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一例原发性骨髓纤维化患者的肾周髓外造血病例。

A case of perirenal extramedullary hematopoiesis in a patient with primary myelofibrosis.

作者信息

Imai Kentaro, Aoi Tomonori, Kitai Hiroki, Endo Nobuhide, Fujino Masahiko, Ichida Shizunori

机构信息

Department of Nephrology, Japanese Red Cross Nagoya Daiichi Hospital, 3-35 Michishita-cho, Nakamura-ku, Nagoya, Aichi, Japan.

Department of Pathology, Japanese Red Cross Nagoya Daiichi Hospital, Nagoya, Japan.

出版信息

CEN Case Rep. 2017 Nov;6(2):194-199. doi: 10.1007/s13730-017-0274-1. Epub 2017 Sep 11.

Abstract

Extramedullary hematopoiesis (EMH) is hematopoiesis in organs outside the bone marrow and most frequently occurs in the liver, spleen, and lymph nodes. We report a case of perirenal EMH revealed by kidney biopsy in a patient with primary myelofibrosis. We observed only bilateral kidney enlargement with plain computed tomography (CT) and ultrasonography before obtaining a renal biopsy. We obtained a percutaneous biopsy from the lower pole of the left kidney using ultrasonographic guidance. Ultrasonography just after the renal biopsy revealed no bleeding around the kidney. However, early the next morning, the patient developed severe hemorrhagic shock. Contrast-enhanced CT at that time revealed a massive hematoma in the left posterior perirenal space and bilateral abnormalities of the perirenal soft tissues. In patients with primary myelofibrosis, if plain CT shows an abnormal renal enlargement, EMH should be considered. In addition, a contrast-enhanced CT should be obtained before performing a percutaneous renal biopsy to assess for the possibility of perirenal EMH in these patients.

摘要

髓外造血(EMH)是指在骨髓以外的器官中进行的造血过程,最常见于肝脏、脾脏和淋巴结。我们报告了一例在原发性骨髓纤维化患者中通过肾脏活检发现的肾周EMH病例。在进行肾脏活检之前,我们通过普通计算机断层扫描(CT)和超声检查仅观察到双侧肾脏肿大。我们在超声引导下从左肾下极进行了经皮活检。肾脏活检后立即进行的超声检查显示肾脏周围无出血。然而,第二天一早,患者出现了严重的失血性休克。当时的增强CT显示左肾后间隙有巨大血肿以及肾周软组织的双侧异常。对于原发性骨髓纤维化患者,如果普通CT显示肾脏异常肿大,应考虑EMH。此外,在进行经皮肾活检之前应进行增强CT检查,以评估这些患者发生肾周EMH的可能性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/268d/5694411/f432900f2f24/13730_2017_274_Fig1_HTML.jpg

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