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超声诊断自体骨髓移植患者的肝静脉闭塞病及用达那唑治疗自身免疫性血小板减少症

Sonographic diagnosis of venocclusive disease of the liver and danazol therapy for autoimmune thrombocytopenia in an autologous marrow transplant patient.

作者信息

Matsuishi E, Anzai K, Dohmen K, Taniguchi S, Gondo H, Kudo J, Shibuya T, Ishibashi H, Harada M, Niho Y

机构信息

First Department of Internal Medicine, Faculty of Medicine, Kyushu University, Fukuoka.

出版信息

Jpn J Clin Oncol. 1990 Jun;20(2):188-92.

PMID:2196389
Abstract

A 16-Year-old boy with lymphoblastic lymphoma underwent an autologous bone marrow transplantation (ABMT) after conditioning with high-dose busulfan and cyclophosphamide. On day 39 post-transplant, right upper quadrant pain occurred with an increase in the size of the liver. Liver function tests showed a subsequent deterioration. Ultrasonographic studies of the abdomen disclosed hepatosplenomegaly, ascites, thickening of the gall bladder wall and a failure to visualize the major hepatic veins. The venocclusive disease of the liver (VOD) diagnosis was confirmed from these findings. Ultrasonographic monitoring reflected the disease status well and demonstrated a complete recovery from the VOD. We emphasize, thus, that abdominal ultrasonography can be applied easily, being a non-invasive procedure, and is useful in diagnosing VOD. Furthermore, the procedure can be repeated serially for evaluating the severity of VOD. Although engraftment was confirmed with granulocytes exceeding 500/microliters, platelet recovery was delayed; the megakaryocytes had not decreased in bone marrow aspirates and platelet-associated IgG was significantly elevated. Since autoimmune thrombocytopenia was highly suspected, to prevent immunosuppression danazol was given as an immune modulator instead of prednisolone, and a complete recovery was obtained. Accordingly, danazol can be used as an alternative to prednisolone for the treatment of autoimmune thrombocytopenia after bone marrow transplantation.

摘要

一名16岁的淋巴母细胞淋巴瘤男孩在接受大剂量白消安和环磷酰胺预处理后进行了自体骨髓移植(ABMT)。移植后第39天,出现右上腹疼痛,肝脏肿大。肝功能检查显示随后恶化。腹部超声检查发现肝脾肿大、腹水、胆囊壁增厚,且未能显示主要肝静脉。根据这些发现确诊为肝静脉闭塞病(VOD)。超声监测很好地反映了病情,并显示VOD完全恢复。因此,我们强调,腹部超声检查作为一种非侵入性检查方法,应用简便,对诊断VOD很有用。此外,该检查可连续重复进行以评估VOD的严重程度。尽管粒细胞超过500/微升时已确认植入,但血小板恢复延迟;骨髓穿刺中巨核细胞未减少,且血小板相关IgG显著升高。由于高度怀疑自身免疫性血小板减少症,为防止免疫抑制,给予达那唑作为免疫调节剂而非泼尼松龙,最终完全康复。因此,达那唑可作为泼尼松龙的替代药物用于治疗骨髓移植后的自身免疫性血小板减少症。

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