Occurrence of a Clonal T-Cell Population in a Case of Chronic Myelomonocytic Leukemia.

IF 3 Q2 Medicine
Clinical Medicine Insights-Blood Disorders Pub Date : 2021-02-12 eCollection Date: 2021-01-01 DOI:10.1177/2634853521991509
Anupama Patil, Balasaheb Wanve, Pradeep Kar, Shanthi Velusamy
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引用次数: 0

Abstract

Chronic myelo-monocytic leukemia (CMML) is an aggressive myeloid neoplasm with some features of a myelodysplastic syndrome (MDS) and others of a myeloproliferative neoplasm (MPN). Rarely, patients with CMML have a co-existing lympho-proliferative disorder (LPD). In most cases, the lymphoid neoplasm is diagnosed first, and the CMML is considered to be a secondary therapy-induced form of leukemia. We report herein a unique case of de-novo CMML, with an underlying clonal T-cell population and describe its clinical presentation and laboratory findings. A 70-year old male presented with a 3-month history of cough, dsypnea, abdominal distension, and low-grade fever. Physical and radiological examination revealed hepatosplenomegaly but no lymphadenopathy. Peripheral blood had absolute monocytosis with marrow showing CMML with 10% blasts along with dysplasia in myeloid and erythroid lineages. Flow cytometry indicated possibility of chronic myelo-monocytic leukemia with 13% monocytic cells along with an additional clonal population of gamma/delta T cells (15%) with aberrant immunophenotype. Polymerase chain reaction (PCR) analysis was positive for clonal T-cell rearrangement. A diagnosis of CMML with an underlying clonal T-CLPD was made. The synchronous occurrence of CMML and T-cell neoplasm may be attributed to a genetic mutation common to both. Currently, there are no treatment guidelines for group of patients; hence individualized therapeutic strategies should be implemented to enable symptomatic improvement and provide optimum care.

Abstract Image

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Abstract Image

慢性髓单细胞白血病一例克隆t细胞群的发生。
慢性骨髓单核细胞白血病(CMML)是一种侵袭性骨髓肿瘤,具有骨髓增生异常综合征(MDS)和骨髓增生性肿瘤(MPN)的一些特征。很少有CMML患者同时存在淋巴细胞增生性疾病(LPD)。在大多数情况下,淋巴样肿瘤首先被诊断出来,CMML被认为是治疗诱导的白血病的次要形式。我们在此报告一个独特的新生CMML病例,具有潜在的克隆t细胞群,并描述其临床表现和实验室结果。70岁男性,咳嗽、呼吸困难、腹胀、低烧3个月。体格及影像学检查显示肝脾肿大,但未见淋巴结病变。外周血绝对单核细胞增多,骨髓CMML伴10%母细胞,伴髓系和红系发育不良。流式细胞术显示可能为慢性骨髓单核细胞白血病,有13%的单核细胞伴有额外的γ / δ T细胞克隆群(15%),免疫表型异常。聚合酶链反应(PCR)分析克隆t细胞重排阳性。诊断CMML伴有潜在的克隆T-CLPD。CMML和t细胞肿瘤的同步发生可能归因于两者共同的基因突变。目前,没有针对患者群体的治疗指南;因此,应实施个体化治疗策略,以改善症状并提供最佳护理。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
CiteScore
3.70
自引率
0.00%
发文量
0
审稿时长
8 weeks
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