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骨髓增生异常综合征中外髓性髓样细胞瘤的淋巴结定位:1例经细针细胞学诊断的病例报告

Lymphnode localization of extramedullary myeloid cell tumor in myelodysplastic syndrome: report of one case diagnosed by fine-needle cytology.

作者信息

Fulciniti Franco, Zeppa Pio, Marino Gilda, Martinelli Vincenzo, Ciancia Rosanna, Del Vecchio Luigi, Rotoli Bruno, Palombini Lucio

机构信息

Dipartimento di Scienze Biomorfologiche e Funzionali, Sezione di Anatomia Patologica e Citopatologia, Università degli Studi di Napoli Federico II, Naples, Italy.

出版信息

Diagn Cytopathol. 2003 Mar;28(3):136-9. doi: 10.1002/dc.10256.

Abstract

Trilineage extramedullary myeloid tumor (EMT) is an uncommon medical condition mostly diagnosed in patients affected by acute or chronic myeloid leukemia or, more rarely, by a myelodysplastic syndrome, among which the most frequent is refractory anemia with excess of blasts in transformation (RAEB-t). The prognostic significance of EMT is still unclear, although the appearance of trilineage EMT is often considered to affect the outcome adversely. A 70-year-old lady with previous history of intestinal resection for colonic adenocarcinoma in 1995 and subsequently treated with 5-fuorouracyl developed a refractory anemia with excess of blasts (RAEB) in 1998. During the follow-up, a progression to RAEB-t was recorded. During chemotherapy for this condition, slight enlargement of left supraclavicular and right submandibular nodes was noticed. Fine-needle biopsy was performed with ancillary studies. A diagnosis of trilineage extramedullary myeloid tumor was reached. The patient was treated with low doses of chemotherapy with a good response lasting 12 months. The peculiar cytologic picture of this condition when corroborated by ancillary studies (immunocytochemistry and flow cytometry) is diagnostic of this rare condition. Furthermore, the extramedullary myeloid tumor in this case did not significantly affect the response to the chemotherapy of RAEB-t.

摘要

三系髓外髓样肿瘤(EMT)是一种罕见的疾病,大多在患有急性或慢性髓性白血病的患者中被诊断出来,或者更罕见地,在骨髓增生异常综合征患者中被诊断出来,其中最常见的是转化型原始细胞过多的难治性贫血(RAEB-t)。EMT的预后意义仍不清楚,尽管三系EMT的出现通常被认为会对预后产生不利影响。一位70岁的女士,1995年因结肠腺癌接受过肠道切除术,随后接受5-氟尿嘧啶治疗,1998年出现了原始细胞过多的难治性贫血(RAEB)。在随访期间,记录到病情进展为RAEB-t。在针对这种情况进行化疗期间,发现左锁骨上和右颌下淋巴结略有肿大。进行了细针穿刺活检并辅以相关检查。诊断为三系髓外髓样肿瘤。该患者接受了低剂量化疗,反应良好,持续了12个月。当这种情况特殊的细胞学表现得到辅助检查(免疫细胞化学和流式细胞术)证实时,可诊断这种罕见疾病。此外,该病例中的髓外髓样肿瘤对RAEB-t化疗反应没有显著影响。

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