Hematoxylin and Eosin Bone Marrow and Liver Sections from Probands and Relatives with Aplastic Anemia, Acute Myeloid Leukemia, and Severe Liver Disease.

<p><b>(A)</b> Family A proband's bone marrow was hypocellular with isolated regions of normal cellularity (hematoxylin and eosin [H&E] staining; low power magnification). <b>(B)</b> Proband's father's bone marrow smear (Subject A-IV-29), illustrating dysplastic changes and increased number of blasts (H&E, high power magnification). <b>(C)</b> Subject A-IV-23's liver biopsy revealing islands of liver surrounded by zones of necrosis and parenchymal collapse (H&E, low magnification). The necrosis was far enough in the past that hepatocytes have mostly disappeared. In the inset, in some of the areas where hepatocytes were preserved there was still necrosis near the central veins. Little evidence of inflammation exists. <b>(D)</b> Subject's A-IV-25's liver biopsy showing small portal areas and poorly formed veins (H&E, low power magnification). <b>(E)</b> Same liver biopsy exhibiting widened hepatocyte plates on the reticulin stain (high power magnification), but clear changes of nodular regenerative hyperplasia were not seen. <b>(F)</b> The CD34 stain by was positive in sinusoidal endothelial cells consistent with an abnormal proportion of arterial blood flow to the sinuses (immunohistochemistry, low power magnification). <b>(G)</b> Liver biopsy of Subject A-III-11 in which the hepatic architecture is distorted by bridging fibrosis (low power magnification); the inset gives a close-up of the fibrosis. The biopsy revealed moderate inflammation but not elevated levels of plasma cells relative to other inflammatory cells. Other changes included interface hepatitis and cholatestasis. <b>(H)</b> Subject B-II-3's liver biopsy demonstrating portal inflammation with interface hepatitis (H&E, low power magnification). In the inset, Masson staining shows sclerosis around central vein with perisinusoidal fibrosis. <b>(I)</b> Subject B-III-7's liver biopsy with mild, macrovesicular steatosis in a zone 3 distribution. The inset indicates that there is mild lymphocytic portal inflammation with focal interface hepatitis (H&E). <b>(J)</b> Subject C-III-3's liver biopsy shows mild hepatocellular iron accumulation in a pericanalicular pattern; the sinusoidal-lining cells show mild to moderate iron accumulation. The inset illustrates mild variation in hepatocyte nuclear size. <b>(K)</b> Subject C-III-3's reticulin staining exemplifying several zones in which the hepatocyte plates were abnormally widened, consistent with regeneration. <b>(L)</b> Subject E-II-1's liver biopsy revealing some portal areas with mild inflammation and all with poorly formed, slit-like veins (H&E). <b>(M)</b> The reticulin stain showed evidence of nodular regenerative hyperplasia, with zones of plate widening alternating with areas of compression. <b>(N)</b> CD34 stain was abnormally positive in the sinusoidal endothelial cells by immunohistochemistry, indicating abnormal proportion of arterial blood flow to the sinuses.