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Jorge J Castillo - One of the best experts on this subject based on the ideXlab platform.
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Plasmablastic Lymphoma and primary effusion Lymphoma
2020Co-Authors: Thomas Guerrerogarcia, Jorge J CastilloAbstract:Plasmablastic Lymphoma (PBL) and primary effusion Lymphoma (PEL) are rare CD20-negative variants of diffuse large B-cell Lymphoma. Both conditions were initially described in patients with underlying HIV infection, but cases of PBL and PEL have been more recently diagnosed in other immunocompromised states and in immunocompetent individuals. Given the rarity of these Lymphomas, the management is challenging due to a lack of treatment guidelines. In addition, the response and survival outcomes of patients with PBL and PEL are poor with standard treatment approaches. In this chapter, we will review the epidemiology, pathophysiology, clinical features, diagnostic evaluation, treatment, and outcomes of patients with PBL and PEL, as well as potential novel therapeutic options.
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primary cutaneous Plasmablastic Lymphoma in an immunocompetent patient is it associated with an indolent course
Leukemia & Lymphoma, 2018Co-Authors: Brady E Beltran, Pilar Quinones, Gadwyn Sanchez, Antonio Paredes, Celia Moises, Esther Cotrina, Carlos A Torrescabala, Roberto N Miranda, Jorge J CastilloAbstract:Plasmablastic Lymphoma (PBL) is a distinct clinicopathological entity of large, mature B-lymphocytes, closely related to diffuse large B-cell Lymphoma (DLBCL) [1–3], but also distinct because of it...
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bortezomib in Plasmablastic Lymphoma a glimpse of hope for a hard to treat disease
Leukemia Research, 2017Co-Authors: Thomas Guerrerogarcia, Renzo Mogollon, Jorge J CastilloAbstract:Plasmablastic Lymphoma (PBL) is a rare and hard to treat disease. With current standard chemotherapeutic regimens, PBL is associated with a median overall survival of 12-15 months. We performed a systematic review of the literature through March 31, 2017 looking for patients with a diagnosis of PBL who were treated with bortezomib, alone or in combination. We identified 21 patients, of which 11 received bortezomib in the frontline setting and 10 received bortezomib in the relapsed setting. Eleven patients were HIV-positive and 10 were HIV-negative. The overall response rate to bortezomib-containing regimens was 100% in the frontline setting and 90% in the relapsed setting. Furthermore, the 2-year overall survival of patients treated upfront was 55%, and the median OS in relapsed patients was 14 months. Although the sample size is small, we believe our results are encouraging and should serve as rationale to investigate bortezomib-based regimens in patients with PBL.
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bortezomib in combination with infusional dose adjusted epoch for the treatment of Plasmablastic Lymphoma
British Journal of Haematology, 2015Co-Authors: Jorge J Castillo, John L Reagan, William M Sikov, Eric S WinerAbstract:Plasmablastic Lymphoma (PBL) is a rare and aggressive CD20-negative Lymphoma. Despite improvements of the biology behind PBL, it still represents a challenge from the diagnostic and therapeutic perspectives for pathologists and clinicians. PBL is characterized by high rates of relapse and short median survival with standard approaches. Here, we report the use of the combination of bortezomib and infusional etoposide, prednisone, vincristine, cyclophosphamide and doxorubicin (V-EPOCH) in three patients with PBL; two were HIV-positive and one was HIV-negative. All three patients obtained a durable complete response to V-EPOCH with survival times of 24, 18 and 12 months respectively.
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the biology and treatment of Plasmablastic Lymphoma
Blood, 2015Co-Authors: Jorge J Castillo, Michele Bibas, Roberto N MirandaAbstract:Plasmablastic Lymphoma (PBL) is an aggressive Lymphoma commonly associated with HIV infection. However, PBL can also be seen in patients with other immunodeficiencies as well as in immunocompetent individuals. Because of its distinct clinical and pathological features, such as lack of expression of CD20, Plasmablastic morphology, and clinical course characterized by early relapses and subsequent chemotherapy resistance, PBL can represent a diagnostic and therapeutic challenge for pathologists and clinicians alike. Despite the recent advances in the therapy of HIV-associated and aggressive Lymphomas, patients with PBL for the most part have poor outcomes. The objectives of this review are to summarize the current knowledge on the epidemiology, biology, clinical and pathological characteristics, differential diagnosis, therapy, prognostic factors, outcomes, and potential novel therapeutic approaches in patients with PBL and also to increase the awareness toward PBL in the medical community.
Geon Kook Lee - One of the best experts on this subject based on the ideXlab platform.
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human immunodeficiency virus negative Plasmablastic Lymphoma in korea
Leukemia & Lymphoma, 2009Co-Authors: Ji Eun Kim, Geon Kook Lee, Young A Kim, Wook Youn Kim, Chul Woo Kim, Suk Jin Choi, Yoon Kyung JeonAbstract:Plasmablastic Lymphoma (PBL) is very rare, and predominantly occurs in Human immunodeficiency virus (HIV)-positive individuals. It shows a strong affinity for the oral cavity and for the Epstein-Barr virus (EBV) positive. We investigated the clinicopathologic characteristics of six cases of PBL in Koreans. All patients were HIV-negative and without underlying immunodeficiency. The age distribution was bimodal, and four patients were older than 60 years. Male predominance was observed with male to female ratio of 5:1. The organs primarily involved were the terminal ileum, stomach, oral cavity, tonsil, nasal cavity and meninges. The tumors were histologically typical of PBL. Three of them were composed of monomorphic large immunoblastic or Plasmablastic cells, and classified as PBL of the oral mucosa type. Another three cases were classified as PBL with plasmacytic differentiation. Five cases revealed loss of B-cell antigens with CD138 or MUM1 substitution. CD10 was positive in two cases (PBLs of the oral mucosa type), and one of them unexpectedly expressed cytokeratin. EBV was detected in one case (PBL with plasmacytic differentiation). Four patients succumbed to PBL in a relatively short period of time. We suggest that PBL is not strongly associated HIV or EBV in Koreans, and that it shows a variable organ distribution without an oro-nasal predilection.
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epstein barr virus and human immunodeficiency virus negative oral Plasmablastic Lymphoma
Journal of Oral Pathology & Medicine, 2006Co-Authors: Okjun Lee, Kyoungwon Kim, Geon Kook LeeAbstract:Plasmablastic Lymphoma (PBL) is an unusual subtype of human immunodeficiency virus (HIV)-related diffuse large B-cell Lymphoma that was first described in the oral cavity. HIV-related Lymphomas are frequently associated with Epstein-Barr virus (EBV). Recently, dual infection with EBV and human herpesvirus 8 (HHV8) has been demonstrated in PBL. So far, a few cases of PBL occurring in an HIV-negative patient have been documented and all of them were associated with immunosuppression status and/or EBV infection. Here we report a EBV and HHV8-negative oral PBL occurring in an immunocompetent HIV-negative male, which would be the first case.
Eric S Winer - One of the best experts on this subject based on the ideXlab platform.
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bortezomib in combination with infusional dose adjusted epoch for the treatment of Plasmablastic Lymphoma
British Journal of Haematology, 2015Co-Authors: Jorge J Castillo, John L Reagan, William M Sikov, Eric S WinerAbstract:Plasmablastic Lymphoma (PBL) is a rare and aggressive CD20-negative Lymphoma. Despite improvements of the biology behind PBL, it still represents a challenge from the diagnostic and therapeutic perspectives for pathologists and clinicians. PBL is characterized by high rates of relapse and short median survival with standard approaches. Here, we report the use of the combination of bortezomib and infusional etoposide, prednisone, vincristine, cyclophosphamide and doxorubicin (V-EPOCH) in three patients with PBL; two were HIV-positive and one was HIV-negative. All three patients obtained a durable complete response to V-EPOCH with survival times of 24, 18 and 12 months respectively.
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hiv negative Plasmablastic Lymphoma not in the mouth
Clinical Lymphoma Myeloma & Leukemia, 2011Co-Authors: Jorge J Castillo, Eric S Winer, Dariusz Stachurski, Kimberly Perez, Melhem Jabbour, Cannon Milani, Gerald A ColvinAbstract:Plasmablastic Lymphoma (PBL) is an aggressive variant of non-Hodgkin Lymphoma initially reported in the oral cavity of HIV-positive individuals. Since its original description, several cases have been reported in patients who do not have HIV infection. However, despite its recognition as a distinct subtype of diffuse large B-cell Lymphoma several years ago, comprehensive reviews of this entity are lacking. A MEDLINE search through June 2010 was performed to identify cases with a pathologic diagnosis of HIV-negative PBL based on morphology and minimal immunohistochemical criteria. Our study included a total of 76 cases. The median age was 57 years (range, 1 to 90 years) with a male-to-female ratio of 1.7. Seventy-four percent of cases did not have an apparent association with immunosuppression, 18% had a concurrent lymphoproliferative or autoimmune disorder and 9% developed PBL after solid organ transplantation. Oral involvement was observed in 21%, advanced stage in 60%, Epstein-Barr virus–encoded RNA expression was positive in 45% and Ki-67 expression of greater than or equal to 80% in 61% of the cases. Chemotherapy was documented in 43 patients, from which 43% received the cyclophosphamide, doxorubicin, vincristine, prednisone (CHOP)–like regimens. The median and the 2-year overall survival for the whole group were 9 months and 10%, respectively. Patients who had HIV-negative PBL have distinct clinicopathological characteristics, such as short overall survival and lower rates of oral involvement and Epstein-Barr virus–encoded RNA expression than the previously reported in HIV-positive patients.
Alison Street - One of the best experts on this subject based on the ideXlab platform.
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aids related Plasmablastic Lymphoma of the oral cavity associated with an igh myc translocation treatment with autologous stem cell transplantation in a patient with severe haemophilia a
Haematologica, 2007Co-Authors: Mark A Dawson, Anthony P Schwarer, Lynda J Campbell, Edwina J Wright, Jake Shortt, Catriona Mclean, Alison StreetAbstract:Plasmablastic Lymphoma is an AIDS related Lymphoma that continues to have a poor prognosis despite significant advances in the management of HIV and lymphoproliferative diseases. In part this has been due to limited insights into the biology of this disease and the molecular mechanisms of oncogenesis. To date molecular abnormalities have not been described in Plasmablastic Lymphoma, and its aggressive clinical behaviour has been difficult to understand. We describe the first reported cytogenetic abnormality in Plasmablastic Lymphoma, an IgH/MYC translocation. It is also the first description of autologous stem cell transplantation in a patient with severe haemophilia A.
Chris Boshoff - One of the best experts on this subject based on the ideXlab platform.
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hhv 8 is associated with a Plasmablastic variant of castleman disease that is linked to hhv 8 positive Plasmablastic Lymphoma
Blood, 2000Co-Authors: N Dupin, Tim L Diss, Paul Kellam, M Tulliez, Didier Sicard, Robin A Weiss, Peter G Isaacson, Chris BoshoffAbstract:Castleman disease (CD) is a lymphoproliferative disorder of unknown etiology that is associated with the development of secondary tumors, including B-cell Lymphoma. Human herpesvirus 8 (HHV-8) (Kaposi's sarcoma-associated herpesvirus) sequences have been described in some cases of multicentric Castleman disease (MCD). Using a monoclonal antibody against an HHV-8-latent nuclear antigen, we show that HHV-8 is specifically associated with a variant of MCD in which HHV-8-positive plasmablasts that show lambda light-chain restriction localize in the mantle zone of B-cell follicles and coalesce to form microscopic Lymphomas in some cases. Furthermore, we show that the frank Plasmablastic Lymphoma that develops in patients with this Plasmablastic variant of MCD is also positive for HHV-8 and lambda light chain. Plasmablastic Lymphoma associated with MCD is a new disease entity associated with HHV-8 infection. (Blood. 2000;95:1406-1412)