Yutani C, Imakita M, Ishibashi-Ueda H, Okubo S, Naito M, Kunieda T
Division of Pathology, National Cardiovascular Center, Osaka, Japan.
Hum Pathol. 1988 Jun;19(6):726-31. doi: 10.1016/s0046-8177(88)80180-1.
Nodular regenerative hyperplasia (NRH), a rare hyperplastic condition of the liver, is reported in two patients with primary pulmonary hypertension (PPH). The first patient was a 26-year-old man who died of PPH and showed multiple NRH without cirrhosis of the liver. The second patient was a 25-year-old man who had a PPH with pulmonary arterial thrombi and NRH of the liver. NRH has been described in association with immune disease, hematopoietic disorder, and diabetes mellitus, so that NRH with PPH is considered to be very rare. Histologic findings of the lungs show typically plexogenic pulmonary arteriopathy in both cases, and the livers of these patients are composed of multiple nodules that are histologically represented by slightly larger hepatocytes arranged in a cobblestone-like fashion, and are ultramicroscopically characterized by massive proliferation of mitochondria. The pathogenetic association of nodular regenerative hyperplasia with primary pulmonary hypertension will be discussed.
结节性再生性增生（NRH）是一种罕见的肝脏增生性疾病，本文报道了两例原发性肺动脉高压（PPH）患者合并NRH的情况。第一例患者为一名26岁男性，死于PPH，肝脏表现为多处NRH，无肝硬化。第二例患者为一名25岁男性，患有PPH并伴有肺动脉血栓及肝脏NRH。NRH曾被报道与免疫疾病、造血系统疾病及糖尿病相关，因此PPH合并NRH被认为极为罕见。两例患者肺部的组织学表现均为典型的丛状肺动脉病，其肝脏由多个结节组成，组织学上表现为稍大的肝细胞呈鹅卵石样排列，超微结构特征为线粒体大量增生。本文将讨论结节性再生性增生与原发性肺动脉高压之间的发病机制联系。
