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Castlemans disease

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https://www.readbyqxmd.com/read/28299525/poems-syndrome-an-enigma
#1
REVIEW
Rahma Warsame, Uday Yanamandra, Prashant Kapoor
POEMS syndrome is a paraneoplastic disorder secondary to an underlying plasma cell dyscrasia. By definition, all patients with POEMS syndrome must display polyneuropathy and monoclonal plasma cell disorder. In addition, at least one major criterion (Castleman's disease, sclerotic bone lesions, or vascular endothelial growth factor elevation) and one minor criterion (organomegaly, extravascular volume overload, endocrinopathy, skin changes, papilledema, thrombocytosis, or polycythemia) are required for diagnosis...
March 15, 2017: Current Hematologic Malignancy Reports
https://www.readbyqxmd.com/read/28291506/immunotactoid-glomerulopathy-leading-to-the-discovery-of-poems-syndrome%C3%A2
#2
Carole Philipponnet, Jean-Louis Kemeny, Cyril Garrouste, Martin Soubrier, Anne-Elisabeth Heng
Monoclonal gammopathy of renal significance (MGRS) can manifest in many different ways depending on the nature of the immunoglobulin and its physicochemical properties. MGRS can lead to the discovery of a hematological malignancy. We report the case of a 32-year-old female patient who underwent renal biopsy on account of an impure nephrotic syndrome associated with immunoglobulin (Ig)G κ monoclonal gammopathy. Histological analysis revealed membranoproliferative glomerulonephritis with IgG, IgM, κ, λ, and C3 deposits...
March 14, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28284028/modulation-of-oncogenic-signaling-networks-by-kaposi-s-sarcoma-associated-herpesvirus
#3
Jason P Wong, Blossom Damania
Kaposi's sarcoma-associated herpesvirus (KSHV) is the etiological agent of three human malignancies Kaposi's sarcoma, primary effusion lymphoma, and multicentric Castleman's disease. To persist and replicate within host cells, KSHV encodes proteins that modulate different signaling pathways. Manipulation of cell survival and proliferative networks by KSHV can promote the development of KSHV-associated malignancies. In this review, we discuss recent updates on KSHV pathogenesis and the viral life cycle. We focus on proteins encoded by KSHV that modulate the phosphatidylinositol-4,5-bisphosphate 3 kinase and extracellular signal-regulated kinases 1/2 pathways to create an environment favorable for viral replication and the development of KSHV malignancies...
March 11, 2017: Biological Chemistry
https://www.readbyqxmd.com/read/28280640/hhv8-ebv-coinfection-lymphoproliferative-disorder-rare-entity-with-a-favorable-outcome
#4
Dhouha Bacha, Beya Chelly, Houda Kilani, Lamia Charfi, Amel Douggaz, Samia Chatti, Emna Chelbi
HHV8/EBV-associated germinotropic lymphoproliferative disorder (GLD) is a challenging diagnosis given its rarity, the particular clinical presentation, and the lack of expression of markers usually used in establishing hematopoietic lineage. We report a new case of HHV8/EBV GLD in an immunocompetent 78-year-old woman. The diagnosis was made in an incidentally discovered lymphadenopathy. Histological examination showed a nodular lymphoid proliferation centered by aggregates of atypical plasmablastic cells admixed with small lymphoid cells...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28274535/two-patients-with-tafro-syndrome-exhibiting-strikingly-similar-anterior-mediastinal-lesions-with-predominantly-fat-attenuation-on-chest-computed-tomography
#5
Yoko Ozawa, Hiroshi Yamamoto, Masanori Yasuo, Hidekazu Takahashi, Kazunari Tateishi, Atsuhito Ushiki, Satoshi Kawakami, Yasunari Fujinaga, Shiho Asaka, Kenji Sano, Hiroshi Takayama, Hiroshi Imamura, Masayuki Hanaoka
We herein report on two middle-aged men with TAFRO (thrombocytopenia, anasarca, fever, reticulin fibrosis or renal failure, and organomegaly) syndrome, a unique clinicopathological variant of multicentric Castleman׳s disease recently proposed in Japan. Strikingly similar anterior mediastinal fat swellings with soft tissue density were observed in the patients on chest computed tomography. In TAFRO syndrome, bilateral pleural effusion and slight lymph node swelling are common in the thoracic region; however, anterior mediastinal lesions have not been previously observed...
March 2017: Respiratory Investigation
https://www.readbyqxmd.com/read/28248818/kshv-associated-and-ebv-associated-germinotropic-lymphoproliferative-disorder-new-findings-and-review-of-the-literature
#6
Tapan Bhavsar, John C Lee, Yvonne Perner, Mark Raffeld, Liqiang Xi, Stefania Pittaluga, Elaine S Jaffe
We report 2 cases of Kaposi sarcoma-associated herpesvirus (KSHV)-and Epstein-Barr Virus (EBV) associated germinotropic lymphoproliferative disorder. Both cases arose in patients from regions endemic for KSHV, Cape Verde, and the Democratic Republic of the Congo, presenting as localized lymphadenopathy. The affected lymph nodes showed colonization of the follicles by clusters of large atypical plasmablasts, but also showed regressive changes with vascular proliferation and interfollicular plasmacytosis, both reminiscent of human herpesvirus 8 (HHV-8) positive multicentric Castleman disease...
February 28, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28241212/developmental-therapeutics-implications-of-next-generation-sequencing-in-human-herpesvirus-8-negative-castleman-disease
#7
Xiaolong Alan Zhou, Young Kwang Chae, Francis Joseph Giles
No abstract text is available yet for this article.
February 22, 2017: JAMA Dermatology
https://www.readbyqxmd.com/read/28241173/jak1-genomic-alteration-associated-with-exceptional-response-to-siltuximab-in-cutaneous-castleman-disease
#8
Maulik Patel, Sadakatsu Ikeda, Susan R Pilat, Razelle Kurzrock
Importance: Castleman disease (CD) is an ultrarare, interleukin-6 (IL-6)-driven lymphoproliferative disorder whose underlying molecular alterations are unknown. Siltuximab (anti-IL-6 antibody) is approved for treatment of this disease. To our knowledge, genomic sequencing of CD has not been reported. Objective: To investigate and identify molecular aberration(s) that help explain the exceptional response to siltuximab in a patient with cutaneous CD. Design, Setting, and Participants: This case study examines data from comprehensive genomic profiling (using targeted next-generation sequencing) of tissue from a patient with cutaneous CD who demonstrated an exceptional response to siltuximab treated at a National Cancer Institute-designated Comprehensive Cancer Center...
February 22, 2017: JAMA Dermatology
https://www.readbyqxmd.com/read/28222449/characterization-of-cutaneous-plasmacytosis-at-different-disease-stages
#9
Pa-Fan Hsiao, Yu-Hung Wu
BACKGROUND/AIMS: Cutaneous plasmacytosis is rare and still not well understood. A retrospective study was made of 9 Chinese patients with 1- to 15-year histories of biopsy-proven cutaneous plasmacytosis diagnosed between 2003 and 2015. METHODS: Patient records and archival photographs helped establish the pattern and duration of skin lesions, and skin biopsy specimens provided additional data. RESULTS: The mean age at diagnosis was 46.4 years...
February 22, 2017: Dermatology: International Journal for Clinical and Investigative Dermatology
https://www.readbyqxmd.com/read/28207938/comparison-of-clinical-and-pathological-features-of-lung-lesions-of-systemic-igg4-related-disease-and-idiopathic-multicentric-castleman-s-disease
#10
Yasuhiro Terasaki, Shinichro Ikushima, Shoko Matsui, Akira Hebisawa, Yasunori Ichimura, Shinyu Izumi, Masuo Ujita, Machiko Arita, Keisuke Tomii, Youko Komase, Isoko Owan, Tetsuji Kawamura, Yasuo Matsuzawa, Miho Murakami, Hiroshi Ishimoto, Hiroshi Kimura, Masashi Bando, Norihiro Nishimoto, Yoshinori Kawabata, Yuh Fukuda, Takashi Ogura
AIMS: The lung lesion (IgG4-L) of IgG4-related disease (IgG4-RD) is a condition that occurs together with IgG4-RD and often mimics the lung lesion (iMCD-L) of idiopathic multicentric Castleman's disease (iMCD). Because no clinical and pathological studies had previously compared features of these diseases, we undertook this comparison with clinical and histological data. METHODS AND RESULTS: Nine patients had IgG4-L (high levels of serum IgG4 and of IgG4(+) cells in lung specimens; typical extrapulmonary manifestations)...
February 16, 2017: Histopathology
https://www.readbyqxmd.com/read/28205564/elevated-serum-interferon-%C3%AE-induced-protein-10-kda-is-associated-with-tafro-syndrome
#11
Noriko Iwaki, Yuka Gion, Eisei Kondo, Mitsuhiro Kawano, Taro Masunari, Hiroshi Moro, Koji Nikkuni, Kazue Takai, Masao Hagihara, Yuko Hashimoto, Kenji Yokota, Masataka Okamoto, Shinji Nakao, Tadashi Yoshino, Yasuharu Sato
Multicentric Castleman disease (MCD) is a heterogeneous lymphoproliferative disorder. It is characterized by inflammatory symptoms, and interleukin (IL)-6 contributes to the disease pathogenesis. Human herpesvirus 8 (HHV-8) often drives hypercytokinemia in MCD, although the etiology of HHV-8-negative MCD is idiopathic (iMCD). A distinct subtype of iMCD that shares a constellation of clinical features including thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis (R), and organomegaly (O) has been reported as TAFRO-iMCD, however the differences in cytokine profiles between TAFRO-iMCD and iMCD have not been established...
February 13, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28197347/hodgkin-lymphoma-and-castleman-disease-when-one-blood-disease-can-hide-another
#12
L Filliatre-Clement, H Busby-Venner, C Moulin, G Roth-Guepin, A Perrot
We describe a rare case of Castleman disease associated de novo with Hodgkin lymphoma. The incidence of Castleman disease is rare; only a few studies have described it in de novo association with Hodgkin lymphoma. The patient described here complained of unique evolutionary axillary adenopathy. A positron-emission tomography/computed tomography scan revealed hypermetabolic activity in this area. Diagnosis was based on a total excision biopsy of the adenopathy. The patient underwent complete remission with ABVD (doxorubicin, bleomycin, vinblastine, and dacarbazine) chemotherapy for treating Hodgkin lymphoma after surgical excision of the unicentric Castleman disease lesion...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28196203/castleman-disease-and-retinal-vascular-tortuosity
#13
Andre Aung, Mathew W MacCumber
No abstract text is available yet for this article.
February 9, 2017: JAMA Ophthalmology
https://www.readbyqxmd.com/read/28191314/diagnosis-and-treatment-of-cd20-negative-b-cell-lymphomas
#14
REVIEW
Tasleem Katchi, Delong Liu
CD20 negative B cell non-Hodgkin lymphoma (NHL) is rare and accounts for approximately 1-2% of B cell lymphomas. CD20- negative NHL is frequently associated with extranodal involvement, atypical morphology, aggressive clinical behaviour, resistance to standard chemotherapy and poor prognosis. The most common types of these include plasmablastic lymphoma, primary effusion lymphoma, large B-cell lymphoma arising from HHV8-associated multicentric Castleman's disease, and ALK+ large B cell lymphoma. This review provides an overview of the diagnostic and treatment modalities for CD20 negative B cell NHL...
2017: Biomarker Research
https://www.readbyqxmd.com/read/28183278/a-rare-case-of-renal-thrombotic-microangiopathy-associated-with-castleman-s-disease
#15
Anubha Mutneja, L Nicholas Cossey, Helen Liapis, Ying Maggie Chen
BACKGROUND: Castleman's disease (CD) is an uncommon, heterogeneous lympho-proliferative disorder leading to high circulating levels of interleukin-6 (IL-6) and vascular endothelial growth factor (VEGF). Renal involvement has been only described in a limited number of small studies. Herein, we report a rare case of renal thrombotic microangiopathy (TMA) associated with CD and investigate the podocyte expression of VEGF in the renal biopsy prior to initiation of treatment. CASE PRESENTATION: An 18-year-old male presented with fever, diarrhea, diffuse lymphadenopathy, ascites and acute kidney injury...
February 10, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28168662/multicentric-castleman-s-disease-associated-with-iga-vasculitis-henoch-sch%C3%A3-nlein-purpura-responding-well-to-tocilizumab-a-case-report
#16
REVIEW
Yoichi Oshima, Junichi Hoshino, Tatsuya Suwabe, Noriko Hayami, Masayuki Yamanouchi, Akinari Sekine, Toshiharu Ueno, Hiroki Mizuno, Junko Yabuuchi, Aya Imafuku, Masahiro Kawada, Rikako Hiramatsu, Eiko Hasegawa, Naoki Sawa, Kenmei Takaichi, Nobukazu Hayashi, Takeshi Fujii, Yoshifumi Ubara
A 41-year-old man was referred to our hospital for the evaluation of hypergammaglobulinemia (IgG 2898 mg/dL and IgA 587 mg/dL), inflammation (CRP 6.7 mg/dL and serum interleukin-6 (IL-6) 15.1 ng/L), and anemia (Hb 10.9 mg/dL). Castleman's disease (CD) was diagnosed by axillary lymph node biopsy. Five months later, painful purpura (multiple palpable 5 mm lesions) developed on his legs, gradually spreading to the upper limbs, thighs, and trunk, accompanied by arthralgia of the wrists, ankles, and knees...
March 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28163657/successful-treatment-of-deep-vein-thrombosis-caused-by-iliac-vein-compression-syndrome-with-a-single-dose-direct-oral-anti-coagulant
#17
Naoya Nakashima, Daisuke Sueta, Yusuke Kanemaru, Seiji Takashio, Eiichiro Yamamoto, Shinsuke Hanatani, Hisanori Kanazawa, Yasuhiro Izumiya, Sunao Kojima, Koichi Kaikita, Seiji Hokimoto, Kenichi Tsujita
BACKGROUND: Although vein stenting is popular for treatment for venous thromboembolism due to mechanical compression, some cases are forced to avoid inserting align agents because of immunodeficiency. CASE PRESENTATION: An 82-year-old man with left extremity redness and swelling presented to a hospital for a medical evaluation. The patient was immunodeficient because of the adverse effects of his treatment for Castleman's disease. A contrast-enhanced computed tomography scan revealed a venous thromboembolism in inferior vena cava and the left lower extremity...
2017: Thrombosis Journal
https://www.readbyqxmd.com/read/28143881/relapse-of-multicentric-castleman-s-disease-following-rituximab-based-therapy-in-hiv-positive-patients
#18
Alessia Dalla Pria, David Pinato, Jennifer Roe, Kikeri Naresh, Mark Nelson, Mark Bower
Successful treatment of HIV associated multicentric Castleman`s disease (HIV+MCD) with rituximab based approaches has dramatically improved survival and reduced the risk of HHV8-associated lymphoma. Longer term outcomes including relapse rates have not been described and are important to establish the potential role of maintenance therapy. A prospective cohort of 84 patients with biopsy proven HIV+MCD were treated with risk stratified rituximab based therapy. Four patients (5%) died from refractory HIV+MCD and 80 achieved clinical remission...
January 31, 2017: Blood
https://www.readbyqxmd.com/read/28140696/siltuximab-and-hematologic-malignancies-a-focus-in-non-hodgkin-lymphoma
#19
REVIEW
Andrea Ferrario, Michele Merli, Claudia Basilico, Margherita Maffioli, Francesco Passamonti
The role of interleukin-6 (IL-6) in tumorigenesis and in particular in haematological malignancies is crucial. On the basis of the favourable results obtained in the subset of multicentric Castleman disease (MCD), Siltuximab, a chimeric, human-murine, immunoglobulin (Ig) Gk monoclonal antibody directed against human IL-6 has been evaluated in haematological malignancies such as multiple myeloma, myelodisplastic syndromes and non Hodgkin lymphomas. Areas covered: This review discusses available data related to the role of IL-6 as a therapeutic target, the characteristics of Siltuximab in term pharmacokinetics and pharmacodynamics properties and a detailed analysis of the studies involving haematological malignancies with a peculiar focus on non Hodgkin lymphoma...
March 2017: Expert Opinion on Investigational Drugs
https://www.readbyqxmd.com/read/28135567/tafro-syndrome-new-subtype-of-idiopathic-multicentric-castleman-disease
#20
REVIEW
Gordan Srkalovic, Inga Marijanovic, Maya B Srkalovic, David C Fajgenbaum
Castleman disease (CD) describes a group of three rare and poorly understood lymphoproliferative disorders that have heterogeneous clinical symptoms and common lymph node histopathological features. Unicentric CD (UCD) involves a single region of enlarged nodes. Multicentric CD (MCD) involves multiple regions of enlarged lymph nodes, constitutional symptoms, and organ dysfunction due to a cytokine storm often including interleukin 6. MCD is further divided into Human Herpes Virus-8 (HHV-8)-associated MCD, which occurs in immunocompromised individuals, and HHV-8-negative/idiopathic MCD (iMCD)...
January 26, 2017: Bosnian Journal of Basic Medical Sciences
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