Department of Pathology, University of Michigan, Ann Arbor, MI 48109, USA.
Hum Pathol. 2011 Nov;42(11):1810-2. doi: 10.1016/j.humpath.2011.01.025. Epub 2011 Jun 12.
Retroperitoneal fibrosis is a rare fibroinflammatory condition involving the abdominal aorta, iliac vessels, and ureters that carries an association with several other autoimmune conditions. Most cases of retroperitoneal fibrosis are thought to be idiopathic. The disorder can affect all age groups but is most common in persons between the ages of 50 and 70 years. A subset of cases is associated with an underlying immunohematologic abnormality including lymphoma. We describe in this case report a highly unusual presentation of a young woman who died with a diagnosis of "idiopathic retroperitoneal fibrosis" based on multiple biopsy procedures. Postmortem examination, however, revealed disseminated anaplastic lymphoma kinase-positive anaplastic large cell lymphoma. The clinical and histopathologic importance of this very unusual presentation of anaplastic lymphoma kinase-positive anaplastic large cell lymphoma with retroperitoneal fibrosis is discussed.
腹膜后纤维化是一种罕见的纤维炎性疾病，涉及腹主动脉、髂血管和输尿管，与几种其他自身免疫性疾病有关。大多数腹膜后纤维化病例被认为是特发性的。该疾病可影响所有年龄段，但最常见于 50 至 70 岁之间的人群。一部分病例与潜在的免疫血液学异常有关，包括淋巴瘤。我们在本病例报告中描述了一位年轻女性的极不寻常表现，她在多次活检后被诊断为“特发性腹膜后纤维化”而死亡。然而，尸检显示弥漫性间变性淋巴瘤激酶阳性间变性大细胞淋巴瘤。讨论了这种腹膜后纤维化伴间变性淋巴瘤激酶阳性间变性大细胞淋巴瘤的非常不寻常表现的临床和组织病理学重要性。
