VENOOCCLUSIVE DISEASE OF THE LIVER AFTER MARROW TRANSPLANTATION - HISTOLOGICAL CORRELATES OF CLINICAL SIGNS AND SYMPTOMS
VENOOCCLUSIVE DISEASE OF THE LIVER AFTER MARROW TRANSPLANTATION - HISTOLOGICAL CORRELATES OF CLINICAL SIGNS AND SYMPTOMS
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DOI：
10.1016/0270-9139(94)90864-8
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发表时间：
1994-05-01
期刊：
影响因子：
13.5
通讯作者：
MCDONALD, GB
中科院分区：
文献类型：
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作者：
SHULMAN, HM;FISHER, LB;MCDONALD, GB
我们评估了76名后来进行尸检的患者的临床症状和体征之间的关系，这些症状被称为肝静脉闭塞症。对编码尸检肝脏的个体组织学特征进行评分，这些特征与患者在生活中表现出的前瞻性临床特征相关。患者被分成两组：有严重临床静脉闭塞症的患者(n=32)和没有出现严重临床静脉闭塞疾病的患者。临床严重的静脉闭塞性疾病与3区肝小静脉闭塞、肝小静脉闭塞次数x闭塞程度、中心腔狭窄/静脉硬化、3区肝窦纤维化和3区肝细胞坏死(P均小于或等于0.03)相关。肝腺泡3区的这些组织学异常的数量与严重静脉闭塞疾病的临床诊断之间存在显著的相关性(p=0.003)。腹水的出现与小静脉闭塞、3区肝窦纤维化和3区肝细胞坏死显著相关(p=0.001)。移植后前20天的最大血清胆红素水平与肝窦纤维化、肝细胞坏死和偏心性管腔硬化/静脉硬化显著相关(p<；0.01)，而与静脉闭塞无关。肝毒性的临床综合征(通常称为静脉闭塞症)是由骨髓移植前的细胞减少治疗引起的，与一系列组织学损害密切相关，这些损害涉及肝腺泡3区和肝小静脉的结构，其中肝窦血液流入其中。这项研究表明，没有单一的诊断组织学特征。临床静脉闭塞疾病的严重程度似乎与此类组织学改变的数量成正比，而不仅仅是由于肝小静脉闭塞。
We evaluated the relationship between the signs and symptoms of the clinical syndrome called venocclusive disease of the liver after bone marrow transplantation and the histological findings in 76 patients who later came to autopsy. Coded necropsy liver was scored for individual histological features that were correlated with prospectively assessed clinical features that the patients had exhibited during life. Patients were stratified into two groups: those with severe clinical venocclusive disease (n = 32) and those without. Clinically severe venocclusive disease was statistically correlated with several zone 3 acinar changes: occluded hepatic venules, the frequency of occluded hepatic venules x degree of occlusion, cccentric luminal narrowing/phlebosclerosis, zone 3 sinusoidal fibrosis and zone 3 hepatocyte necrosis (all p less than or equal to 0.03). There was a significant relationship between the number of these histological abnormalities in zone 3 of the liver acinus and a clinical diagnosis of severe venocclusive disease (p = 0.003). The presence of ascites was significantly correlated with occluded venules, zone 3 sinusoidal fibrosis and zone 3 hepatocyte necrosis (p = 0.001). Maximum serum bilirubin in the first 20 days after transplant was significantly correlated with sinusoidal fibrosis, hepatocyte necrosis and eccentric luminal sclerosis/phlebosclerosis (p < 0.01) but not with venular occlusion. The clinical syndrome of liver toxicity (commonly called venocclusive disease) that results from cytoreductive therapy before bone marrow transplant is strongly correlated with a constellation of histological lesions involving structures in zone 3 of the liver acinus and the hepatic venules into which sinusoidal blood flows. This study suggests that there is no single diagnostic histological feature. The severity of clinical venocclusive disease appears to be proportional to the number of such histological changes and is not due solely to occlusion of small hepatic venules.