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Lymphocyte homing and immunology of extranodal lymphoid tissues
Published in Franco Cavalli, Harald Stein, Emanuele Zucca, Extranodal Lymphomas, 2008
Mariagrazia Uguccioni, James J Campbell, Katrin Kuscher, Marshall E Kadin
Hepatosplenic T-cell lymphoma is distinguished by hepatosplenomegaly, minimal or no lymphadenopathy, frequent bone marrow involvement, and a distinctive pattern of intrasinusoidal infiltration of tumor cells. The tumor cells appear to be adjacent to endothelial cells within sinuses of the liver, spleen, and bone marrow.82 Although this tumor exemplifies a curious and distinctive pattern of lymphocyte trafficking, there are no published studies which have determined the molecules responsible for these phenomena.
Special considerations for biologic medications in pediatric ulcerative colitis
Published in Expert Opinion on Biological Therapy, 2020
Logan Jerger, Jeffrey S. Hyams
Considerable concern has been raised about the risk of lymphoproliferative disease with the use of thiopurines alone, or in combination with anti-TNFα therapy. In particular, a significantly aggressive and often fatal disorder, hepatosplenic T cell lymphoma, has been described in both pediatric and adult patients [54]. For this reason, most clinicians caring for children with IBD do not use combination therapy with thiopurines and anti-TNF agents. Based on clinical guidelines, the grading of recommendations for the use of thiopurines in adults as compared to children has stronger recommendations (Grade B for adults compared to Grade C for pediatrics) based on higher levels of studies conducted with adult populations [55]. This recommendation and evidence likely impacts the use of these medications in the adult population and the caution of using these in pediatric patients.
An unusual presentation of an uncommon lymphoma, hepatosplenic T-cell lymphoma
Published in Baylor University Medical Center Proceedings, 2019
Sima Amin, Sarah K. Findeis, Andrew Whiteley, John R. Krause
Hepatosplenic T-cell lymphoma (HSTCL) is a rare T-cell lymphoma, primarily characterized by extranodal distribution of the malignant cells with intrasinusoidal infiltration of the liver, spleen, and bone marrow. It most commonly occurs in adults with a median age of 34 years and B symptoms of fever, fatigue, weight loss, abdominal pain, splenomegaly, and jaundice.1 The disease has been associated with immunosuppressed states, including patients with solid organ transplants as well as patients taking tumor necrosis factor blockers or thiopruine.1,2 It is hypothesized that T cells in these patients are subject to increased antigenic stimulation because of decreased pathogen clearance. Antigenic stimulation acts as a promoter of increased cell proliferation, facilitating mutations. Most cases of HSTCL express γδ T-cell receptor (TCR); however, rare cases of αβ TCR expression have been described. HSTCL has a poor prognosis, with a 5-year overall survival rate of 7%.1 We describe a unique case of HSTCL in a patient with a primarily indolent course.
Optimization of biologic therapy in Crohn’s disease
Published in Expert Opinion on Biological Therapy, 2018
Potential side effects of escalating biologic dose or employing combination therapy also need to be carefully weighed. Males less than 40 years of age have a risk of developing hepatosplenic T-cell lymphoma on combination therapy after greater than 12 months [58]. While exceedingly rare, this risk is not seen for other populations. Combination therapy should also be used with caution in elderly patients, given risk for drug reactions and infections [59]. Pushing the biologic dose may also give rise to adverse effects, though higher doses of anti-TNF have not been shown to increase the risk for infection [60]. While anti-TNF agents such as IFX have been linked with the development of psoriasiform lesions [61], there does not seem to be a correlation between higher serum anti-TNF levels and development of psoriasiform lesions [60]. Vedolizumab has been noted to have less concern for infections and malignancy compared to other biologics, and this may be a better choice for elderly patients or patients with significant cardiopulmonary comorbidities. Ustekinumab also appears to have a favorable safety profile. Notably, there is currently a lack of long-term safety data on vedolizumab and ustekinumab compared to the more well-established anti-TNF agents.