Plasmablastic lymphoma of the oral cavity in an HIV-positive child

Radhakrishnan, Raghu; Setty, Suhas; Kumar, Rekha V.; Krishnanand, Geeta; Srinivasan, R.; Rao, Nirmala N

doi:10.1016/j.tripleo.2005.03.028

Cited by 43 publications

(28 citation statements)

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“…Interestingly, there were two cases reported in children. One case was a 7-year-old-boy who presented with stage I disease and an oral primary site [18]. The second pediatric case was an 11-year-old girl with an EBV-positive cutaneous PBL, without specification of stage [26].…”

Section: Resultsmentioning

confidence: 99%

HIV‐associated plasmablastic lymphoma: Lessons learned from 112 published cases

2008

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Plasmablastic lymphoma (PBL) is a distinct subtype of non-Hodgkin B-cell lymphoma, originally described with a strong predilection to the oral cavity of human immunodeficiency virus (HIV)-infected individuals. Data regarding patient age and gender, HIV status, initiation of and response to highly active antiretroviral therapy (HAART), tumor extent, pathology, treatment, and outcome were extracted from 112 cases of PBL identified in the literature. The median age at presentation was 38 years with a male predominance of 7:1, and the median CD41 count was 178 cells/mm 3 . PBL presented on average 5 years after diagnosis of HIV. Common primary sites of presentation included the oral cavity, gastrointestinal tract, and lymph nodes. Most cases presented with either stage I or stage IV disease. There was a variable expression of B-cell markers in tumor cells, but plasma cell markers were expressed in all cases. EBV was detected in 74%. Chemotherapy was used to treat 55% patients and was combined with radiotherapy in 21% cases. Complete response was obtained in 66% of treated cases; the majority of these responses were seen after CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone). The refractory/relapsed disease rate was 54%. Death occurred in 53% of patients, with a median overall survival of 15 months. Sex, CD41 count, viral load, clinical stage, EBV status, primary site of involvement, and use of CHOP failed to show an association with survival. PBL is an aggressive B-cell lymphoma that presents in both oral and extra-oral sites of chronically HIV-infected immunosuppressed young men. Am. J. Hematol. 83:804-809, 2008. V

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Section: Resultsmentioning

confidence: 99%

HIV‐associated plasmablastic lymphoma: Lessons learned from 112 published cases

2008

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“…PBL usually develops in middle-aged adults, with the age at onset ranging from 35 to 55 years [5], but it can occur in the pediatric age group [6,7].…”

Section: Introductionmentioning

confidence: 99%

Clinicopathologic features of plasmablastic lymphoma: Single-center series of 8 cases from Saudi Arabia

et al. 2015

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Background: Plasmablastic lymphoma (PBL) is a rare subtype of non-Hodgkin's lymphoma. Characterized by its aggressive nature and plasmacytic differentiation, PBL remains a therapeutic and diagnostic challenge; it generally has a poor prognosis with very few long-term survivors and most patients dying within 2 years from initial presentation. PBL has been reported in several other countries; however, there have been no reported cases from Saudi Arabia. Here, we report 8 cases of PBL depicting the clinical presentation, immunocompetency, immunphenotypic characterization, diagnostic challenges and treatment outcome. Methods: The medical records were reviewed for clinical presentation, staging, laboratory data, radiological studies, treatments, and outcomes. A broad immunohistochemical panel consisting of CD45, CD3, CD20, CD79a, Pax5, CD38, CD138, MUM1, EMA, Kappa, Lambda, CD 56, CD30, Bcl-2, Bcl-6, Alk-1, Ki-67, EBV-LMP-1, and HHV8 was performed. Results: The tumors predominantly exhibited immunoblastic/plasmablastic or plasmacytic morphologic features and had a plasma cell-like immunophenotype. All cases were immunoreactive for CD38, CD138 and MUM1 confirming plasma cell differentiation of the tumor cells. CD20 was negative for all cases; whereas CD79a and Pax5 were weakly positive in 2cases. All 8 cases were EBV-LMP-1/EBER-1 negative, and 1 case was HHV8 positive. Similar to previously published studies, PBL in Saudi Arabia is characterized by male predominance (6/8), median age 51.5 years (mean age 46 years), associated with early dissemination, poor response to therapy, and limited survival (average survival time, 6.4 months, median overall survival 5.5 months). However, it does have some unique features. It occurs more commonly in immunocompetent persons (6/8, 75 %), is not associated with EBV infection (0/8), and nodal involvement (either primary or secondary) is common among patients (6/8). In addition, extra-oral sites are more common than oral/nasal cavities (7/8) and the c-myc gene is not common (1/8, 12.5 %).

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“…29 HIV enfeksiyonunu vertikal yolla kazanan yedi yaşında bir erkek çocukta oral PBL ilk olgu olarak 2005 yılında bildirilmiş, CD138 (+) bulunmuştur. 30 Yine sıklıkla orta-yüksek dereceli ve agresif seyirli olarak tanımlanan PBL, bir olguda zayıf diferansiye özofajeal tümör olarak bildirilmiş, HAART ve 6 kür kemoterapi sonrası olgu remisyona girmiştir. İHK testlerde CD20 (-), CD3 (-), CD30 (-), CD45 (+), CD138 (+) ve EBV (+) bulunmuştur.…”

Section: Discussionunclassified

Oral Plasmablastic Lymphoma in a Case with HIV Infection

Ural¹,

Kaptan²,

Türker³

et al. 2012

Turkiye Klinikleri J Med Sci

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Ö ÖZ ZE ET T Plazmablastik lenfoma (PBL) nadir görülen bir malignitedir ve en sık HIV (human immunodeficiency virus) ile enfekte olgularda ve yine sıklıkla oral kavitede saptanmaktadır. İlk kez 1997 yılında tanımlanmıştır. Diffüz büyük B-hücreli lenfomanın farklı bir alttipi olarak tarif edilmiştir. Son yıllarda sıklıkla oral kavite dışında da rapor edilmiştir. Gastrointestinal sistem ve deri, oral kavite dışında en sık tutulan organlardır. Plazmablastik lenfoma genellikle tipik B-hücre göstergelerinde (örnek olarak CD20) negatiflik, CD38, CD138 ve VS38c gibi plazma hücre göstergelerinde pozitiflik içeren karekteristik bir immunofenotip gösterir. Biz, bu makalede 44 yaşında, oral kavitede 30x30 mm boyutlarında kitle, akciğer tüberkülozu ve beyinde sol talamik bölgede 18x30 mm boyutlarında kitle lezyonu saptanan HIV ile enfekte bir erkek olgu sunduk. Oral kavitedeki lezyonun histopatolojik incelemesi PBL ile uyumlu idi. Neoplastik hücreler CD20 negatif bulundu. Hematoksilen eozin boyasında çok katlı yassı epitel altında plazmasitoid özellikte, iri eozinofilik nükleuslu, bazıları nükleolus içeren mitoz aktif neoplastik lenfoid infiltrasyon izlendi. Kemik iliği biyopsisinde non-Hodgkin lenfoma (NHL) tutulumu tespit edildi. Olguya HIV enfeksiyonu açısından HAART (Highly active antiretroviral therapy) tedavisi olarak stavudin + lamivudin + lopinavir/ritonavir; akciğer tüberkülozu tedavisi için dörtlü anti-tüberküloz tedavi olarak izoniazid + etambutol + rifampisin/streptomisin + morfoziamid; PBL için kemoterapi olarak siklofosfamid + doksorubisin + onkovin + prednizon (CHOP) ve radyoterapi verildi. Tedaviye rağmen olgu 7,5 ay izlendikten sonra kaybedildi. Erken tanı ve uygun tedavi olguların yaşam sürelerini uzatabileceği için HIV enfekte olguların nadir görülen maligniteler açısından taranmasına dikkati çekmek amacıyla ve ülkemizdeki ilk olgu olması nedeniyle bu olgu bildirilmiştir.A An na ah ht ta ar r K Ke el li im me el le er r: : HIV enfeksiyonları; lenfoma, Aids ile ilişkili; lenfoma, büyük B-hücreli, yaygın A AB BS ST TR RA AC CT T Plasmablastic lymphoma (PBL) is a rarely seen malignant lesion and it is most commonly detected in HIV (Human-immunodeficiency virus)-infected subjects predominantly in the oral cavity. It was first described in 1997. It has been recognized as a distinct entity, as a subtype of diffuse large B-cell lymphoma. Recently, PBL presentation has been reported frequently outside the oral cavity. Gastrointestinal tract and skin were the most commonly involved extra-oral organ systems. Plasmablastic lymphoma usually displays a characteristic immunophenotype that includes negativity for typical B-cell markers (eg, CD20), and positivity for plasma cell markers such as CD38, CD138 and VS38c. In this report, we described a case of PBL in a 44-year-old HIV infected male patient who presented with an expanding 30x30 mm oral lesion, pulmonary tuberculosis and an 18x30 mm cerebral left thalamic lesion. Histopathological evaluation of the lesion in oral cavity was consistent with PBL. The neoplastic c...

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Plasmablastic lymphoma of the oral cavity in an HIV-positive child

Cited by 43 publications

References 24 publications

HIV‐associated plasmablastic lymphoma: Lessons learned from 112 published cases

HIV‐associated plasmablastic lymphoma: Lessons learned from 112 published cases

Clinicopathologic features of plasmablastic lymphoma: Single-center series of 8 cases from Saudi Arabia

Oral Plasmablastic Lymphoma in a Case with HIV Infection

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