{"id":6545,"date":"2026-05-07T07:18:33","date_gmt":"2026-05-07T07:18:33","guid":{"rendered":"http:\/\/biodigestor.net\/?p=6545"},"modified":"2026-05-07T07:18:33","modified_gmt":"2026-05-07T07:18:33","slug":"six-p-group-cases-also-received-allogeneic-hematopoietic-cell-transplant-ahct-of-which-one-died-of-disease-and-one-died-from-ahct-related-complications","status":"publish","type":"post","link":"https:\/\/biodigestor.net\/?p=6545","title":{"rendered":"\ufeffSix P-group cases also received allogeneic hematopoietic cell transplant (AHCT), of which one died of disease and one died from AHCT related complications"},"content":{"rendered":"<p>\ufeffSix P-group cases also received allogeneic hematopoietic cell transplant (AHCT), of which one died of disease and one died from AHCT related complications. systemic EBV + T-cell lymphoproliferative disease of childhood. We also performed gene expression profiling in a subset of cases to identify markers that may be of prognostic relevance and validated our results using immunohistochemistry. == Results == The median age is 14.9 years and two of our 22 cases occurring in patients older than 30 years. Fifteen of 17 cases (88%) with adequate data were of T-cell origin. Eleven of 22 cases revealed polymorphic cellular infiltrate (P-group) while the rest showed monomorphic lymphoid infiltrate (M-group). We found a significant difference in survival between P-group vs M-group patients with median survival not yet reached in P-group, and 1 month in M-group (p = 0.0001), suggesting a role for morphology in predicting patient outcome. We also performed gene expression profiling in a subset of patients and compared the genes differentially expressed between P-group and M-group cases to identify markers of prognostic value. We identified cyclin E2 gene and protein to be differentially expressed between patients with good outcome (P-group, median expression 8%) E3 ligase Ligand 9 and poor outcome (M-group, median expression 42%) (p = 0.0005). In addition, the upregulation of cyclin E2 protein in M-group cases correlated with a higher <a href=\"http:\/\/www.ncbi.nlm.nih.gov\/entrez\/query.fcgi?db=gene&#038;cmd=Retrieve&#038;dopt=full_report&#038;list_uids=10413\">YAP1<\/a> Ki67 <a href=\"https:\/\/www.adooq.com\/e3-ligase-ligand-9.html\">E3 ligase Ligand 9<\/a> proliferation rate (Pearson correlation r = 0.73, p = 0.0006) detected by immunohistochemistry. High cyclin E2 expression was also significantly associated with shorter survival (p = 0.0002). == Conclusion == Our data suggests the potential role of monomorphic morphology, high cyclin E2 and Ki67 expression as adverse prognostic factors for TNKLPD. == Electronic supplementary material == The online version of this article (doi:10.1186\/s13023-014-0165-x) contains supplementary material, which is available to authorized users. Keywords:EBV-associated T\/NK lymphoproliferative disease, Molecular signature, Morphology, Cyclin E2, Proliferation, Prognosis == Background == The term EBV-associated T\/NK-cell lymphoproliferative disorders (TNKLPD) encompasses a spectrum of disease entities in non-immunocompromised patients characterised by an Epstein-Barr virus (EBV)-infected, cytotoxic T or NK-cell proliferation that is polyclonal or monoclonal. The systemic form of TNKLPD includes chronic active EBV infection of T\/NK-cell type (CAEBV), aggressive NK cell leukemia (ANKL) and systemic EBV-positive T-cell lymphoproliferative disease of childhood (STLPDC) [1-4]. TNKLPD is rare in the Western population and occurs with increased frequency in Asians, Native Americans from Central and South America and Mexico [1,2] Morphologically, it is characterized by a wide-ranging cytological appearance from reactive appearance to overt leukemia\/lymphoma [5-7]. The diagnosis of TNKLPD can be challenging because cytologic features may be deceptively benign despite the frequently aggressive clinical behaviour. Historically, the terminology and classification of TNKLPD have been problematic as this process has been described under a variety of terms including: fulminant EBV + T-cell lymphoproliferative disease (LPD) of childhood [8], fatal infectious mononucleosis; fulminant hemophagocytic syndrome in children in Taiwan [9], fatal EBV-associated hemophagocytic syndrome in Japan [10] and severe CAEBV [11]. However, recent efforts have already been made to obtain international consensus over the nomenclature and classification of TNKLPD which is today recognized that a lot of from the EBV-associated T\/NK LPD in immunocompetent kids and adults with systemic display participate in the same spectral range of disease, including STLPDC and CAEBV. A consensus conference was held on the Country wide Institute of Wellness (NIH) in 2008 and it had been recommended that the word CAEBV ought to be put on systemic LPDs that aren&#8217;t frank lymphomas which arise during principal an infection and persist for over six months. Alternatively, the word systemic EBV-positive T-cell LPD, as followed with the WHO classification, may be the chosen pathologic E3 ligase Ligand 9 designation over CAEBV for all those situations that are clonal with an intense clinical E3 ligase Ligand 9 training course and require intense treatment [12]. A clinicopathological categorization of TNKLPD was suggested by Ohshima et al. in 2008 [13]. Based on the proposal, EBV-associated T\/NK LPD in kids could be categorised into 4 groupings (A1,.<\/p>\n","protected":false},"excerpt":{"rendered":"<p>\ufeffSix P-group cases also received allogeneic hematopoietic cell transplant (AHCT), of which one died of disease and one died from AHCT related complications. systemic EBV + T-cell lymphoproliferative disease of childhood. We also performed gene expression profiling in a subset of cases to identify markers that may be of prognostic relevance and validated our results&hellip; <a class=\"more-link\" href=\"https:\/\/biodigestor.net\/?p=6545\">Continue reading <span class=\"screen-reader-text\">\ufeffSix P-group cases also received allogeneic hematopoietic cell transplant (AHCT), of which one died of disease and one died from AHCT related complications<\/span><\/a><\/p>\n","protected":false},"author":1,"featured_media":0,"comment_status":"closed","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[4492],"tags":[],"class_list":["post-6545","post","type-post","status-publish","format-standard","hentry","category-ca2-binding-protein-modulators","entry"],"_links":{"self":[{"href":"https:\/\/biodigestor.net\/index.php?rest_route=\/wp\/v2\/posts\/6545"}],"collection":[{"href":"https:\/\/biodigestor.net\/index.php?rest_route=\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/biodigestor.net\/index.php?rest_route=\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/biodigestor.net\/index.php?rest_route=\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/biodigestor.net\/index.php?rest_route=%2Fwp%2Fv2%2Fcomments&post=6545"}],"version-history":[{"count":1,"href":"https:\/\/biodigestor.net\/index.php?rest_route=\/wp\/v2\/posts\/6545\/revisions"}],"predecessor-version":[{"id":6546,"href":"https:\/\/biodigestor.net\/index.php?rest_route=\/wp\/v2\/posts\/6545\/revisions\/6546"}],"wp:attachment":[{"href":"https:\/\/biodigestor.net\/index.php?rest_route=%2Fwp%2Fv2%2Fmedia&parent=6545"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/biodigestor.net\/index.php?rest_route=%2Fwp%2Fv2%2Fcategories&post=6545"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/biodigestor.net\/index.php?rest_route=%2Fwp%2Fv2%2Ftags&post=6545"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}