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Effects of treatment on the abdomen and pelvis
Published in Anju Sahdev, Sarah J. Vinnicombe, Husband & Reznek's Imaging in Oncology, 2020
Patients with liver metastases from breast cancer may rarely develop a striking ‘pseudocirrhosis’ appearance following chemotherapy (Figure 39.5). The CT features are capsular retraction of the liver surface at the site of previous metastases, resulting in a lobular hepatic contour, segmental volume loss, and enlargement of the caudate lobe. Ascites and splenomegaly may also be present, as in true cirrhosis. Pathologically, there is nodular regenerative hyperplasia and absence of cirrhosis (21). It is important to recognize this pattern as a complication of treatment as opposed to true cirrhosis.
Mucosal manifestations of immunodeficiencies
Published in Phillip D. Smith, Richard S. Blumberg, Thomas T. MacDonald, Principles of Mucosal Immunology, 2020
Scott Snapper, Jodie Ouahed, Luigi D. Notarangelo
Finally, persistent elevation of liver enzymes can be seen in more than 50% of CVID patients. Most patients are found to have cholestasis without jaundice and, when liver biopsies have been available, have been found to exhibit a nodular regenerative hyperplasia. Nodular regenerative hyperplasia is characterized by the presence of regenerating liver cell plates alternating with atrophic areas with no significant portal fibrosis, bridging fibrosis, or definite cirrhosis. Noncaseating granulomas are often an associated feature. Portal vein endotheliitis, intrasinusoidal inflammation with prominent T-cell infiltration, and portal hypertension in the absence of cirrhosis are additional common features. The presence of nodular regenerative hyperplasia has been correlated with other autoimmune phenomena, and it has been suggested that these liver modifications may represent the consequence of chronic gastrointestinal inflammation with excessive translocation of luminal antigens into the liver. Severe liver disease has been reported in the past in CVID patients who had acquired hepatitis C virus (HCV) infection through contaminated blood products (including immunoglobulin preparations). While this risk has been virtually eliminated with improvement in screening of blood products and the preparation of immunoglobulins, it appears that progression of liver disease is faster and more severe in patients with CVID who are infected with hepatotropic viruses.
Miscellaneous
Published in Joseph Kovi, Hung Dinh Duong, Frozen Section In Surgical Pathology: An Atlas, 2019
The parenchymal nodule resembles cirrhosis, particularly congestive or cardiac cirrhosis. Nodular regenerative hyperplasia can be differentiated from cirrhosis by the absence of dense collagen fibers around the nodule (Figure 151).
Long-term clinical outcome after thiopurine discontinuation in elderly IBD patients
Published in Scandinavian Journal of Gastroenterology, 2021
C. Jorissen, B. Verstockt, N. Schils, J. Sabino, M. Ferrante, S. Vermeire
Inflammatory bowel diseases (IBD), Crohn’s disease (CD), and ulcerative colitis (UC) are chronic relapsing digestive diseases requiring chronic anti-inflammatory therapy in the majority of patients to maintain remission and prevent relapses. Treatment with immunosuppressive and/or biological agents is the cornerstone for maintaining remission. Although several anti-cytokine and anti-integrin therapies entered the therapeutic landscape, thiopurines (TP), including azathioprine and its metabolites mercaptopurine and thioguanine, are still very frequently used to maintain disease control [1–3]. In a recent nationwide Danish cohort study, 15% of all the IBD patients were treated with thiopurines (1999–2014) [4]. Nevertheless, thiopurines are associated with substantial safety issues including risk of opportunistic infections and malignancies, particularly with prolonged use and in patients over the age of 60. Mainly the risk of lymphoma, but also non-melanoma skin cancer and nodular regenerative hyperplasia of the liver are well-reported. Especially the lymphoma risk is age-related and is increased 5-fold above the age of 65 [5]. Given the aging IBD population, discontinuation of thiopurines in IBD patients above 60–65 is an important clinical discussion. Indeed, stopping therapy may trigger relapses and therefore, in a patient in clinical and/or endoscopic remission, needs to be balanced against the safety risk [6–8]. To date, optimal timing for withdrawal and the outcome after stopping thiopurines remains unclear and prevents clear guidelines for physicians [6,9].
Keeping it in the family: the case for considering late-onset combined immunodeficiency a subset of common variable immunodeficiency disorders
Published in Expert Review of Clinical Immunology, 2018
Rohan Ameratunga, Yeri Ahn, Anthony Jordan, Klaus Lehnert, Shannon Brothers, See-Tarn Woon
In her early forties, she developed ascites and portal hypertension consequent to nodular regenerative hyperplasia of the liver. She has also experienced diarrhea caused by mild nonspecific colitis, not requiring treatment. Her CRP was 1 mg/l at the time. She had profoundly reduced CD4+ T cells on three separate occasions (Table 1). Her CD4+ counts were 138 and 204 (500–1650 × 106/l) and she had impaired in vitro lymphocyte proliferation (Table 2), prior to developing malignancy and subsequent chlorambucil treatment. There was further reduction in her CD4+ count (Table 1) following chemotherapy. She has been on Pneumocystis jiroveci prophylaxis. She appears to best fit both the revised ESID registry criteria for impaired T cell function as well as the DEFI criteria for LOCID. Sadly, she recently passed away from Escherichia coli sepsis.
Non-cirrhotic portal hypertension associated with multicentric Castleman’s disease: a case report
Published in Acta Oncologica, 2018
Ana Luísa Pinto, Marília Gomes, Maria Augusta Cipriano, Maria Letícia Ribeiro
Idiopathic multicentric Castleman’s disease (iMCD) is a rare polyclonal lymphoproliferative disorder. It is characterized by systemic inflammatory symptoms, cytopenias and multi-organ dysfunction caused by proinflammatory cytokines [1]. Even though hepatosplenomegaly and ascites are frequently associated with iMCD [1], portal hypertension appears to be infrequent [2]. Nodular regenerative hyperplasia (NRH) of the liver is also an unusual condition, which is characterized by a generalized benign transformation of the hepatic parenchyma into small regenerative nodules, with minimal or absent perisinusoidal or periportal fibrosis [3]. NRH is known to be associated with autoimmune, infectious, neoplastic, toxic and hematological conditions. It is usually asymptomatic, unless (non-cirrhotic) portal hypertension develops – estimated in 50% of the cases [3]. NRH in iMCD patients has only seldom been reported [4,5]. We present a case of iMCD with portal hypertension and pathological features of NRH.