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人类免疫缺陷病毒和人类疱疹病毒8阴性的真性红细胞增多症患者发生浆母细胞淋巴瘤所致纵隔综合征:一例报告

Mediastinal syndrome from plasmablastic lymphoma in human immunodeficiency virus and human herpes virus 8 negative patient with polycythemia vera: a case report.

作者信息

Cajozzo Massimo, Palumbo Vincenzo Davide, Buscemi Salvatore, Damiano Giuseppe, Florena Ada Maria, Cabibi Daniela, Raffaele Francesco, Anzalone Antonino Alessio, Fatica Federica, Cocchiara Gerlando, Dioguardi Salvatore, Bruno Antonio, Caronia Francesco Paolo, Lo Monte Attilio Ignazio

机构信息

Department of Surgical, Oncological and Stomatological Disciplines, University of Palermo, Via Del Vespro 129, 90127, Palermo, Italy.

Euro-Mediterranean Institute of Science and Technology (IEMEST), Palermo, Italy.

出版信息

J Med Case Rep. 2017 Mar 21;11(1):75. doi: 10.1186/s13256-016-1183-1.

Abstract

BACKGROUND

Plasmoblastic lymphoma is a rare and aggressive subtype of diffuse large B cell lymphoma, which occurs usually in the jaw of immunocompromised subjects.

CASE PRESENTATION

We describe the occurrence of plasmoblastic lymphoma in the mediastinum and chest wall skin of an human immunodeficiency virus-negative 63-year-old Caucasian man who had had polycytemia vera 7 years before. At admission, the patient showed a superior vena cava syndrome, with persistent dyspnoea, cough, and distension of the jugular veins. Imaging findings showed a 9.7 × 8 × 5.7 cm mediastinal mass. A chest wall neoformation biopsy and ultrasound-guided fine-needle aspiration biopsy of the mediastinal mass allowed diagnosis of plasmoblastic lymphoma and establishment of an immediate chemotherapeutic regimen, with rapid remission of compression symptoms.

CONCLUSIONS

Plasmoblastic lymphoma is a very uncommon, difficult to diagnose, and aggressive disease. The presented case represents the first rare mediastinal plasmoblastic lymphoma in a human immunodeficiency virus-/human herpesvirus-8-negative patient. Pathologists should be aware that this tumor does appear in sites other than the oral cavity. Fine-needle aspiration biopsy is a low-cost, repeatable, easy-to-perform technique, with a high diagnostic accuracy and with very low complication and mortality rates. Fine-needle aspiration biopsy could represent the right alternative to surgery in those patients affected by plasmoblastic lymphoma, being rapid and minimally invasive. It allowed establishment of prompt medical treatment with subsequent considerable reduction of the neoplastic tissue and resolution of the mediastinal syndrome.

摘要

背景

浆母细胞淋巴瘤是弥漫性大B细胞淋巴瘤的一种罕见且侵袭性的亚型,通常发生于免疫功能低下者的颌部。

病例报告

我们描述了一名63岁的人类免疫缺陷病毒阴性白种男性浆母细胞淋巴瘤的发生情况,该患者7年前曾患真性红细胞增多症,肿瘤位于纵隔和胸壁皮肤。入院时,患者表现为上腔静脉综合征,伴有持续呼吸困难、咳嗽和颈静脉扩张。影像学检查发现一个9.7×8×5.7 cm的纵隔肿块。胸壁新生物活检及纵隔肿块超声引导下细针穿刺活检确诊为浆母细胞淋巴瘤,并立即制定化疗方案,压迫症状迅速缓解。

结论

浆母细胞淋巴瘤是一种非常罕见、难以诊断且具有侵袭性的疾病。该病例是首例人类免疫缺陷病毒/人类疱疹病毒8阴性患者罕见的纵隔浆母细胞淋巴瘤。病理学家应意识到这种肿瘤确实会出现在口腔以外的部位。细针穿刺活检是一种低成本、可重复、易于操作的技术,诊断准确性高,并发症和死亡率极低。对于浆母细胞淋巴瘤患者,细针穿刺活检可能是手术的合适替代方法,因为它快速且微创。它使得能够迅速进行医学治疗,随后肿瘤组织显著减少,纵隔综合征得以缓解。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5b17/5360031/35b3ca2b2067/13256_2016_1183_Fig1_HTML.jpg

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