Successful treatment of noncirrhotic portal hypertension with eculizumab in paroxysmal nocturnal hemoglobinuria: A case report

Figure 1 Selected images from the liver biopsy showing changes of portal venopathy and features of nodular regenerative hyperplasia. A: A sclerosed PT without a visible portal vein. Peripherally, a dilated portal vein radicle herniates into the adjacent hepatic parenchyma (HE, x 10); B: Nodular regenerative hyperplasia. White dotted line circumscribes a nodule of regenerating hepatocytes surrounded by atrophic hepatocyte trabeculae (arrow), reticulin stain, x 10; C: THV with perivenular sclerosis. Sinusoidal dilatation in zone 3 (arrow), (HE, x 10); D: Sinusoidal capillarization in ischemic areas highlighted by CD34 immunostain. Arrow points to a THV (brown CD 34-positive sinusoidal staining), x 10; E: Keratin 7-positive atrophic hepatocytes indicative of ischemia in zone 3 (brown staining), x 10; F: Sinusoidal zone 3 fibrosis in zone 3 (arrow) and perivenular fibrosis in midleft, Masson trichrome stain, x 10; G: A fibrosed portal tract with a slit-like portal venule (arrow), PAS stain, x 20. PT: Portal tract; THV: Terminal hepatic vein; HE: Hematoxylin & eosin.

Figure 2 Clinical and laboratory course of the disease before and after treatment with eculizumab.