Blast and Bursts: Unveiling Splenic Rupture in Blastic Plasmacytoid Dendritic Cell Neoplasia.

IF 0.9 Q3 MEDICINE, GENERAL & INTERNAL
Abdulahi Hassan, Matthew Simpson, Rahim Jiwani, Abigail Arrigo, Palgun Nisarga, Olukemi A Esan, Anna Koget
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引用次数: 0

Abstract

Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) is a rare hematologic cancer, accounting for less than 1% of acute leukemias in the U.S. Diagnosis involves detecting markers like CD123, CD4, CD56, TCL1, and TCF4. Treatment typically involved acute leukemia therapies, but Tagraxofusp, a targeted therapy, was recently approved. Despite advancements, prognosis remains grim, with a median survival of around 1 year. Atraumatic splenic rupture (ASR) is a rare complication of this condition, with only five cases reported from 1994 to 2018. Here we present a case of BPDCN complicated by ASR. This case emphasizes the challenges of diagnosing and treating BPDCN, noting its rarity and absence of standard therapy. Tagraxofusp has shown promising results but presents safety concerns like capillary leak syndrome, particularly in elderly patients with comorbidities.

爆炸与爆发:揭开浆细胞性树突状细胞肿瘤的脾破裂之谜
在美国,BPDCN 是一种罕见的血液肿瘤,在急性白血病中的发病率不到 1%。治疗通常采用急性白血病疗法,但最近批准了一种靶向疗法 Tagraxofusp。尽管取得了进步,但预后仍然不容乐观,中位生存期约为 1 年。创伤性脾破裂(ASR)是该病的罕见并发症,从1994年到2018年仅报道了5例。在此,我们介绍一例并发 ASR 的 BPDCN 病例。该病例强调了诊断和治疗 BPDCN 所面临的挑战,指出了其罕见性和标准疗法的缺失。Tagraxofusp 已显示出良好的效果,但也存在毛细血管渗漏综合征等安全问题,尤其是在有合并症的老年患者中。
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来源期刊
自引率
0.00%
发文量
106
审稿时长
17 weeks
期刊介绍: JCHIMP provides: up-to-date information in the field of Internal Medicine to community hospital medical professionals a platform for clinical faculty, residents, and medical students to publish research relevant to community hospital programs. Manuscripts that explore aspects of medicine at community hospitals welcome, including but not limited to: the best practices of community academic programs community hospital-based research opinion and insight from community hospital leadership and faculty the scholarly work of residents and medical students affiliated with community hospitals.
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