Kuga T, Kohda K, Hirayama Y, Matsumoto S, Nakazawa O, Ando M, Ezoe A, Nobuoka A, Mochizuki C
Department of Internal Medicine, Japanese Red Cross Asahikawa Hospital, Japan.
Int J Hematol. 1996 Aug;64(2):143-50. doi: 10.1016/0925-5710(96)00467-7.
Although hepatic veno-occlusive disease (HVOD) is a common complication of allogenic bone marrow transplantation (BMT), pulmonary veno-occlusive disease (PVOD) is very rare. Only three cases have been described in the literature. We report the case of a 19-year-old woman who developed PVOD accompanied by microangiopathic hemolytic anemia (MAHA) and hemolytic uremic syndrome (HUS) 1 year after a second BMT for relapsed acute lymphoblastic leukemia (ALL). Autopsy examination revealed obstruction of the small pulmonary veins with edematous thickening of the intima. These findings are compatible with PVOD. Pulmonary GVHD and pulmonary aspergillosis were also observed. Various etiologic factors have been implicated in PVOD after BMT. We postulate that pulmonary GVHD and pulmonary infection including aspergillosis played an important role in the occurrence of both PVOD and HUS in our patient. Microangiopathic cytokines released in response to the GVHD and infection may damage the intima of microvessels that were previously injured by the two BMT. Despite appropriate therapy, the microangiopathic process was irreversible and the patient died. Thus, measures must be taken to prevent and treat PVOD after BMT.
尽管肝静脉闭塞性疾病（HVOD）是同种异体骨髓移植（BMT）的常见并发症，但肺静脉闭塞性疾病（PVOD）却非常罕见。文献中仅报道过3例。我们报告了1例19岁女性患者，其在因复发性急性淋巴细胞白血病（ALL）接受第二次BMT后1年，发生了PVOD，并伴有微血管病性溶血性贫血（MAHA）和溶血尿毒综合征（HUS）。尸检显示小肺静脉阻塞，内膜水肿增厚。这些发现符合PVOD。同时还观察到了肺部移植物抗宿主病（GVHD）和肺曲霉菌病。BMT后PVOD涉及多种病因。我们推测肺部GVHD和包括曲霉菌病在内的肺部感染在该患者PVOD和HUS的发生中起了重要作用。针对GVHD和感染释放的微血管病性细胞因子可能会损害先前两次BMT所损伤的微血管内膜。尽管进行了适当治疗，但微血管病过程不可逆，患者死亡。因此，必须采取措施预防和治疗BMT后的PVOD。
