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https://read.qxmd.com/read/30782610/viral-immunologic-and-clinical-features-of-primary-effusion-lymphoma
#1
Kathryn Lurain, Mark N Polizzotto, Karen Aleman, Manisha Bhutani, Kathleen M Wyvill, Priscila H Gonçalves, Ramya Ramaswami, Vickie Ann Marshall, Wendell Miley, Seth M Steinberg, Richard F Little, Wyndham Wilson, Armando C Filie, Stefania Pittaluga, Elaine S Jaffe, Denise Whitby, Robert Yarchoan, Thomas S Uldrick
Primary effusion lymphoma (PEL) is an aggressive HIV-associated lymphoma with a relatively poor prognosis in the era of effective HIV therapy. Kaposi sarcoma herpesvirus (KSHV) is the etiologic agent, and approximately 80% of tumors are Epstein Barr virus (EBV) co-infected. A better understanding of how KSHV-related immune dysregulation contributes to the natural history of PEL will improve outcomes. We identified 20 PEL patients diagnosed 2000-2013, including 19 treated with modified infusional etoposide, vincristine, and doxorubicin with cyclophosphamide and prednisone (EPOCH) and compared their clinical, virologic, and immunologic features with 20 patients with HIV-associated diffuse large B-cell lymphoma (HIV-DLBCL) and 19 patients with symptomatic interleukin (IL)-6 related KSHV-associated multicentric Castleman disease (KSHV-MCD)...
February 19, 2019: Blood
https://read.qxmd.com/read/30779786/human-ifit-proteins-inhibit-lytic-replication-of-kshv-a-new-feed-forward-loop-in-the-innate-immune-system
#2
Dajiang Li, Sankar Swaminathan
Kaposi's sarcoma-associated herpesvirus (KSHV) is causally associated with Kaposi's sarcoma, primary effusion lymphoma (PEL) and multicentric Castleman's disease. The IFIT family of proteins inhibits replication of some viruses, but their effects on KSHV lytic replication was unknown. Here we show that KSHV lytic replication induces IFIT expression in epithelial cells. Depletion of IFIT1, IFIT2 and IFIT3 (IFITs) increased infectious KSHV virion production 25-32-fold compared to that in control cells. KSHV lytic gene expression was upregulated broadly with preferential activation of several genes involved in lytic viral replication...
February 19, 2019: PLoS Pathogens
https://read.qxmd.com/read/30774824/tafro-syndrome-a-case-report-from-turkey-and-review-of-the-literature
#3
Zeki Islamoğlu, Ali Erkan Duman, Göktuğ Sirin, Hasan Yılmaz, Meral Uluköylü Mengüç, Yiğit Erçetin, Süheyla Bozkurt, Sadettin Hülagü, Altay Çelebi
TAFRO syndrome is a rare subtype of the Castleman's disease which has been described over the last years. The name of TAFRO syndrome comes from thrombocytopenia, anasarca, myelofibrosis, renal dysfunction, and organomegaly. We report a young Turkish male patient presented with fever, night sweats, fatigue, nausea, bilateral pretibial pitting edema, abdominal pain and watery diarrhea. PET/CT revealed multiple lymphadenopathies in cervical, axillary, mediastinal, paraaortic, mesenteric and inguinal lymph nodes...
October 1, 2018: International Journal of Hematology-oncology and Stem Cell Research
https://read.qxmd.com/read/30770900/latent-infection-with-kaposi-s-sarcoma-associated-herpesvirus-enhances-retrotransposition-of-long-interspersed-element-1
#4
Ryota Nakayama, Yumiko Ueno, Keiji Ueda, Tomoyuki Honda
Kaposi's sarcoma (KS)-associated herpesvirus (KSHV), a gamma-2 herpesvirus, is the causative agent of KS, primary effusion lymphoma (PEL), and a plasma cell variant of multicentric Castleman's disease. Although KSHV latency is detected in KS-related tumors, oncogenic pathways activated by KSHV latent infection are not fully understood. Here, we found that retrotransposition of long interspersed element-1 (L1), a retrotransposon in the human genome, was enhanced in PEL cells. Among the KSHV latent genes, viral FLICE-inhibitory protein (vFLIP) enhanced L1 retrotransposition in an NF-κB-dependent manner...
February 15, 2019: Oncogene
https://read.qxmd.com/read/30764698/letter-to-the-editor-regarding-non-cirrhotic-portal-hypertension-associated-with-multicentric-castleman-s-disease-a-case-report
#5
Dale M Kobrin, Ana Luisa Pinto, Sophia T Parente, Marilia Gomes, Maria Augusta Cipriano, Maria Leticia Ribeiro, David C Fajgenbaum
No abstract text is available yet for this article.
February 15, 2019: Acta Oncologica
https://read.qxmd.com/read/30760451/phase-2-study-using-oral-thalidomide-cyclophosphamide-prednisone-for-idiopathic-multicentric-castleman-disease
#6
Lu Zhang, Ai-Lin Zhao, Ming-Hui Duan, Zhi-Yuan Li, Xin-Xin Cao, Jun Feng, Dao-Bin Zhou, Ding-Rong Zhong, David C Fajgenbaum, Jian Li
Idiopathic multicentric Castleman disease (iMCD) is a rare lymphoproliferative disorder. The anti-interleukin(IL)-6 therapy siltuximab is not available everywhere, and is not effective for over half of patients. Alternative treatment approaches are urgently needed. In the first iMCD clinical trial directed against a target other than IL-6 signaling, we investigated thalidomide-cyclophosphamide -prednisone (TCP) regimen in newly diagnosed iMCD patients. This single-center, single-arm, phase 2 study enrolled 25 newly diagnosed iMCD patients between June 2015 and June 2018 (registered on ClinicalTrials...
February 13, 2019: Blood
https://read.qxmd.com/read/30746379/castleman-disease-presenting-with-jaundice-a-case-report-and-review-of-literature
#7
Bo Zhai, Hai-Yang Ren, Wei-Dong Li, Shiva Reddy, Shu-Jun Zhang, Xue-Ying Sun
BACKGROUND: Castleman disease (CD) is a rare lymphoproliferative disorder that presents with various symptoms. CD accompanied with jaundice is uncommon since there are only 11 cases reported in the literature. CASE SUMMARY: Here we report a 62-year-old woman who was admitted to the hospital with signs and symptoms of intermittent jaundice. Biochemical tests showed higher serum levels of total and direct bilirubin, and normal serum levels of tumor markers and interleukin-6...
February 6, 2019: World Journal of Clinical Cases
https://read.qxmd.com/read/30718654/gper-activation-protects-against-epithelial-barrier-disruption-by-staphylococcus-aureus-%C3%AE-toxin
#8
Kathleen D Triplett, Srijana Pokhrel, Moriah J Castleman, Seth M Daly, Bradley O Elmore, Jason A Joyner, Geetanjali Sharma, Guy Herbert, Matthew J Campen, Helen J Hathaway, Eric R Prossnitz, Pamela R Hall
Sex bias in innate defense against Staphylococcus aureus skin and soft tissue infection (SSTI) is dependent on both estrogen production by the host and S. aureus secretion of the virulence factor, α-hemolysin (Hla). The impact of estrogen signaling on the immune system is most often studied in terms of the nuclear estrogen receptors ERα and ERβ. However, the potential contribution of the G protein-coupled estrogen receptor (GPER) to innate defense against infectious disease, particularly with respect to skin infection, has not been addressed...
February 4, 2019: Scientific Reports
https://read.qxmd.com/read/30705099/igg4-related-disease-what-a-hematologist-needs-to-know
#9
Luke Y C Chen, Andre Mattman, Michael A Seidman, Mollie N Carruthers
IgG4-related disease is a fibroinflammatory condition that can affect nearly any organ system. Common presentations include major salivary and lacrimal gland enlargement, orbital disease, autoimmune pancreatitis, retroperitoneal fibrosis and tubulointerstitial nephritis. This review focuses on the hematological manifestations of IgG4-related disease, including lymphadenopathy, eosinophilia, and polyclonal hypergammaglobulinemia. The disease can easily be missed by unsuspecting hematologists, as patients may present with clinical problems that mimic disorders such as multicentric Castleman disease, lymphoma, plasma cell neoplasms and hypereosinophilic syndromes...
January 31, 2019: Haematologica
https://read.qxmd.com/read/30680887/castleman-disease-in-pediatrics-insights-on-presentation-treatment-and-outcomes-from-a-two-site-retrospective-cohort-study
#10
Jenna Sopfe, Ashley Endres, Kristen Campbell, Kari Hayes, Andrew T Trout, Xiayuan Liang, Robert Lorsbach, Maureen M O'Brien, Carrye R Cost
BACKGROUND: Castleman disease (CD) is an uncommon lymphoproliferative disorder that is rare in pediatric populations; the literature describing this population is sparse. We sought to describe pediatric CD, including unicentric CD (UCD) and human herpes virus-8 (HHV8)-negative multicentric CD (MCD), in a multi-institutional cohort. METHODS: We retrospectively reviewed 24 patients, aged 0 to 26 years at diagnosis, who were diagnosed with CD between January 1, 2005, and May 16, 2017, at two tertiary children's hospitals...
January 24, 2019: Pediatric Blood & Cancer
https://read.qxmd.com/read/30680214/human-immunodeficiency-virus-negative-multicentric-castleman-s-disease-coexistent-with-kaposi-s-sarcoma-on-18-f-fdg-pet-ct-a-case-report
#11
Jae Pil Hwang, Jiyoon Kim, Jung Mi Park
Multicentric Castleman's disease and Kaposi's sarcoma are more frequently observed in human immunodeficiency virus (HIV)-infected patients; however, 40-50% of the cases are HIV-negative. The present study reports the case of a 61-year-old man who presented with palpable masses in the axillary and right inguinal areas. The blood test results revealed increased serum erythrocyte sedimentation rate and C-reactive protein level, with negative serological markers, including for HIV. The patient was investigated using contrast-enhanced computed tomography (CT) and fluorine- 18 fluorodeoxyglucose positron emission tomography (18 F-FDG PET)/CT; the images revealed multiple enlarged and intensely hypermetabolic lymph nodes in the cervical, thoracic and abdominopelvic areas...
February 2019: Molecular and Clinical Oncology
https://read.qxmd.com/read/30646936/mediastinal-follicular-dendritic-cell-sarcoma-a-rare-potentially-under-recognized-and-often-misdiagnosed-disease
#12
You-Li Wu, Feng Wu, Cheng-Ping Xu, Guo-Lei Chen, Yu Zhang, Wei Chen, Xiao-Chu Yan, Guang-Jie Duan
BACKGROUND: Mediastinal follicular dendritic cell sarcoma (FDCS) is extremely rare. Due to potential under-recognization of this disease, it happens to be misdiagnosed, especially on core needle biopsy. We report 3 cases of mediastinal FDCS and provide a literature review to improve better understanding of the tumor and to reduce misdiagnosis. METHODS: Three cases of mediastinal FDCS in our clinic practice were studied, including their core needle biopsy and resected specimens, and those cases reported previously in English literature were retrieved and analyzed...
January 15, 2019: Diagnostic Pathology
https://read.qxmd.com/read/30642741/an-extranodal-histopathological-analysis-of-idiopathic-multicentric-castleman-disease-with-and-without-tafro-syndrome
#13
REVIEW
Nozomu Kurose, Ken-Ichi Mizutani, Motona Kumagai, Akihiro Shioya, Xin Guo, Satoko Nakada, Shino Fujimoto, Hiroshi Kawabata, Yasufumi Masaki, Kazue Takai, Sadao Aoki, Masaru Kojima, Shigeo Nakamura, Masatoshi Kida, Sohsuke Yamada
Thrombocytopenia, anasarca, fever, renal failure or reticulin fibrosis, and organomegaly (TAFRO) syndrome, a poor prognostic clinical condition showing similar histopathological findings to idiopathic multicentric Castleman disease (iMCD), has been reported in Japan. In our previous report, a clinicopathological analysis was performed on 70 nodal cases of iMCD with/without TAFRO. iMCD is classified into three types based on histopathology: (i) plasmacytic (PC), (ii) mixed, and (iii) hypervascular (hyperV). In this report, extranodal histopathological changes of iMCD with/without TAFRO were analyzed...
December 26, 2018: Pathology, Research and Practice
https://read.qxmd.com/read/30637803/castleman-s-disease-presenting-as-a-mediastinal-mass-with-recurrent-pericardial-effusions
#14
Hongduan Liu, Xiaojun Xie, Ming-Bin Deng, Fengxu Yu, Cuiwei Zhang
No abstract text is available yet for this article.
January 14, 2019: Journal of Cardiac Surgery
https://read.qxmd.com/read/30632181/tufted-angioma-like-lesion-associated-with-vegf-and-il-6-in-tafro-syndrome-is-it-a-common-histological-feature-of-multicentric-castleman-disease-poems-syndrome
#15
Kumi Fujita, Kazuhiro Hatta
The histology of skin lesions of TAFRO (thrombocytopenia, anasarca, reticulin fibrosis/renal failure, and organomegaly) syndrome has rarely been reported. We report herein 2 cases of TAFRO syndrome with characteristic vascular skin lesions. The lesions resembled a tufted angioma (TA), although those of case 1 partially resembled a glomeruloid hemangioma, which was known as a specific lesion in POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes), a variant of multicentric Castleman disease (MCD)...
January 10, 2019: Journal of Cutaneous Pathology
https://read.qxmd.com/read/30606932/-replication-machinery-of-kaposi-s-sarcoma-associated-herpesvirus-and-drug-discovery-research
#16
Tadashi Watanabe, Masahiro Fujimuro
Kaposi's sarcoma-associated herpesvirus (KSHV) is the causative agent of Kaposi's sarcoma (KS), primary effusion lymphoma (PEL), and Castleman's disease. While liposomal doxorubicin has been used as an effective treatment for KS patients, the cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) regimen used for PEL patients was reported to have 1-year survival rates of less than 40%. Moreover, the development of anti-KSHV drugs inhibiting viral replication has been delayed. KSHV establishes a lifelong infection in its host and alternates between a "latent infection" and "lytic infection" state...
2019: Yakugaku Zasshi: Journal of the Pharmaceutical Society of Japan
https://read.qxmd.com/read/30598916/unicentric-castleman-s-disease-as-a-localized-retroperitoneal-mass-a-case-report-and-review-of-literature
#17
Praveen Gopi, Vasudevan Sambu Potty, Rustam Singh Kaurav, Krishna Govindan
Castleman's disease (CD) is a rare disorder characterized by proliferation of the lymphoid tissue. Clinically, it presents in two forms either a unicentric (UC) or multicentric. Mediastinum is the most common location. UC retroperitoneal presentation is rare. We report a case of 29-year-old female who presented with left loin pain, and on abdominal imaging, evaluation identified a retroperitoneal mass in the left hypochondrium in the pararenal space. Mass was surgically excised entirely. Histopathological examination demonstrated hyaline vascular type of CD...
October 2018: International Journal of Applied and Basic Medical Research
https://read.qxmd.com/read/30596615/synchronous-unicentric-castleman-disease-and-inflammatory-hepatocellular-adenoma-a-case-report
#18
Claudio De Vito, Thomas G Papathomas, Federica Pedica, Kane Pauline, Amir Ali, Nigel Heaton, Alberto Quaglia
Systemic symptoms such as fever and fatigue are non-specific manifestations spanning from inflammation to neoplasia. Here we report the case of a 34 year-old man who presented with systemic symptoms for four months. CT-scan and MRI revealed a 3.4 cm arterialized hepatic lesion and a 7 cm paraduodenal mass. Surgical resection of both lesions and histological examination revealed an inflammatory hepatocellular adenoma and a unicentric plasma cell type of Castleman disease. Moreover, a diffuse AA amyloid deposition in the liver was observed...
December 13, 2018: Annals of Hepatology
https://read.qxmd.com/read/30592111/castleman-s-disease-a-rare-cause-of-an-axillary-mass-mimicking-breast-metastasis
#19
Benazir Barna, Shariq Sabri, Abhishek Chitnis, Pardeep Arora, Roger Hunt
No abstract text is available yet for this article.
January 2019: Breast Journal
https://read.qxmd.com/read/30586033/radiologic-and-histopathologic-features-in-an-intracranial-localized-castleman-disease-a-case-report-and-review-of-literature
#20
REVIEW
J Bernardo Escribano Paredes, Rodrigo Carrasco Moro, María López Gutiérrez, Héctor Pian Arias, Mónica García-Cosío, Sebastián García Madrona, Velina Nedkova Hristova, Javier Martínez Poles, Javier Buisán Catevilla Francisco
INTRODUCTION: Castleman disease (CD) is a rare pathologic process of unknown etiology, characterized by non-neoplastic lymph node enlargement. Two distinct histologic patterns are recognized; the hyaline-vascular type and the less common plasma cells type. Another intermediate type has been described. The clinical features are classified into 2 categories, localized (unicentric) and generalized (multicentric), the later associated with systemic manifestations and poor prognosis. CD affecting the central nervous system is extremely rare...
January 2019: Neurologist
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