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伴有肿瘤溶解综合征的 B 淋巴细胞白血病患者的心肌钙化。

Myocardial calcification in a patient with B-lymphoblastic leukemia accompanied by tumor lysis syndrome.

机构信息

Department of Diagnostic Pathology, NTT Medical Center Tokyo, Tokyo, Japan.

Department of Diagnostic Pathology, NTT Medical Center Tokyo, Tokyo, Japan; Faculty of Healthcare, Tokyo Healthcare University, Tokyo, Japan.

出版信息

Cardiovasc Pathol. 2019 Nov-Dec;43:107146. doi: 10.1016/j.carpath.2019.07.005. Epub 2019 Aug 7.

Abstract

Myocardial calcification, a rare disease that leads to chronic or acute heart failure and with a poor prognosis, occurs in patients with abnormal calcium-phosphorus metabolism. The association between myocardial calcification and tumor lysis syndrome has not been reported to date. A 50-year-old man with hyperthermia and general malaise presented to our hospital and was clinically diagnosed with B-lymphoblastic leukemia (B-ALL) and febrile neutropenia accompanied by septic shock. Prednisolone was administered for tumor reduction. Two to three hours later, electrocardiography demonstrated ST elevation in V4-6, and blood tests showed elevated levels of cardiac enzymes. Transthoracic echocardiogram revealed diffuse severe hypokinesis with decreased left ventricular ejection fraction. Additionally, blood tests showed that serum phosphorus level increased to 8.0 mg/dl, which was likely due to tumor lysis syndrome. Circulatory and respiratory failure due to left heart failure progressed, and he died 3 days after administration of prednisolone. Pathological autopsy revealed diffuse proliferation of atypical B-lymphoblasts in the bone marrow, which led to the pathological diagnosis of B-ALL, accompanied by necrosis. On the cut surface of the heart, the left ventricle was dilated, and patchy yellowish-brown areas were present in the epicardial-side of the myocardium and spread through the circumferential wall of the left ventricle and interventricular septum. Microscopically, myocardial fibers were granularly basophilic in that area and were revealed as calcium deposits by Von Kossa staining. He was diagnosed with myocardial calcification. The drastic increase in the serum phosphorus level caused by tumor lysis syndrome seemed to be associated with myocardial calcification.

摘要

心肌钙化是一种罕见的疾病,可导致慢性或急性心力衰竭,预后不良,发生于钙磷代谢异常的患者。目前尚未有心肌钙化与肿瘤溶解综合征相关的报道。一名 50 岁男性因高热和全身不适就诊于我院,临床诊断为 B 淋巴细胞白血病(B-ALL)合并发热性中性粒细胞减少症,伴有感染性休克。给予泼尼松龙进行肿瘤减灭。2 至 3 小时后,心电图显示 V4-6 导联 ST 段抬高,血液检查显示心肌酶升高。经胸超声心动图显示弥漫性严重节段性运动异常,左心室射血分数降低。此外,血液检查显示血清磷水平升高至 8.0mg/dl,可能是由于肿瘤溶解综合征所致。由于左心衰竭导致循环和呼吸衰竭,他在给予泼尼松龙 3 天后死亡。病理尸检显示骨髓中弥漫性增生的非典型 B 淋巴细胞,导致 B-ALL 的病理诊断,伴有坏死。心脏切面显示左心室扩张,心外膜侧心肌有斑片状黄棕色区域,分布于左心室和室间隔的环形壁。镜下,该区域心肌纤维呈颗粒状嗜碱性,经 Von Kossa 染色显示为钙沉积。诊断为心肌钙化。肿瘤溶解综合征导致的血清磷水平急剧升高似乎与心肌钙化有关。

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