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复发的血管免疫母细胞性T细胞淋巴瘤伴暴发性白血病浸润

Relapsed Angioimmunoblastic T Cell Lymphoma with Fulminant Leukemic Involvement.

作者信息

Altahan Rahaf, AlMugairi Areej, Almahayni Muhamed Hitham, Damlaj Moussab, Al-Zghoul Ahmad

机构信息

Hematology Section, Pathology and Clinical Laboratory Medicine Administration, King Fahad Medical City, Riyadh, Saudi Arabia.

Division of Hematopathology, Department of Pathology and Laboratory Medicine, King Abdulaziz Medical City, Riyadh, Saudi Arabia.

出版信息

Am J Case Rep. 2022 Aug 12;23:e936448. doi: 10.12659/AJCR.936448.

Abstract

BACKGROUND Angioimmunoblastic T cell lymphoma (AITL) is an aggressive and rare entity that comprises about 1-2% of all non-Hodgkin lymphomas. This entity carries many challenges that start at the diagnosis, as most patients present with non-specific symptoms affecting different systems. As a result, the optimal approach, reaching the accurate diagnosis, and delivering needed treatment are delayed. Furthermore, it is not surprising that the initial set of biopsies are non-diagnostic given the heavy inflammatory background and scarcity of malignant cells in the early course of the disease. Other challenges include delivering the optimal curative therapy, as there is no such therapeutic option available yet. Although stem cell transplantation (SCT) can be considered a curative option, some patients have comorbidities and are not eligible for this option, and some other patients have relapse despite this aggressive approach, as was seen in our case. CASE REPORT We present an interesting case of AITL with florid leukemic infiltration at the time of relapse. We included a description of the patient's symptoms, diagnostic challenges, and clinical course, and provided therapy with demonstrative peripheral blood and flow cytometry images. Interestingly, there are very few reports in the literature that described leukemic infiltration of this entity. CONCLUSIONS Acknowledging the rarity of this aggressive lymphoma combined with all the challenges that face the involved health care workers, publishing this elaborative case report adds some insight and knowledge and helps improve our understanding of this entity.

摘要

背景

血管免疫母细胞性T细胞淋巴瘤(AITL)是一种侵袭性罕见疾病,约占所有非霍奇金淋巴瘤的1%-2%。该疾病从诊断开始就面临诸多挑战,因为大多数患者表现出影响不同系统的非特异性症状。因此,达到准确诊断并提供所需治疗的最佳方法被延迟。此外,鉴于疾病早期炎症背景严重且恶性细胞稀少,最初的一系列活检无法确诊并不奇怪。其他挑战包括提供最佳的治愈性治疗,因为目前尚无此类治疗选择。尽管干细胞移植(SCT)可被视为一种治愈选择,但一些患者有合并症,不适合这种选择,而且其他一些患者尽管采取了这种积极的方法仍会复发,我们的病例就是如此。

病例报告

我们报告一例有趣的AITL病例,复发时伴有明显的白血病浸润。我们描述了患者的症状、诊断挑战和临床过程,并提供了具有代表性的外周血和流式细胞术图像。有趣的是,文献中很少有关于该疾病白血病浸润的报道。

结论

认识到这种侵袭性淋巴瘤的罕见性以及相关医护人员面临的所有挑战,发表这份详尽的病例报告增加了一些见解和知识,有助于提高我们对该疾病的认识。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/79cd/9380396/4472b9fc7845/amjcaserep-23-e936448-g001.jpg

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