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Sudden death due to leukostasis in a subject with undiagnosed chronic lymphocytic leukemia
Forensic Science, Medicine and Pathology ( IF 1.8 ) Pub Date : 2021-08-20 , DOI: 10.1007/s12024-021-00406-9
Lorenzo Gitto 1 , Rohin Mehta 1 , Kanish Mirchia 1 , Christopher Ramos 1 , Michel Tawil 1 , Serenella Serinelli 1
Affiliation  

Sudden death due to leukostasis and lymphocyte thrombi in patients with chronic hematologic malignancies is rare. Leukostasis is characterized by highly elevated leukemic cell count and decreased tissue perfusion symptoms, leading to severe complications and even death. Chronic lymphocytic leukemia (CLL) is a chronic lymphoproliferative disorder that shows a highly heterogeneous clinical course, ranging from indolent form to very aggressive disease. Due to its low metabolic and mitotic rate, there is a lower incidence of clinically significant leukostasis in patients with CLL. Two main theories have been proposed in the development of leukostasis: (1) increased blood viscosity due to large leukemic cell populations; (2) high metabolic activity and cytokine production by leukemic cells. Both mechanisms lead to local hypoxic damage.

We present a case of a 70-year-old man who died suddenly in the absence of symptoms. Autopsy and histology examinations revealed findings consistent with CLL and diffuse leukostasis involving the major organs’ vessels.

In the presence of gross and/or microscopic findings suggesting a potential hematologic malignancy, undiagnosed or relapsing hematologic malignancies should be considered in the differential diagnosis of sudden deaths.



中文翻译:

未确诊慢性淋巴细胞白血病患者因白细胞淤滞猝死

慢性血液系统恶性肿瘤患者因白细胞淤滞和淋巴细胞血栓而猝死的情况很少见。白细胞淤滞的特征是白血病细胞计数高度升高和组织灌注症状减少,导致严重的并发症甚至死亡。慢性淋巴细胞白血病 (CLL) 是一种慢性淋巴组织增生性疾病,其临床病程具有高度异质性,范围从惰性形式到侵袭性很强的疾病。由于其低代谢和有丝分裂率,CLL 患者临床显着白细胞淤滞的发生率较低。在白细胞淤滞的发展过程中提出了两种主要理论:(1)由于大量白血病细胞群导致血液粘度增加;(2)白血病细胞的高代谢活性和细胞因子产生。这两种机制都会导致局部缺氧损伤。

我们介绍了一个 70 岁男性在没有症状的情况下突然死亡的案例。尸检和组织学检查显示与 CLL 和涉及主要器官血管的弥漫性白细胞淤滞一致的发现。

在存在提示潜在血液系统恶性肿瘤的肉眼和/或显微镜检查结果时,在猝死的鉴别诊断中应考虑未确诊或复发性血液系统恶性肿瘤。

更新日期:2021-08-20
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