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Broad Spectrum of Hepatocyte Inclusions in Humans, Animals, and Experimental Models

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Abstract

We focus on hepatic inclusions, which are defined as intracellular aggregates of stainable substances. They represent established hallmarks of their respective human disorders, but unlike aggregates found in neurodegenerative disorders are often not well studied. Hepatic inclusions can be subdivided into primary liver aggregates and aggregates found in multiple tissues. The former ones consist of inclusions found in endoplasmic reticulum storage diseases such as α 1‐antitrypsin aggregates or ground‐glass hepatocytes, p62‐containing (Mallory‐Denk bodies and intracellular hyaline bodies) and porphyrin‐containing inclusions. p62‐containing aggregates are not restricted to the liver but are found in multiple other disorders such as Parkinson or Alzheimer disease. Inclusions such as pale bodies or intracellular hyaline bodies are typical for malignant disorders while others (ground‐glass hepatocytes and α1‐antitrypsin aggregates) are predominantly seen in non‐neoplastic tissues. The inclusions, which are not restricted to the liver, are often due to a systemic viral infection, but also due to disruption of glycogen metabolism or systemic inclusion‐forming diseases such as polyglutamine disorders or sarcoidosis. Despite their heterogeneity, inclusions share several pathogenic principles such as an imbalance between protein damage/misfolding on one side and repair/degradation on the other side. This is why hepatic aggregates represent a valuable tool to study the aggregation process in general and to improve our understanding of inclusions found in multiple human disorders. © 2013 American Physiological Society. Compr Physiol 3:1393‐1436, 2013.

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Figure 1. Figure 1. Overview of hepatic inclusions. Aggregates found predominantly/exclusively in the liver are termed as primary liver inclusions. IB, Inclusion body.
Figure 2. Figure 2. Inclusions found in ER‐storage disorders share common pathogenic mechanisms with p62‐containing cytoplasmic aggregates. Aggregates seen in ER‐storage disorders and p62‐containing cytoplasmic aggregates represent the major subtypes of primary hepatocellular inclusions. The former ones form as a consequence of disturbed processing of secreted proteins with a retention in the endoplasmic reticulum (ER) and include ground‐glass hepatocytes (GGHs) and aggregates consisting of mutant α1‐antitrypsin (AAT) as prime examples. The latter ones are made from nonsecreted proteins, which aggregate in the cytoplasm as it happens in the case of Mallory‐Denk bodies (MDBs). During MDB formation, keratin network becomes disrupted to form large perinuclear aggregates consisting of cross‐linked, hyperphosphorylated (P) and ubiquitinated (Ub) keratins 8/18. (K8/K18). Heat‐shock proteins (hsp) and p62 constitute additional established MDB components. Although both aggregate subtypes are clearly distinct, they share common pathogenic mechanisms such as accumulation of misfolded proteins which is not sufficiently counteracted by protein degradation. Last but not least, an imbalance in protein stoichiometry (i.e., between K8/K18 in MDBs and isoforms of hepatitis B antigen in GGHs) promotes the formation of both aggregate subtypes.
Figure 3. Figure 3. α1‐antitrypsin aggregates in a patient carrying a PiZZ mutation. Liver sections were stained with hematoxylin and eosin (H&E) (A) or with periodic acid Schiff after diastase digestion (PAS‐D) (C). The arrows point to the aggregates, which are difficult to be demonstrated in H&E where they are faintly eosinophilic, but are clearly distinguished as brilliant red structures in PAS‐D. In electron microscopy, PiZZ subject display dilated ER (arrows in C) which is seen before the large inclusions form (I in D). N, nucleus. Pictures by courtesy of Dr. Jeffrey Teckman, Saint Louis University School of Medicine, St Louis, MO.
Figure 4. Figure 4. α1‐antichymotrypsin aggregates in a patient with serum α1‐antichymotrypsin deficiency. Hematoxylin and eosin (H&E)‐stained liver sections (A) revealed chronic active hepatitis in a cirrhotic stage without any apparent aggregates, while immunohistochemical staining (B) with antibody against α1‐antichymotrypsin showed granular inclusions predominantly in the periportal area adjacent to fibrous bands. Transmission electron microscopy uncovered fluffy material located in a dilated ER. Pictures are reprinted (in a modified version), with permission, from Lindmark et al., Histopathology 1990 () (permission to reprint published figures has been granted by the publisher).
Figure 5. Figure 5. Ground‐glass hepatocytes in patients with chronic hepatitis B. Liver sections were stained with hematoxylin and eosin (H&E) (A) or immunohistochemically with an antibody against hepatitis B surface antigen (HBs) (B and C). In H&E staining, GGHs are defined as finely granular, eosinophilic and uniformly dull appearing cytoplasm, sometimes with a halo at the cell periphery (arrows in A). GGHs can be further subdivided into type I inclusions, which are disseminated and display excentric nuclei (B) and type II aggregates, which are present in clusters and located rather in cell periphery (C).
Figure 6. Figure 6. Fibrinogen aggregates in a patient with familial hypofibrinogenemia. Liver sections incubated without (A) and with (B and D) an antibody against fibrinogen. Massive fibrinogen accumulation and formation of aggregates in subjects carrying fibrinogen mutation is shown. On the other hand, no obvious aggregates are seen in hematoxylin and eosin (H&E) staining (C). Electron microscopy (E) shows the inclusions composed of densely packed tubular structures, arranged in curved bundles. Immunogold labeling (F) confirms the abundant presence of fibrinogen within the aggregates. Pictures by courtesy of Drs. Peter Schirmacher (University of Heidelberg Medical School, Germany) and Stephan O. Brennan (Canterbury Health Laboratories, Christchurch, New Zealand).
Figure 7. Figure 7. Pale bodies in hepatocellular carcinoma. Hematoxylin and eosin (H&E)‐stained liver sections (A) revealed a large number of hepatocytes with pale or eosinophilic cytoplasm (arrows in A). Immunohistochemically, the inclusions were strongly positive for fibrinogen (B) and in electron microscopy, they appeared as nonmembrane‐bound, amorphous, fine granular, or fibrillar aggregates located within the dilated rough ER (C). Pictures are reprinted (in a modified version), with permission, from () Moon et al., J Korean Med Sci 2000 (permission to reprint published figures has been granted by the publisher).
Figure 8. Figure 8. Mallory‐Denk bodies (MDBs) in patients with alcoholic steatohepatitis. Formaldehyde‐fixed, paraffin‐embedded liver sections were stained with hematoxylin and eosin (H&E, A) or immunohistochemically labeled using antibodies against keratin 8/18 (K8/18) (B), ubiquitin (C), or p62 (D). MDBs are highlighted by arrows and appear as irregular eosinophilic inclusions in H&E, while they are bright red in immunohistochemistry. Note the insert in (A) which contains a cell with a prominent MDB surrounded by polymorphonuclear leucocytes—a phenomenon termed satellitosis.
Figure 9. Figure 9. Mallory‐Denk bodies (MDBs) formation is associated with profound keratin 8/18 redistribution. Double immunofluorescence staining depicts the distribution of keratins 8 and 18 (red) as well as p62 (green). Control (A) and DDC‐fed mouse livers (B‐D) are shown, the latter ones representing an established animal MDB model (B‐D). In untreated livers (A), keratins form a fine cytoplasmic meshwork (A), which undergoes a variety of changes after DDC administration (B‐D). These include a partial disruption of keratin filament network with formation of small keratin/p62‐positive aggregates alongside the residual keratin filaments (B), formation of large keratin/p62‐positive perinuclear inclusions (C) or an almost complete loss of keratin 8/18 fluorescence (D).
Figure 10. Figure 10. Intracellular hyaline bodies in hepatocellular carcinoma. Hematoxylin and eosin (H&E)‐stained hepatocellular cancer sections (A) depict a large number of eosinophilic structures surrounded by a clear halo which correspond to intracellular hyaline bodies (IHBs, arrows in A). Insert in (A) shows this aggregate in a large magnification. These deposits appear red in chromotrope aniline blue‐stained sections (arrows in B) and display an indistinct fibrillar/granular structure in electron microscopy (asterisk in C). In immunofluorescence, IHBs are p62 positive (arrows in D), but keratin 8/18 negative (E). Micrograph (F) shows the merged signal from double‐fluorescence staining and thereby discriminates between IHBs (red) and Mallory‐Denk bodies, which are yellow due to presence of both keratins and p62.
Figure 11. Figure 11. Needle‐like inclusions in patients with porphyria cutanea tarda. Liver sections were stained with Nuclear Fast Red alone (A) or in combination with Ferric ferricyanine reduction reaction (B). Alternatively, Needle‐like inclusions were visualized in polarized light (C) or by using transmission electron microscopy (D) Pictures by courtesy of Dr. Alena Chlumska, Charles University School of Medicine, Pilsen, Czech Republic.
Figure 12. Figure 12. Viral inclusions. Representative hematoxylin and eosin (H&E)‐stained liver sections from subjects with cytomegalovirus (CMV, A), herpes simplex (HSV, B), or Ebola virus (C) infection. Hepatocellular CMV affection leads to formation of large CMV intranuclear as well as small cytoplasmic inclusion bodies, the latter ones being termed dense bodies (arrows in A). Systemic HSV‐infection causes glassy, amphophilic, intranuclear herpetic inclusions (arrows in B) while Ebola virus forms typical cytoplasmic eosinophilic and filamentous inclusions (arrows in C). The picture (A) is by courtesy of Dr. Michael Mihalov, LUMEN—Loyola University Medical Education Network, USA. Pictures (B and C) are reprinted, with permission, from 6th edition of Mac Sween's Pathology of the Liver (permission to reprint published figures has been granted by the publisher) ().
Figure 13. Figure 13. Polyglucosan inclusions. Liver histology in polyglucosan body disease. (A) Liver biopsy in polyglucosan body disease with mild chronic hepatitis. Polyglucosan bodies (PGBs) are present in periportal hepatocytes (long arrow). The portal tract is expanded by a mild lymphocytic infiltrate with interface hepatitis, pale polyglucosan‐laden macrophages, fibrosis, and ductular reaction (short arrows). (H&E stain, original magnification 100×.) (B) The trichrome connective tissue stain highlights mild periportal fibrosis (collagen fibers in blue). Numerous pale eosinophilic PGBs are evident. (Trichrome stain; original magnification 100×.) (C) Periportal hepatocytes predominantly demonstrate PGBs in adult polyglucosan body disease. The inclusions resemble the ground‐glass inclusions of chronic hepatitis B. (C) One of many portal tracts (PT) showing variable mild chronic inflammation and the adjacent round‐to‐oval pale eosinophilic PGBs in hepatocytes. (D) PGBs are sharply demarcated within hepatocytes and often displace the hepatocyte nucleus to the cell periphery. (C and D) H&E, original magnifications ×200, ×400. (E) PGBs are strongly positive with periodic acid Schiff (PAS) stain, with the most inclusions being located near portal tracts (PT). Inset: several dense oval inclusions are evident in periportal hepatocytes. (F) The hepatocellular inclusions display retention of moderate staining with diastase‐treated PAS (arrows). (G) Colloidal iron stain shows numerous periportal PGBs with blue‐green staining. (Original magnifications E: ×100; inset: ×400; F: ×400; G: ×200). [Modified and reprinted with permission by the publisher from ().]


Figure 1. Overview of hepatic inclusions. Aggregates found predominantly/exclusively in the liver are termed as primary liver inclusions. IB, Inclusion body.


Figure 2. Inclusions found in ER‐storage disorders share common pathogenic mechanisms with p62‐containing cytoplasmic aggregates. Aggregates seen in ER‐storage disorders and p62‐containing cytoplasmic aggregates represent the major subtypes of primary hepatocellular inclusions. The former ones form as a consequence of disturbed processing of secreted proteins with a retention in the endoplasmic reticulum (ER) and include ground‐glass hepatocytes (GGHs) and aggregates consisting of mutant α1‐antitrypsin (AAT) as prime examples. The latter ones are made from nonsecreted proteins, which aggregate in the cytoplasm as it happens in the case of Mallory‐Denk bodies (MDBs). During MDB formation, keratin network becomes disrupted to form large perinuclear aggregates consisting of cross‐linked, hyperphosphorylated (P) and ubiquitinated (Ub) keratins 8/18. (K8/K18). Heat‐shock proteins (hsp) and p62 constitute additional established MDB components. Although both aggregate subtypes are clearly distinct, they share common pathogenic mechanisms such as accumulation of misfolded proteins which is not sufficiently counteracted by protein degradation. Last but not least, an imbalance in protein stoichiometry (i.e., between K8/K18 in MDBs and isoforms of hepatitis B antigen in GGHs) promotes the formation of both aggregate subtypes.


Figure 3. α1‐antitrypsin aggregates in a patient carrying a PiZZ mutation. Liver sections were stained with hematoxylin and eosin (H&E) (A) or with periodic acid Schiff after diastase digestion (PAS‐D) (C). The arrows point to the aggregates, which are difficult to be demonstrated in H&E where they are faintly eosinophilic, but are clearly distinguished as brilliant red structures in PAS‐D. In electron microscopy, PiZZ subject display dilated ER (arrows in C) which is seen before the large inclusions form (I in D). N, nucleus. Pictures by courtesy of Dr. Jeffrey Teckman, Saint Louis University School of Medicine, St Louis, MO.


Figure 4. α1‐antichymotrypsin aggregates in a patient with serum α1‐antichymotrypsin deficiency. Hematoxylin and eosin (H&E)‐stained liver sections (A) revealed chronic active hepatitis in a cirrhotic stage without any apparent aggregates, while immunohistochemical staining (B) with antibody against α1‐antichymotrypsin showed granular inclusions predominantly in the periportal area adjacent to fibrous bands. Transmission electron microscopy uncovered fluffy material located in a dilated ER. Pictures are reprinted (in a modified version), with permission, from Lindmark et al., Histopathology 1990 () (permission to reprint published figures has been granted by the publisher).


Figure 5. Ground‐glass hepatocytes in patients with chronic hepatitis B. Liver sections were stained with hematoxylin and eosin (H&E) (A) or immunohistochemically with an antibody against hepatitis B surface antigen (HBs) (B and C). In H&E staining, GGHs are defined as finely granular, eosinophilic and uniformly dull appearing cytoplasm, sometimes with a halo at the cell periphery (arrows in A). GGHs can be further subdivided into type I inclusions, which are disseminated and display excentric nuclei (B) and type II aggregates, which are present in clusters and located rather in cell periphery (C).


Figure 6. Fibrinogen aggregates in a patient with familial hypofibrinogenemia. Liver sections incubated without (A) and with (B and D) an antibody against fibrinogen. Massive fibrinogen accumulation and formation of aggregates in subjects carrying fibrinogen mutation is shown. On the other hand, no obvious aggregates are seen in hematoxylin and eosin (H&E) staining (C). Electron microscopy (E) shows the inclusions composed of densely packed tubular structures, arranged in curved bundles. Immunogold labeling (F) confirms the abundant presence of fibrinogen within the aggregates. Pictures by courtesy of Drs. Peter Schirmacher (University of Heidelberg Medical School, Germany) and Stephan O. Brennan (Canterbury Health Laboratories, Christchurch, New Zealand).


Figure 7. Pale bodies in hepatocellular carcinoma. Hematoxylin and eosin (H&E)‐stained liver sections (A) revealed a large number of hepatocytes with pale or eosinophilic cytoplasm (arrows in A). Immunohistochemically, the inclusions were strongly positive for fibrinogen (B) and in electron microscopy, they appeared as nonmembrane‐bound, amorphous, fine granular, or fibrillar aggregates located within the dilated rough ER (C). Pictures are reprinted (in a modified version), with permission, from () Moon et al., J Korean Med Sci 2000 (permission to reprint published figures has been granted by the publisher).


Figure 8. Mallory‐Denk bodies (MDBs) in patients with alcoholic steatohepatitis. Formaldehyde‐fixed, paraffin‐embedded liver sections were stained with hematoxylin and eosin (H&E, A) or immunohistochemically labeled using antibodies against keratin 8/18 (K8/18) (B), ubiquitin (C), or p62 (D). MDBs are highlighted by arrows and appear as irregular eosinophilic inclusions in H&E, while they are bright red in immunohistochemistry. Note the insert in (A) which contains a cell with a prominent MDB surrounded by polymorphonuclear leucocytes—a phenomenon termed satellitosis.


Figure 9. Mallory‐Denk bodies (MDBs) formation is associated with profound keratin 8/18 redistribution. Double immunofluorescence staining depicts the distribution of keratins 8 and 18 (red) as well as p62 (green). Control (A) and DDC‐fed mouse livers (B‐D) are shown, the latter ones representing an established animal MDB model (B‐D). In untreated livers (A), keratins form a fine cytoplasmic meshwork (A), which undergoes a variety of changes after DDC administration (B‐D). These include a partial disruption of keratin filament network with formation of small keratin/p62‐positive aggregates alongside the residual keratin filaments (B), formation of large keratin/p62‐positive perinuclear inclusions (C) or an almost complete loss of keratin 8/18 fluorescence (D).


Figure 10. Intracellular hyaline bodies in hepatocellular carcinoma. Hematoxylin and eosin (H&E)‐stained hepatocellular cancer sections (A) depict a large number of eosinophilic structures surrounded by a clear halo which correspond to intracellular hyaline bodies (IHBs, arrows in A). Insert in (A) shows this aggregate in a large magnification. These deposits appear red in chromotrope aniline blue‐stained sections (arrows in B) and display an indistinct fibrillar/granular structure in electron microscopy (asterisk in C). In immunofluorescence, IHBs are p62 positive (arrows in D), but keratin 8/18 negative (E). Micrograph (F) shows the merged signal from double‐fluorescence staining and thereby discriminates between IHBs (red) and Mallory‐Denk bodies, which are yellow due to presence of both keratins and p62.


Figure 11. Needle‐like inclusions in patients with porphyria cutanea tarda. Liver sections were stained with Nuclear Fast Red alone (A) or in combination with Ferric ferricyanine reduction reaction (B). Alternatively, Needle‐like inclusions were visualized in polarized light (C) or by using transmission electron microscopy (D) Pictures by courtesy of Dr. Alena Chlumska, Charles University School of Medicine, Pilsen, Czech Republic.


Figure 12. Viral inclusions. Representative hematoxylin and eosin (H&E)‐stained liver sections from subjects with cytomegalovirus (CMV, A), herpes simplex (HSV, B), or Ebola virus (C) infection. Hepatocellular CMV affection leads to formation of large CMV intranuclear as well as small cytoplasmic inclusion bodies, the latter ones being termed dense bodies (arrows in A). Systemic HSV‐infection causes glassy, amphophilic, intranuclear herpetic inclusions (arrows in B) while Ebola virus forms typical cytoplasmic eosinophilic and filamentous inclusions (arrows in C). The picture (A) is by courtesy of Dr. Michael Mihalov, LUMEN—Loyola University Medical Education Network, USA. Pictures (B and C) are reprinted, with permission, from 6th edition of Mac Sween's Pathology of the Liver (permission to reprint published figures has been granted by the publisher) ().


Figure 13. Polyglucosan inclusions. Liver histology in polyglucosan body disease. (A) Liver biopsy in polyglucosan body disease with mild chronic hepatitis. Polyglucosan bodies (PGBs) are present in periportal hepatocytes (long arrow). The portal tract is expanded by a mild lymphocytic infiltrate with interface hepatitis, pale polyglucosan‐laden macrophages, fibrosis, and ductular reaction (short arrows). (H&E stain, original magnification 100×.) (B) The trichrome connective tissue stain highlights mild periportal fibrosis (collagen fibers in blue). Numerous pale eosinophilic PGBs are evident. (Trichrome stain; original magnification 100×.) (C) Periportal hepatocytes predominantly demonstrate PGBs in adult polyglucosan body disease. The inclusions resemble the ground‐glass inclusions of chronic hepatitis B. (C) One of many portal tracts (PT) showing variable mild chronic inflammation and the adjacent round‐to‐oval pale eosinophilic PGBs in hepatocytes. (D) PGBs are sharply demarcated within hepatocytes and often displace the hepatocyte nucleus to the cell periphery. (C and D) H&E, original magnifications ×200, ×400. (E) PGBs are strongly positive with periodic acid Schiff (PAS) stain, with the most inclusions being located near portal tracts (PT). Inset: several dense oval inclusions are evident in periportal hepatocytes. (F) The hepatocellular inclusions display retention of moderate staining with diastase‐treated PAS (arrows). (G) Colloidal iron stain shows numerous periportal PGBs with blue‐green staining. (Original magnifications E: ×100; inset: ×400; F: ×400; G: ×200). [Modified and reprinted with permission by the publisher from ().]
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Pavel Strnad, Renwar Nuraldeen, Nurdan Guldiken, Daniel Hartmann, Vineet Mahajan, Helmut Denk, Johannes Haybaeck. Broad Spectrum of Hepatocyte Inclusions in Humans, Animals, and Experimental Models. Compr Physiol 2013, 3: 1393-1436. doi: 10.1002/cphy.c120032