blood and bone marrow, and (3) peripheral T-cell lymphoma (PTCL) of the liver. He received intravenous and oral dexamethasone therapy as palliative care. He died because of the rapid progression of abdominal masses 2 months after the diagnosis of triple transformation CLL. An autopsy revealed aggressive PTCL with aggressive systemic involvement of the liver, spleen, gall bladder, pericardium, bone marrow, and mediastinal-paraaortic-intraceliac lymph nodes. T-cell receptor study of an autopsy specimen supported the diagnosis of PTCL that spread to the intraceliac organs and lymph nodes. We concluded that his pathogenicity progressed to a mixture of triple lymphoma as a result of double malignant transformations, which included PTCL from CLL, CD20-negative CLL, and CD20-positive DLBCL by Richter's transformation.

Conclusions: Our case provides information on the biology of CLL, to transform from a low-grade chemosensitive status to a malignant chemoresistant status.

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来源期刊
BMC Clinical Pathology
BMC Clinical Pathology Medicine-Pathology and Forensic Medicine
CiteScore
3.30
自引率
0.00%
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0
期刊介绍: BMC Clinical Pathology is an open access journal publishing original peer-reviewed research articles in all aspects of histopathology, haematology, clinical biochemistry, and medical microbiology (including virology, parasitology, and infection control). BMC Clinical Pathology (ISSN 1472-6890) is indexed/tracked/covered by PubMed, CAS, EMBASE, Scopus and Google Scholar.
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